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Organised By: Supported By:

IFCN

ASIAN-OCEANIAN
CONGRESS ON
CLINICAL
30 January to 1 February 2021
*Postponed from 9 to 12 April 2020
NEUROPHYSIOLOGY
Virtual Congress:
www.aoccn2020.com

info@aoccn2020.com

ABSTRACT BOOK
CONTENT

Invited Speakers

Subject Page

Plenary Lectures 6

Symposium

1. Non-Invasive Brain Stimulation 10


2. Movement Disorders 16
3. Motor Neuron Disease 23
4. Neuromuscular Junction and Related Disorders 27
5. Intraoperative Neurophysiological Monitoring 30
6. Epilepsy: Advanced EEG in Clinical Practice 35
7. Peripheral Neuropathy 40

Workshops

1. Electroencephalogram 45
2. Transcranial Magnetic Stimulation 55
3. Nerve Conduction Studies/Electromyography 58
4. Advanced Neurophysiology Techniques 64
5. Intraoperative Neurophysiological Monitoring 67
6. Single-Fiber EMG 74
7. Neuromuscular Ultrasound 78

Meet-The-Expert 87

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Conference abstracts

Oral Presentation

Topic Page

Electroencephalogram 91

Epilepsy 95

Transcranial Stimulation 98

Nerve Conduction Studies/Electromyography 102

Autonomic Dysfunction 106

Movement Disorders 111

Motor Neuron Disease 114

Neuromuscular Junction Disorders 118

Intraoperative Monitoring 120

Evoked Potential Studies 121

Peripheral Nerve Disorders 124

Neuromuscular Ultrasound 127

Basic Neuroscience 129

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E-Poster Presentation

Topic Page

Electroencephalogram 132

Epilepsy 138

Transcranial Stimulation 162

Nerve Conduction Studies/Electromyography 167

Autonomic Dysfunction 186

Sleep 189

Movement Disorders 193

Motor Neuron Disease 212

Neuromuscular Junction Disorders 216

Intraoperative Monitoring 222

Evoked Potential Studies 224

Peripheral Nerve Disorders 227

Neuromuscular Ultrasound 257

Muscle Disorders 263

Pain 268

Basic Neuroscience 271

Miscellaneous 274

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Speaker Abstracts

5
Plenary Lectures (PL)
PL1. Face Perception in Infants Using Near-Infrared Spectroscopy (NIRS)

Ryusuke Kakigi1
1Department of Integrative Physiology, National Institute for Physiological Sciences,

Okazaki, Japan

We have been studying the underlying mechanisms of face perception in humans using
magneto-(MEG), electroencephalography (EEG) and near-infrared spectroscopy (NIRS). In
this lecture, I will mainly focus on the development of face perception in infants. NIRS is a
spectroscopic method using the near-infrared region of the electromagnetic spectrum (from
780 nm to 2500 nm). The biggest advantages of NIRS compared with functional magnetic
resonance imaging (fMRI) are as follows: (1) NIRS can be used when subjects are moving, for
example, during walking and cooking. Therefore, NIRS can be used to measure brain activity
of infants and children who cannot help moving; (2) It is much cheaper. We recently made a
special light probe of NIRS for studying infants. I will mainly introduce our basic studies of face
perception in infants using NIRS. In addition, I will introduce our latest clinical studies in
patients with attention deficit hyperactivity disorder (ADHD) and anorexia nervosa.

PL2. Noninvasive Brain Stimulation - The Past, the Present and the Future

Walter Paulus1
1Department of Clinical Neurophysiology, University Medical Center Göttingen, Germany

Non-invasive brain stimulation started in the antiquity by treating headache with electric fishes
(~ 1 A, ~ 700 V). Transcranial direct current stimulation dominated research in the 19 th century
after the invention of the voltaic pile (~ 1 mA, ~10 V). At present the main applications involve
either weak electric stimulation of many different types (transcranial direct, alternating, random
noise or combinations) of currents or high energy pulsed stimulations (~8 kA, ~ 10 µs)
mediated by transcranial magnetic energy transfer (transcranial magnetic stimulation: TMS).
Inhibitory or excitatory after-effects are guided by stimulus intensity, duration and intervals (cf.
theta burst, quadripulse and others). In general, lower intensities lead to inhibition and higher
intensities to excitation. There is no linear correlation between the duration of stimulation and
the size of the after-effects, prolongation of stimulus duration may lead to an oscillating pattern
of inhibition or excitation. Missing the right windows in time or in amplitude may lead to no or
opposite results explaining the variability and conflicting data in the field. Intracellular calcium
increase may be a key leader for deciding between LTD- or LTP-like after-effects. Uncontrolled
consumption of nicotine and caffeine increase variability further. Co-application of drugs may
guide after-effects in terms of selectivity and duration of after-effects.

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Current developments encompass new physical techniques such as varying TMS pulse
widths, multi-electrode stimulation, interference fields, ultrasound or near infrared stimulation,
hypothesis driven protocols based on neurophysiological features of cortical neurons,
optimized techniques on realistic individual head models for targeting current flow to
predefined areas and others. Clinical applications targeting cortical excitability increases (e.g.
depression, pain) or decreases (e.g. epilepsy, migraine) seem to be more rewarding than
those requiring structural repair (e.g. stroke).

PL3. Clinical Neurophysiology of Movement Disorders - Example of


Functional Movement Disorders
Mark Hallett1
1Human Motor Control Section, NINDS, NIH, Bethesda, MD, USA

Functional movement disorders are best diagnosed by positive features. Much can be done
with the clinical examination, but often there are ambiguities. Further positive evidence can be
obtained with clinical neurophysiology which can then lead to a “laboratory supported”
diagnosis. The two entities for which clinical neurophysiology is useful are myoclonus and
tremor.

Functional tremor may well exhibit variability in frequency at different times or excessive
fluctuations in the short term. The tremor might be in-phase in two different limbs, and this
should not be the case except for orthostatic tremor. The tremor might entrain or be altered in
frequency with tapping with another limb or be briefly interrupted by a quick movement of
another limb. There might be a co-contraction sign where there is a co-contraction prior to the
development of tremor.

For functional myoclonus, the EMG activity shows long bursts of activity and often
inconsistency with different jerks. EEG back-averaging can show a normal looking
Bereitschaftspotential prior to the jerk. Comparison with a voluntary mimic or other voluntary
movement is often valuable to see what the Bereitschaftspotential looks like in that individual.
When the myoclonus is stimulus sensitive, the responses look like voluntary reaction time
movements both in latency and variability.

TMS can be used in functional paresis to demonstrate a normal pathway from motor cortex to
muscle. Interestingly, this technique has sometimes also been therapeutic. Another feature of
the TMS response in functional paresis is that the MEP amplitude decreases with movement
imagination of that muscle. Since ordinarily imagination produces facilitation, this shows a high
level inhibition of movement in the brain.

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PL4. The Clinical Application of ALS Electrodiagnostic Criteria
David Burke1
1Department of Neurology, Royal Prince Alfred Hospital, and University of Sydney, Australia

A major driving force behind efforts to make an early diagnosis of ALS relates to the criteria for
inclusion in clinical trials at a stage when the patient still has some function that can be
rescued. The diagnostic criteria for ALS have always been clinical, but diagnosis has been
assisted by neurophysiological evidence of dysfunction. The value of neurophysiology became
more explicit with the publication of the Awaji criteria (de Carvalho et al., 2008) and a
subsequent update (Geevasinga et al., 2016). Meta-analyses have revealed higher
sensitivities of Awaji criteria, when compared to the revised El-Escorial criteria in limb-onset
disease but not in bulbar-onset disease. There was slightly better performance of the updated
Awaji criteria (Geevasinga et al., 2016).

There are now approaches that may demonstrate UMN features in patients with EMG
evidence of LMN abnormalities, and a diagnosis of ALS is difficult to avoid when the UMN and
LMN features involve the same muscle group. Twelve years have passed since the original
Awaji publication, and a small consensus meeting was held on the Gold Coast, Queensland,
Australia last September to address whether research advances could now be incorporated
into the diagnostic criteria. Rather than making yet another modification to old criteria, we
resolved to start again using a more simple approach, discarding the terms “possible”,
“probable” and “definite”, on the grounds that, in practice, these terms are often used to define
extent of involvement rather than diagnostic certainty. These thoughts will be discussed.

de Carvalho et al. Clin Neurophysiol 2008; 119: 497-503


Geevasinga et al. Clin Neurophysiol 2016; 127: 2684–2691

PL5. Training and Education in Europe, Middle East, Africa, Latin America
and Asia Oceania Chapters, IFCN: An International Survey

Jonathan Cole1
1University of Bournemouth and Poole Hospital, UK

The chapters of the IFCN, Asia Oceania (AOC), Europe Middle East and Africa, (EMEAC),
Latin America (LAC), and North America (NAC), have cooperated to undertake, for the first
time, a survey of training and education practice in their national societies. The results have
been pooled so that data on aspects of our practice may now be compared and contrasted.
The survey looked at the status of clinical neurophysiology, (mono- or subspecialty) within
countries, training duration, numbers of cases seen independently for competence,
examination methods, the presence of a national curriculum and who controls it, how
competency was examined, and accreditation. It also considered training for technicians,

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clinical practice including waits and methods of funding, revalidation and, lastly, concerns
within our specialty.

Clinical Neurophysiology remains a monospecialty in 16 out of 59 countries. Training durations


vary between 1 to 5 years, with most lasting 2-3 years in the LAC and AOC, whilst in the
EMAEC there is a fairly even spread of durations between the 1 to 5 years. An exit exam is
present in 50% EMEAC societies, 75% of AOC and in 3 out for 12 LAC ones. The numbers of
EMG and EEG tests performed necessary for competence were <250 to >750. In the EMEAC,
half the societies required over 500 EMG and EEG tests, whilst the AOC required fewer.
Technicians were usually trained for 2-4 years. Within the EMEAC there is no formal
requirement for continuing education or for revalidation in 64%. In the AOC, continuing medical
education is required in 11 out of 12 countries, but revalidation needed only in 4. The main
concerns were over workload in EMEAC and in the provision of training in AOC. Work has also
been taking place in a draft curriculum. The utility of these initiatives remains to be determined,
but at least, for the first time, some comparative data on training and education around the
world is being assembled.

PL6. Treatment Continuum of Nonconvulsive Status Epilepticus

Aatif M. Husain1
1Department of Neurology, Duke University Medical Center; Neuroscience Medicine, Duke

Clinical Research Institute; Neurodiagnostic Center, Veterans Affairs Medical Center, Durham,
North Carolina, USA

Acute seizures and status epilepticus (SE) are commonly encountered in critically ill patients.
With continuous EEG (cEEG) monitoring it is possible to differentiate whether patients are in
on-going, continuous SE or having intermittent electrographic seizures. This data should be
used to determine the urgency and aggressiveness of treatment for each patient. In this
presentation the rationale for “appropriately aggressive” treatment will be discussed in the
context of the risk-benefit calculus. Initially the evolving definition of SE will be discussed. This
will be followed by a discussion about measuring the severity of electrographic SE, and how
this is valuable in determining appropriate therapeutic targets. Finally, the concept of
“appropriately aggressive” therapy for SE will be discussed.

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Symposium (SY)
Symposium 1: Non-Invasive Brain Stimulation

SY1.1. The Effects of Non-Invasive Brain Stimulation and Neuroplasticity

Ying-Zu Huang1
1Medical School, Chang Gung University, Department of Neurology, Chang Gung Memorial

Hospital Taoyuan, Taiwan

The recent development of non-invasive brain stimulation (NIBS) techniques, e.g. repetitive
transcranial magnetic stimulation (rTMS) and transcranial direct/alternating current stimulation,
are capable of suppress or enhance the brain excitability outlasting the period of stimulation for
up to one hour or more. These protocols mimic the repetitive or constant electrical stimulation
that is commonly used to change the synaptic efficiency forming the basic concept of synaptic
plasticity mechanism in animal brain slices. Neuroplasticity or plasticity is generally believed as
the fundamental mechanism of many aspects of brain function, e.g. learning, memory and
recovery. Several lines of evidence based on pharmacological physiological and behavioural
studies in humans and protein analysis in animals have confirmed that the after effects of NIBS
are induced through a plasticity-like mechanisms. In this talk, the basic concept of plasticity will
be introduced and how the effects of NIBS are linked to the mechanisms of plasticity will be
explained. The audience will understand how NIBS works on the brain and how to design their
applications of NIBS based on the knowledge.

SY1.2. The Diagnostic Utility of Threshold Tracking TMS in ALS

Steve Vucic1
1Western Clinical School, University of Sydney, Sydney, Australia

Concomitant dysfunction of upper (UMN) and lower motor (LMN) neurons is the
pathognomonic feature of amyotrophic lateral sclerosis (ALS). The relationship between upper
and lower motor neuron dysfunction in ALS remains to be elucidated, although it has been
proposed that the disease origin lies within the central nervous system, with subsequent LMN
degeneration progressing via an anterograde glutamatergic excitotoxic mechanism.
Transcranial magnetic stimulation (TMS) is a neurophysiological tool that provides a non-
invasive and painless assessment of cortical function. Recently, threshold tracking
methodologies have been adopted for TMS, whereby changes in threshold rather than motor
evoked potential (MEP) amplitude serve as outcome measures, leading to the development of
a more reliable and quicker tool for assessing cortical function in ALS. Importantly, this
threshold tracking TMS technique has established that cortical hyperexcitability is an early
feature in sporadic ALS that precedes the onset of LMN dysfunction and mediates the pattern

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of disease spread. Separately, cortical hyperexcitability was reported to precede the clinical
onset of familial ALS phenotypes. Of further relevance, the threshold tracking TMS technique
was proven to reliably distinguish ALS from mimicking disorders, even in the presence of a
comparable degree of LMN dysfunction, suggesting the diagnostic utility of TMS.

Taken in total, threshold tracking TMS has provided support for a central origin of ALS,
underscoring the utility of the technique for probing the underlying pathophysiology. This
presentation will discuss the physiological processes underlying threshold tracking TMS, while
further evaluating the pathophysiological and diagnostic utility of threshold tracking TMS in
ALS.

SY1.3. Probing the Pathophysiology of Movement Disorders with


Transcranial Magnetic Stimulation
Jung E Park1
1Department of Neurology, Dongguk University Ilsan Hospital, Goyang, Republic of Korea

Transcranial magnetic stimulation (TMS) is a widely-used noninvasive brain stimulation


method for research in the field of neurology, psychiatry, and rehabilitation. TMS can be used
to study connectivity and therefore help to shed light on brain networks. This mode of
noninvasive brain stimulation is particularly useful to study brain regions relevant to movement,
as stimulating the primary motor cortex results in motor-evoked potential (MEP)s, which can be
useful for interpreting the effects of various brain regions on the motor cortex. Connectivity
among the primary motor cortex and other movement-relevant regions can be studied by using
a paired- or multiple-pulse technique. This is possible by assessing the effects of a pre-
conditioning stimulus on the conditioning-test pair or a conditioning stimulus on the test pulse.
TMS can also be used in conjunction with electroencephalography (EEG),
magnetoencephalography (MEG) or imaging tools such as functional magnetic resonance
imaging (fMRI) or positron emission tomography (PET) to further assess TMS-induced
changes. Combining these techniques can enable researchers to obtain spatial or temporal
information of the effects of TMS on the brain.

Movement in healthy human subjects has been studied using TMS, and information obtained
from these studies has been useful in understanding which brain regions are relevant in
producing movement. Furthermore, there are various TMS measures of the motor cortex that
reflect different aspects of cortical excitability. Some examples are short and long intracortical
inhibition (SICI and LICI), intracortical facilitation (ICF), short- and long- afferent inhibition (SAI
and LAI), transcallosal inhibition, premotor cortex inhibition, etc. TMS has also been used to
study movement disorders such as Parkinson’s disease, dystonia, essential tremor, and
Huntington’s disease. While TMS still has limited use in the clinical realm, it has provided a
wealth of data in our understanding of movement disorders.
In this talk, I will give an overview of TMS studies that probe the physiology of normal human
movement and also discuss those focusing on movement disorders. I will also review

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limitations of TMS and the findings of TMS studies to date, and briefly discuss the need for
future studies.

SY1.4. Can TMS Be Used to Improve Prediction Accuracy After Stroke?

Winston Byblow1
1University of Auckland, Auckland, New Zealand

Recovery of upper limb function is critical for regaining independence after stroke. Inter-subject
variability complicates trials of novel stroke rehabilitation therapies, particularly in the sub-
acute phase after stroke, when predicting upper limb outcomes based on clinical impression
alone is no better than chance. Fortunately TMS can be used to determine a patients motor
evoked potential MEP status, a key biomarker of corticospinal tract integrity. If MEP status is
determined within 5 days of stroke it can be used for individual patients to predict their
recovery potential of upper limb function at 12 weeks, in the context of the PREP2 algorithm.
Having such information available at the beginning of rehabilitation allows therapists and
patients to set realistic goals, and has been shown to improve the efficiency of the
rehabilitation service. Selecting patients using MEP status can also improve trial efficiency.
A retrospective analysis of data from 207 patients was used to estimate sample sizes and
recruitment rates required to detect a 7-point difference between hypothetical control and
treatment groups in upper-limb Fugl-Meyer and Action Research Arm Test scores at 90 days
post-stroke. Selecting patients according to MEP status significantly reduced the required
sample size and time required to recruit compared to an unselected sample. In conclusion,
MEP status is a key biomarker that can be obtained by the clinical neurophysiologist to help
select patients and improve stroke rehabilitation trial efficiency to more efficiently detect a
clinically meaningful effect of a tested intervention.

SY1.5. EEG-TMS: Physiology and First Evidence of Clinical Utility

Ulf Ziemann1
1University of Tübingen, Tübingen, Germany

Major advancements in neuro-technology now allow to record and analyze activity of the
human brain in real-time non-invasively by EEG. This information of instantaneous brain
activity can be used to trigger non-invasive brain stimulation (transcranial magnetic stimulation,
TMS) brain-state-dependently. This is of immense conceptual importance. We have shown
that µ-oscillations in human sensorimotor cortex determine fluctuations in corticospinal
excitability, as measured by motor evoked potential (MEP) size. The positive peak of the µ-

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oscillation is associated with smaller MEPs (low-excitability state), whereas the negative peak
of the µ-oscillation (trough) is associated with larger MEPs (high-excitability state) [Zrenner et
al. 2018, Brain Stimulation 11:374-389]. If these two states are now being stimulated
repetitively, in two different sessions, by high-frequency bursts (100 Hz triplets) of TMS, then
the identical stimulation protocol results in long-term potentiation-like corticospinal plasticity
(long-term increase in MEP size) if the high-excitability state (the negative peak of the µ-
oscillation) is consistently targeted, but in a trend towards long-term depression if the low-
excitability state (the positive peak of the µ-oscillation) is targeted.

Therefore, the outcome of non-invasive brain stimulation depends highly significantly on the
instantaneous brain state. We expect that this novel technology of EEG-informed brain-state-
dependent stimulation will enable a new era of highly efficacious therapy for brain network
disorders, such as stroke, Alzheimer’s disease or major depression. First proof-of-principle
data in depression will be demonstrated.

SY1.6. Therapeutic Application of Transcranial Magnetic Stimulation in


Stroke

Cathy Stinear1
1Department of Medicine, University of Auckland, Auckland, New Zealand

Hundreds of studies have investigated the possible benefits of non-invasive brain stimulation
techniques for promoting recovery and outcomes after stroke. Most of these have applied
suppressive forms of transcranial magnetic and direct current stimulation to contralesional
motor cortex, based on the interhemispheric imbalance model. This model posits that the
contralesional primary motor cortex is hyperexcitable after stroke and excessively inhibits the
ipsilesional primary motor cortex via transcallosal projections. The assumption is that
reorganisation and recovery in the ipsilesional hemisphere can be promoted by reducing the
excitability of contralesional hemisphere, to reduce its transcallosal inhibition of the ipsilesional
hemisphere. However, this general strategy doesn’t reliably improve motor performance, and
non-invasive brain stimulation techniques have not translated to routine clinical care. This
presentation will identify the limitations of previous work, and draw from more recent studies
that prompt a re-think of the interhemispheric imbalance model, and the role of
interhemispheric inhibition in motor control. Deepening our understanding of interhemispheric
interactions after stroke may enable more personalised therapeutic applications of transcranial
stimulation to promote recovery and improve outcomes.

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SY1.7. Rewiring Pain Through Non-Invasive Brain Stimulation

Nahian Chowdhury1
1Neuroscience Research Australia, Sydney, Australia

Chronic pain occurs when pain in an injured tissue persists beyond the normal recovery time.
This crippling condition causes significant burden to the individual and to society. Despite
years of research, treatments for chronic pain are largely ineffective, which may in part be due
to a lack of understanding of the mechanisms that underpin chronic pain. There is increasing
evidence that chronic musculoskeletal pain is associated with sensorimotor dysfunction, and
that targeting the mechanisms associated with this dysfunction using non-invasive brain
stimulation may alleviate symptoms of pain. This presentation will highlight studies
investigating the manifestation of sensorimotor neuroplasticity in a range of musculoskeletal
pain conditions such as low back pain, persistent elbow pain and persistent patellofemoral
pain. These studies have used transcranial magnetic stimulation and electromyography
methodologies and have shown that the organisation of the sensorimotor cortical
representation of the pain afflicted muscle is altered. Novel treatment studies will then be
reviewed. These studies have attempted to “rewire” pain by targeting sensorimotor cortical
organisation using ‘top-down’ (non-invasive brain stimulation) and “bottom-up” approaches
(trans-spinal stimulation, peripheral electrical stimulation and exercise).

The potential limitations of these approaches will then be discussed including the large
variation in responsiveness to treatment, and the need for individualized treatment. Indeed, our
work using transitional pain models in healthy human subjects has shown that inter-individual
differences in sensorimotor function in a pain free state and during persistent pain, can predict
the severity of pain. It will be argued that tailoring treatment in accordance with an individual’s
sensorimotor function in the early stages of pain may facilitate the diagnosis, prevention and
treatment of pain. A planned project will be presented which will focus on modulating pain in
accordance with these individual differences using non-invasive brain stimulation.

SY1.8. Application of TMS in Movement Disorders

Yoshikazu Ugawa1
1Fukushima Medical University, Fukushima, Japan

I will summarize several TMS applications in movement disorders.

Function of supplementary motor area (SMA) and preSMA revealed by QPS (Shimzu T et al).
We studied functional differences between the pre-SMA and SMA in sequence learning in
humans. To induce LTP/LTD, we administered quadripulse transcranial magnetic stimulation
(QPS) with an inter-stimulus interval of 5 or 50 ms (QPS-5/50) over the pre-SMA or SMA in
healthy volunteers. QPS-5 over the pre-SMA increased the error rate without any changes in

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movement speed. When administered over the SMA, QPS affected the rate of reaction time
reduction across trials without any changes in the error rate.

Cerebellar involvement and cerebellar stimulation treatments.


Essential tremor (Hanajima et al).
Several lines of evidence suggested a cerebellar pathophysiology of essential tremor (ET). It
was recently confirmed by human pathology, neuroimaging studies and others. This
hypothesis was also confirmed by cerebellar TMS over cerebellum (cerebellar inhibition: CBI)
and prism adaptation task in ET patients (Hanajima et al).

Cerebellar stimulation treatments in movement disorders (Çan, M et al).


Many researchers have been interested in cerebellar involvement in movement disorders and
also cerebellar stimulation as one of treatment methods. However, our magnetic coil must
activate cerebellum in some extent but not purely cerebellum alone, and always activate some
other structures. The simulation study showed considerable amount of currents must be
induced in the cerebellum even with a small coil. Cerebellar stimulation is one of future
projects of TMS.

Transcranial focused ultrasound stimulation (tFUS).


Very low intensity tFUS makes reversible functional changes in human brain. This reversible
modulation is used for functional studies of human brains. Its focality and ability to induce
functional changes in deep brain structures, such as thalamus, must enable this method to be
used in movement disorders.

Alternative leg movements induction by lumbar rTMS (Nishimura et al).


The lumbar gait center is known to produce gait-like alternative leg movements in animals. We
made a system to voluntary induce a gait-like leg movement triggered by her/his own upper
limb EMGs. This system induced alternative leg movements, gait like movements, in patients
with paraplegia due to spinal cord injury.

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Symposium 2: Movement Disorders

SY2.1. Evaluation of Tremor

Pattamon Panyakaew1
1Chulalongkorn Center of Excellence for Parkinson’s Disease & Related Disorders,

Department of Medicine, Faculty of Medicine, Chulalongkorn University and King


Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand

Tremor is defined as an involuntary, rhythmic (regularly recurrent) and oscillatory (rotating


around a central plane) movement of the body part. The evaluation of tremor should begin with
careful history taking and a focused clinical examination on tremor phenomenology and
associated neurological signs. The first step of the approach is to exclude tremor mimics such
as myoclonus, clonus or other hyperkinetic movements. The second step is to carefully
evaluate clinical features and establish whether the tremor is isolated or combined with other
neurological problems and the predominant tremor is at rest or action. Tremor can be
classified into syndromes depending on the characteristics (e.g. activation of tremor,
distribution of tremor) and prominent additional signs. The common tremor syndromes during
action are essential tremor, enhanced physiologic tremor, dystonic tremor, drug-induced
tremor, cerebellar tremor, rubral tremor, and orthostatic tremor while the common tremor
syndromes at rest are parkinsonian tremor.

A detailed tremor history and neurological examination are usually enough for the clinical
evaluation of tremor syndromes. However, the clinical presentations are sometimes complex
and electrophysiologic investigations can be helpful in making the diagnosis of tremor
subtypes. The basic neurophysiologic test is a multi-channel surface EMG combined with
accelerometry to assess the distribution of tremor, tremor frequency, and amplitude during
various types of activation of tremor, the effect of loading and distraction on tremor frequency
and amplitude. The characteristics of central tremor syndromes are more stable tremor
frequency and less influenced by loading compared to peripheral tremor syndromes. The peak
tremor frequency can be overlapped among tremor subtypes but a unique tremor frequency
can be found in some syndrome such as orthostatic tremor, cerebellar tremor and rubral
tremor. The specific neurophysiologic procedures including distractibility and entrainment are
sensitive and specific for functional tremors. Recently, more advanced neurophysiologic tests
have emerged. There is a promising role of intermuscular coherence analysis in the differential
diagnosis of tremor subtypes. Evaluation of tremor based on clinical and neurophysiological
features are important, leading to the appropriate diagnosis and treatment.

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SY2.2. Evaluation of Myoclonus

Ritsuko Hanajima1
1Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori

University, Yonago, Japan

Myoclonus is a shock-like jerky movement observed in the extremities or the trunk without
consciousness loss. This involuntary movement is generated by abnormal neuronal
discharges in the central nerve system caused by many kinds of etiologies.

Based on the physiological generator mechanism, myoclonus is classified mainly into the three
groups; cortical myoclonus, subcortical myoclonus and spinal myoclonus.
Cortical myoclonus includes spontaneous cortical myoclonus and cortical reflex myoclonus. In
cortical myoclonus, an EEG spike precedes a muscle jerk. Backward EEG averaging time-
locked to the onset of myoclonus (jerk-locked back averaging: JLA) often detects cortical spike
preceding the myoclonus, which is unable to be shown by EEG-EMG polygraph recordings.
The duration of EMG jerks is usually shorter than 50-100ms. Somatosensory evoked potentials
are often enlarged (giant SEP) in cortical reflex myoclonus. Enhanced long loop reflex is also
often seen in the polygraph in the cortical reflex myoclonus (so called “C” reflex). The
disinhibition of the sensory cortex can be revealed by SEP recovery curve studied by paired
pulse stimulation. The hyperexcitability of the motor cortex is shown by the paired pulse
transcranial magnetic stimulation (TMS) technique. The short interval intra-cortical inhibition
(SICI) was abnormally reduced. These physiological features indicate the cortical hyper-
excitability.

Subcortical myoclonus is supposed to be generated at the basal ganglia or brainstem. The


duration of muscle jerk is longer than 50-100ms. In some subcortical myoclonus (reticular
myoclonus), the latency of long loop reflexes is shorter than those of enhanced C-reflexes. JLA
reveals no cortical spikes, and no giant SEPs are seen.
Spinal myoclonus includes “segmental spinal myoclonus” which is generated by activation of
certain spinal segments, and “propriospinal myoclonus” which originates from a certain spinal
segment and spreads upward/downward slowly though the propriospinal tract. Simultaneous
EMG recordings of many muscles are useful to estimate the spinal conduction between
different spinal cord levels.
Electrophysiological studies are useful to evaluate myoclonus and to suggest the generator
mechanisms for myoclonus. Here, I will introduce some neurophysiological methods for
analyses of myoclonus.

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SY2.3. Evaluation of Dystonia

Pramod Kumar Pal1


1National Institute of Mental Health and Neurosciences, India

Dystonia is a syndrome characterized by sustained or intermittent muscle contractions causing


abnormal, often repetitive, movements, postures, or both. It can be primary or secondary and
the patient can present with pure dystonia or dystonia along with other movement disorders
such as tremor, myoclonus, parkinsonism, etc. Evaluation of a patient presenting primarily
with dystonia includes detailed clinical evaluation, haematologic and neurometabolic work up,
imaging and neurophysiological/ electrophysiological studies. This presentation will focus on
the electrophysiological evaluation of dystonia, which helps in understanding the
pathophysiology and better characterization of the abnormal movement.

Compared to tremor and myoclonus, electrophysiological evaluation of muscle spasms and


dystonia is difficult. The first step is recording surface EMG from the involved muscles for
prolonged periods during abnormal movements (dystonia), rest, and with voluntary contraction.
The agonists and antagonists should be included to detect co-contraction, and the unaffected
muscles of the same or opposite limbs should also be studied to document overflow dystonia if
present. During EMG evaluation, patients are also asked to perform various tasks which can
induce dystonia, such as mental arithmetic, finger tapping and opening and closing fist of the
unaffected hand, writing (in patients with writer’s cramp). EMG is analyzed to determine: (i) the
pattern of EMG activity in agonists and antagonists during dystonia (spasm) (ii) duration of
each EMG burst (iii) the regularity of occurrence of EMG bursts, (iv) presence of overflow
activity in remote muscles while performing discrete voluntary acts, and (v) the difference in
the degree of EMG activity (area of rectified EMG) between epochs with and without muscle
spasms.

Other investigations which are often useful to characterize dystonia or a dystonic syndrome
include: (i) H-reflex recovery curve (ii) mechanically and electrically induced long-latency
muscle stretch reflex (iii) reciprocal inhibition between antagonistic muscles (iv) brainstem
reflexes such as blink reflex (v) cortical SEP (vi) Bereitschaftpotential (BP) and Contingent
negative variation (CNV) and (viii) transcranial magnetic stimulation (TMS). TMS specifically
helps to study the excitatory and inhibitory changes in the brain in patients with dystonia and
may also be a useful tool to monitor therapeutic interventions.

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SY2.4. Sensori-motor Organisation in Focal Hand Dystonia

Mathew Alexander1
1Brunei Neuroscience Stroke and Rehabilitation Center, PJSC, Brunei

Focal Hand Dystonia is a task specific dystonia with co-contraction of agonists and
antagonistic muscles. This disorder is attributed to a plausible dysfunction of the basal ganglia,
but the exact role and sites are not yet well characterized.

At our institution, we did experiments’ to explore the ontogeny of muscle contraction, sensory
abnormalities and precision grip studies to explore dysfunction. The first study looked at the
utility of 16 Channel surface EMG using customized software to objectively assess FHD during
different phases of a writing task used to study timing, activation patterns and spread of
muscle contractions in FHD when compared to normal controls. SEMG of FHD subjects
showed ‘early onset’ during motor imagery, rapid proximal recruitment, co-contraction of
agonist-antagonist proximal muscle groups, delayed offset after stopping to write, mirror
activity in contralateral limb when compared to controls.

The second experiment aimed to look at the degree of sensory dysfunction in subjects with
Writer’s cramp using tasks for spatial discrimination and Aristotle illusory phenomenon and to
look for Neural correlates using f MRI. There were abnormalities of sensory processing in
patients with FHD in both spatial and illusory phenomenon. Spatial discriminatory abnormality
was see in both hands indicating abnormalities in bilateral somatosensory cortex leading to
abnormal sensory motor integration and resultant maladaptive plasticity and f MRI showed lack
of good separation of activity for index and little finger stimulation with JVP dome.

The third experiment looked at precision grip (both qualitative and quantitative measures) to
discriminate FHD and PD from normal subjects. Healthy controls and patients (PD/FHD)
gripped and lifted objects of 2 different weights;1.3 N &1.7 N with Dry Skin Condition and Very
Wet Skin Condition. After practice trials, subjects were asked to reach, lift and hold stationary
an object for 5 seconds and release the object on command. The force, rate and time
parameters are measured and a ROC curve was measured and this study could qualitatively
differentiate FHD and PD.

These studies highlight that in FHD there is motor and sensory processing abnormalities
demonstrating abnormal plasticity.

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SY2.5. Balance and Gait Assessment

Margaret Mak1
1Department of Rehabilitation Sciences, The Hong Kong Polytechnic University, Hong Kong

Postural instability and walking difficulties are the most disabling symptoms in Parkinson
disease (PD), leading to falls, physical injuries, functional decline and early institutionalization.
Medical, surgical and rehabilitation are the mainstay of management for balance and gait
disorders. Evaluation of balance and gait disorders is crucial to determine the problems of the
patients so that targeted treatment can be given. Postural instability manifests as reductions in
limits of stability, postural adjustment, and postural responses. Gait disorders comprise
reduced gait speed, short stride length, freezing of gait, and dual-task deficits. Postural
instability and gait disorders can be evaluated using reliable and validated clinical tests,
measurement scales, questionnaires and technological equipment. Objective clinical tests and
measurement scales require simple equipment and needs a short time to complete the tests.
Questionnaires are useful to reflect patients’ perceived balance and gait ability. Technological
equipment can be costly but they can increase the measurement sensitivity.

The clinical tests used to evaluate postural instability include retropulsion tests, Functional
reach test, and one-leg-stance test, whilst those for gait disorders include single or dual-task
walk test, timed-up-and-go-test, and 6-minute walk test. Measurement scales including Berg’s
balance scale, mini-Balance evaluation system test and Tinetti mobility test, are used to
assess overall balance and gait limitations.

Most of the measurement scales have cut-off scores for predicting fall risk. Validated
questionnaires such as Activities-specific balance confidence scale and Fall efficacy scale
evaluate one’s perceived level of balance confidence. Freezing-of-gait questionnaire
subjectively assess the severity of freezing of gait. Most of the technological equipment are
bulky, laboratory-based and may require a long time to complete the test and/or to analysis the
data.

Nowadays, body worn sensors is getting more popular because they allow measurement to be
taken anywhere including patients’ home and in the community. Algorithm has been built in
some systems to shorten the data analyzing time. There are many choices of outcome
measures and clinicians/researchers can choose the evaluation methods that is best for their
measurement purposes.

20
SY2.6. Eye Movement Disorders due to Brainstem Dysfunction

Ji-Soo Kim1
1Department of Neurology, Seoul National University College of Medicine, Seoul National

University Bundang Hospital, Seoul, South Korea

The brainstem contains numerous structures and their afferent and efferent fibers that are
involved in generation and control of eye movements. These structures give rise to distinct
patterns of abnormal eye movements when damaged. Thus, defining these ocular motor
abnormalities allows a topographic diagnosis of a lesion in various disorders involving the
brainstem. Even though diverse patterns of impaired eye movements may be observed in
lesions affecting anywhere along the brainstem, medullary lesions mostly present various
patterns of nystagmus and impaired vestibular eye movements without obvious
ophthalmoplegia. In contrast, pontine ophthalmoplegia is characterized by abnormal eye
movements in the horizontal plane while midbrain lesions typically show vertical
ophthalmoplegia in addition to pupillary and eyelid abnormalities. Recognition of the patterns
and characteristics of abnormal eye movements observed in brainstem lesions is important in
understanding the roles of each neural structure and circuit in ocular motor control as well as in
localizing the lesions by determining the involved structures.

SY2.7. Evaluation of the Vestibular System: cVEMP and oVEMP

James Colebatch1
1Prince of Wales Clinical School and Neuroscience Research Australia, University of New South

Wales, Sydney NSW 2052, Australia

The vestibular system makes strong connections with the oculomotor nuclei, the neck, the
cerebellum and also projects to the cortex. Over- or under-activity of vestibular afferents
causes disturbances of posture, vision and perception. While methods have been available for
some time for assessing some of the properties of the vestibular system, these have had
limitations. These methods have been difficult to apply widely, either being indirect and not
always well tolerated (rotating chairs, centrifuges and caloric testing) or poorly standardised,
such as galvanic stimulation in standing subjects.

Relatively simple methods of assessing short latency vestibular projections have become
available. The best known of these require averaging of muscle activity thus are termed
“vestibular evoked myogenic potentials” or VEMPs. Short latency potentials can be recorded
from the sternocleidomastoid muscle in the neck (cervical VEMPs) or from the inferior oblique
(ocular VEMPs). Both can be evoked by either air conducted sound (AC) or bone conducted
sound (BC). The AC-evoked cVEMP mainly reflects saccular function and the oVEMP mainly
utricular. Most recently it has been possible to record an evoked potential from the cerebellum
(VsCEP).

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The VEMP techniques are well tolerated and have been widely applied in vestibular diagnosis.
They have also been used in investigations of central disorders such as multiple sclerosis,
Parkinson’s disease and progressive supranuclear palsy (PSP).

SY2.8. Focal Botulinum Toxin Injections in Movement Disorders


Raymond L. Rosales1
1Department of Neurology and Psychiatry, University of Santo Tomas (UST) Hospital, Manila

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Symposium 3: Motor Neuron Disease

SY3.1. Monomelic Amyotrophy - Experience from a Large Cohort

Atchayaram Nalini1, Kiran Polavarapu1, Veeramani Preethish-Kumar1, Seena Vengalil1,


Saraswati Nashi1, Nupur Pruti2, Dhananjaya Bhat2, Alok Mohan Uppar2
1Department of Neurology and 2Neurosurgery, National Institute of Mental Health and

Neurosciences, Bengaluru, India

In 1959, Hirayama described the clinical entity of progressive atrophy of distal muscles of a
single upper limb and called it Hirayama disease (HD). Later on Gourie-Devi from India coined
the term Monomelic Amyotrophy. The other phenotypes described by us are: Distal Bimelic,
Proximo-distal bimelic and unilateral / bilateral proximal amyotrophy. Illness has sporadic
occurrence with male preponderance, but familial forms are also reported. Most cases are
described from Asian countries. There is insidious onset and slowly progressive weakness and
atrophy of intrinsic hand and forearm muscles, with oblique atrophy with sparing of
Brachioradialis and reverse split hand syndrome.

Typically, they have cold paresis. In the year 2000, HD was confirmed to occur secondary to
dynamic compression of the lower Cervical Cord due to forward displacement of the tight
cervical dural sac causing arterial / venous ischemic damage to anterior horn cells. Classically
MRI at cervical level shows “eye of the snake” appearance, reverse lordosis, dural detachment
with forward displacement and epidural venous congestion and enhancement. From our
experience on 480 cases seen over 38 years, the mean age at onset is 19.5 years; range,15-
25 years, with majority being in the progressive phase. Progression generally occurs over 5
years but even rapidly and slower in certain cases, and the disability is moderate to severe in
the majority. Rarely patients experienced Lhermitte’s phenomenon and had spasticity of the
lower limbs with sphincter disturbance.

Over the last 4.5 years, 101 cases with HD have undergone anterior cervical decompressive
surgery at our center. Predominantly C5-C6 single level discectomy and fusion was performed.
Pre-operative and serial post-operative assessment included, Fugl Meyer and Jebsen Taylor
hand function tests which demonstrated significant improvement in functions in the majority at
3,6,12 and 24 months follow-up. In the remaining patients the illness has stabilized. No major
complications were encountered except for post-operative neck pain and restricted movements
which improved in a few weeks. To conclude Hirayama disease is now confirmed to be a
cervical compressive myelopathy due to dynamic neck flexion induced ischemic damage and
surgery has a major role in stabilizing / improving the neurological deficits. The most
appropriate term for this disease would be “CERVICAL FLEXION INDUCED MYELOPATHY”.

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SY3.2. Neurophysiological Biomarkers in ALS

Steve Vucic1
1Western Clinical School, University of Sydney, Sydney, Australia

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the


motor neurons, characterized by upper motor neuron (UMN) and lower motor neuron (LMN)
dysfunction. There have been significant technological advances in the development of
neurophysiological biomarkers of UMN and LMN dysfunction in ALS. In this presentation, I will
discuss the major advances in development of neurophysiological biomarkers in ALS,
critiquing their potential in diagnosis and prognosis of ALS, as well as utility in monitoring
treatment effects.

The threshold tracking transcranial magnetic stimulation (TMS) technique has established
cortical hyperexcitability as an early and specific biomarker of UMN dysfunction in ALS and
associated with neurodegeneration. In addition to establishing cortical hyperexcitability as a
pathophysiological mechanism, threshold tracking TMS has enabled an earlier diagnosis of
ALS and provided a means of monitoring effects of therapeutic agents. Biomarkers of LMN
dysfunction, including motor unit number estimation, the neurophysiological and split hand
index, electrical impedance myography and axonal excitability techniques, all of which have
exhibited utility in monitoring disease progression and contributing to diagnosis.

In addition to enhancing ALS diagnosis, the development of novel neurophysiological


biomarkers has implications for clinical trials research and drug development, enabling the
assessment of biological efficacy of agents in early stages of drug development.

SY3.3. Role of Ultrasound in ALS

Yu-ichi Noto1
1Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of

Medicine, Kyoto, Japan

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder defined


through the involvement of upper and lower motor neuronal systems. The clinical and
neurophysiological investigation remains the gold standard in the diagnosis of ALS due to the
lack of a definitive biomarker.

Recently, a high-resolution ultrasound technique has been developed and is becoming popular
in the field of the diagnosis of neuromuscular diseases. Also in the diagnosis and monitoring of
ALS, neuromuscular ultrasound provides complementary information to clinical assessment
and neurophysiological testing as well as insights into the underlying pathophysiology of this
disease.

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Muscle ultrasound is used for detecting widespread fasciculations and assessing muscle
atrophy. Dynamic diaphragm thickness change can be measured by ultrasound and its change
correlates with respiratory function. Bulbar muscle ultrasound is also useful for the assessment
of bulbar dysfunction and dysphagia.

Nerve ultrasound can detect a reduction in cross-sectional area (CSA) and diameter of
peripheral nerves and nerve roots in patients with ALS, reflecting nerve atrophy due to motor
nerve axonal loss. These findings help us to distinguish ALS from mimicking diseases such as
multifocal motor neuropathy, in which increased nerve size is observed. In addition, nerve CSA
has a correlation with CSF level of progranulin which increases to maintain structure and
function of neurons.

Ultrasound examination is an operator-dependent and machine-dependent. However, the


neuromuscular ultrasound technique is easy to learn. To have a skill of neuromuscular
ultrasound is valuable to diagnose ALS accurately and to understand the pathophysiology of
ALS deeper.

SY3.4. Electrophysiological Characteristics of NMJ in Amytrophic Lateral


Sclerosis

Liying Cui1
1Peking Union Medical College Hospital, Beijing, China

ALS is characterized by degeneration of UMN and LMN. Patients experience progressive


muscle atrophy and weakness and problems with swallowing, which typically lead to
respiratory failure and death. Median survival time is about 3.5 from onset. So far, the
pathogenesis is not clear. More than 20 mutant genes have been found to relate ALS.
Diagnosis of ALS includes progressive course of disease, clinical features characterized by
degeneration of UMN and LMN and diffuse progressive and chronic denervation
simultaneously in EMG which is important in the early diagnosis.

In 1959, Mulder first discovered that ALS patient with muscle fatigable phenomena produced a
decremental response during low-frequency RNS, which indicated the possibility that NMJ was
involved in ALS. The accessory nerve which dominates proximal muscles shows the highest
positive rate while decremental pattern in low-frequency RNS for the facial nerve is rarely seen.
In different studies, the positive rate of RNS with low frequency stimulation. Positive rate of
decremental pattern in low-frequency RNS was higher in limb onset group than bulbar onset.
The decrement of low-frequency RNS in GMGs was a typical “U-shaped” decrement. But in
ALS patients, there was no significant recovery after the nadir of decrement.

25
SFEMG is regarded as a technique used in evaluating neuromuscular junction function and
significantly increases the sensitivity in the diagnosis of OMG. It is introduced by Prof. Stålberg
and Ekstedt in 60s. Remarkable increased jitter with block and increased FD are found in ALS
patients. It is useful for difficult cases in differential diagnosis.

The mechanisms of neuromuscular junction transfer in ALS is not clear. It may related to
immature NMJ, caused by progressive denervation and reinnervation of NMJ in new sprouts.
Wollman found that MEPP was decreased from intracellular recording of NMJ in ALS patients.
All of Ach release amount, the amount of immediately release quanta and the storage quanta
to resupply are decreased.

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Symposium 4: Neuromuscular Junction and Related Disorders

SY4.1. Myasthenia Gravis and Related Disorders

Donald B. Sanders1
1Duke University Medical Center, Durham, NC, USA

Autoimmune myasthenia gravis (MG) results from autoantibodies that bind to the
neuromuscular junction (NMJ). Incidence and prevalence vary worldwide; the estimated US
prevalence is 20/100,000, or ~60,000 patients. Women develop MG more often before age
40, men after 50. Drooping eyelids or double vision is the first symptom in 60%; weakness
remains limited to periocular muscles in 10-15% – ocular myasthenia. The voice may be nasal
or hoarse, especially after prolonged talking, and swallowed liquids may escape through the
nose. Weakness typically worsens throughout the day. Spontaneous improvement may occur
early on but is rarely permanent. The diagnosis depends on recognizing distinctive patterns of
weakness and confirmation by serologic and electrodiagnostic tests. 80-85% of patients have
circulating anti-AChR antibodies; repetitive nerve stimulation (RNS) demonstrates abnormal
neuromuscular transmission (NMT) in up to 90% of generalized MG and 60% of ocular
myasthenia. Increased jitter is found in almost all patients if appropriate muscles are
examined. Approximately 10% of patients have antibodies to muscle specific tyrosine kinase
(MuSK). Clinical findings resemble non-MuSK MG in some patients; many have predominant
weakness in cranio-bulbar muscles with marked atrophy. Others have predominant neck,
shoulder and respiratory weakness. With selected treatment most non-MuSK and MuSK-MG
patients have only minimal long-term morbidity.

Congenital myasthenic syndromes result from genetic defects of muscle endplate molecules
involved in NMT; most have autosomal recessive inheritance. The diagnosis is suggested by
clinical features and response to cholinesterase inhibitors, or electrodiagnostic findings.
Identification of specific genetic or physiologic defects requires genetic studies or specialized
morphological and electrophysiological studies.

Lambert-Eaton myasthenia results from antibodies to voltage-gated calcium channels (VGCC)


on presynaptic nerve terminals, which produces the classic triad of weakness, reduced muscle
stretch reflexes and autonomic dysfunction. Approximately 50% have an underlying
malignancy, usually small cell lung cancer. The diagnosis is confirmed by a decrementing
pattern during low frequency RNS and low muscle responses that increase at least 60% after
muscle activation. Antibodies to the VGCC are found in most cases. Treatment is initially
directed against any underlying malignancy. Amifampridine produces symptomatic
improvement in most patients; immunomodulatory therapy may provide modest benefit in
some.

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SY4.2. Seronegative Myasthenia Gravis: Auto-antibody Profiles and Clinical
Features
Yoon-Ho Hong1
1Seoul National University College of Medicine, Seoul Metropolitan Government Boramae

Medical Center, Seoul, South Korea

Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular


junction, caused in most patients by antibodies to the muscle nicotinic acetylcholine receptor
(AChR). There seem to be ethnic and regional differences in the frequency and clinical
features of MG seronegative for the AChR antibody. However, serological tests based on
novel assays and recently identified antigens are not available for routine clinical practice in
many regions where the overall frequency and features of seronegative MG according to
antibody have not been determined yet.

Recently, we performed a multi-centers study in South Korea to investigate the clinical features
and autoantibody profiles in adult patients seronegative for AChR antibodies. Serological tests
included the antibodies to MuSK (Muscle-specific tyrosine kinase), LRP4 (Lipoprotein receptor-
related protein 4), and clustered AChR, In this talk, an overview will be presented that covers
the results of our recent study and what is currently known on seronegative MG in Asia from
basic epidemiology and clinical features through to treatments.

SY4.3. Therapeutic Considerations in Myasthenia Gravis

Tomihiro Imai1
1Sapporo Medical University, Sapporo, Japan

Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by fatigable


muscle weakness. Treatment of MG has depended largely on high-dose corticosteroids for
decades. Although this procedure remarkably reduced the mortality rate of MG, it highlighted
issues such as the adverse effects of steroids and reduction in quality of life. A number of
alternative therapeutic options have become available. Disease-modifying therapies for MG
include immunosuppression with azathioprine, cyclosporine, or tacrolimus other than oral
prednisolone (PSL). More aggressive exacerbation of generalized MG often requires additional
immunomodulatory treatments with intravenous immunoglobulin (IVIg), plasma exchange
(PE)/plasmapheresis (PP) and steroid pulse therapy.

The Japanese clinical guidelines for MG were published in 2014, and they proposed a novel
treatment strategy utilizing these therapeutic options, which might replace high-dose steroids.
In the Japanese clinical guidelines, the first goal in MG treatment is minimal manifestations
(MM) with PSL 5 mg/day or below (MM-or-better-5mg). Immunotherapy should be initiated
from early stages in order to shorten the period of time during which MG symptoms interfere
with daily activities. One suggested strategy to increase success rate is reduction of oral

28
steroid dose and early initiation of calcineurin inhibitors, followed by fast-acting treatments
such as IVIg, PE/PP and steroid pulse therapy to resolve remaining symptoms quickly. Despite
the availability of an increasing variety of disease-modifying therapies for use in the new
strategy, the proportion of patients with generalized MG who achieved MM-or-better-5mg
remained approximately 50-60% in our latest survey. Therefore, we need further development
of new treatment to increase the success rate of generalized MG patients to the treatment
goal.

In this symposium, I will review positive clinical trials in the previous literatures and
guidance/guideline for management of MG in individual countries. In addition, I will introduce
further development of new treatment options based on novel mechanisms of action for MG
treatment. Especially, I will discuss monoclonal antibodies to prevent complement-mediated
damage at the endplate of neuromuscular junction in MG with acetylcholine receptor antibody
(Eculizumab, Ravulizumab and Zilucoplan) and neonatal Fc receptor antibodies which would
be rational therapeutic agents for decreasing the levels of pathogenic autoantibodies with IgG
isotypes (Efgartigimod and Rozanolixizumab).

29
Symposium 5: Intraoperative Neurophysiological Monitoring
(NIOM)

SY5.1. Intraoperative Neuromonitoring for Cerebrovascular Surgery

Kunihiko Kodama1
1Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan

Background and Objective


Cerebrovascular diseases are one of the most popular filed of neurosurgery. The most
frequent procedure is clipping surgery for intracranial aneurysms (ICAns). Arteriovenous
malformation (AVM) is also the lesion for which intraoperative neurmonitoring (IOM) play
important role as well as for carotid endoarterectomy (CEA) and carotid artery stenting (CAS).
Intraoperative neuromonitoring (IOM) plays indispensable role to reduce the surgical risks.
Here, IOM for these cerebrovascular surgeries is presented.
IOM of intracranial aneurysms clipping surgery

The IOM play a crucial role to preserve neuronal tracts such as corticospinal tract (CST). Motor
evoked potential (MEP) is the most sensitive modality for monitoring CST, especially for
anterior choroidal artery and lenticulostriate arteries. Somatosensory evoked potential (SSEP)
can detect ischemia in broader area of cerebral cortex than MEP. It is important to understand
each surgical step, so that neuromonitoring alteration can sometimes be anticipated.
Temporary occlusion of proximal parent artery is frequently used, and the safe obliteration time
varies case by case. IOM can advise on this issue. Obliteration of aneurysm and preserved
patency of the relevant vessels need be confirmed by supportive technique including IOM,
intraoperative video angiography and Doppler flowmetry.
IOM of AVM surgery

Surgery of AVM is one of the most complicated procedures in neurosurgery. Surgical risk is
calculated and classified by Spetzler-Martin grading system (nidus location: eloquent or non-
eloquent, size: <3cm, 3-6cm, >6cm, and draining system: superficial only or deep). Surgical
resection of the nidus is the primary goal for AVM treatment; however, multimodality treatment
strategy is considered for each case. IOM can detect ischemia of normal structure in
subcortical and cortical area to reduce surgical risk.

IOM in CEA and CAS


IOM includes EEG, SEP, MEP, near infra-red spectroscopy in CEA and CAS, and can detect
the ischemia during proximal carotid artery occlusion and may detect embolic ischemia. Some
pitfall exists, however, in each modality, so care need to be paid these aspects.

Conclusions
IOM in cerebrovascular surgery is reviewed. Sudden IOM alteration indicates ischemia of
neural structure, which might lead formidable consequence. The IOM plays a crucial role in
cerebrovascular surgery.

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SY5.2. Intraoperative Monitoring and Mapping during Brain Tumor Surgery

Tsui Fen Yang1


1Department of Physical Medicine and Rehabilitation, Taipei Veterans General Hospital, Taipei,

Taiwan

The aggressive removal of a glioma is expected to improve long-term patient survival and
quality of life. However, removal of a supratentorial glioma, particularly in proximity to the
eloquent cortex, poses a great risk of creating new functional deficits. Thus, it is critical to
consider functional and oncological outcomes simultaneously when treating patients with
supratentorial glioma.

Neurophysiological intraoperative monitoring (NIOM) has long been regarded as the “gold
standard” for glioma resection, allowing for maximal resection of lesions while preserving
sensorimotor function. NIOM during surgery involves the use of both mapping and monitoring
strategies with multiple modalities. Brain mapping is necessary due to individual variability in
cortical organization and the unexpected displacement of normal anatomic landmarks by tumor
growth. Typically, the median somatosensory evoked potential phase reversal technique is
used to localize the central sulcus, followed by direct cortical stimulation with a monopolar
electrode and multipulse train stimulation, to confirm the location of the eloquent motor cortex.
During resection, motor and somatosensory evoked potentials (MEP and SSEP) from the
contralateral hemibody and direct waves (d waves), if indicated, are monitored to safeguard
the integrity of functional structures. If resection extends beyond the cortical boundary,
subcortical mapping can be used to confirm the approximate distance from the resection cavity
to the corticospinal tract.

Concomitant use of subcortical mapping and MEP monitoring is believed to be associated with
more extensive resection and fewer severe late functional deficits. In addition, a meta-analysis
indicated that use of intraoperative brain mapping during glioma surgery resulted in a 2-fold
reduction of long term neurological deficits. Further, this was not at the expense of extent of
resection.

In conclusion, while functional deficits are still possible even with NIOM during the surgical
procedure, in most cases, the use of this technique significantly improves patient outcomes.

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SY5.3. IONM in Spine Deformity Surgery

Sandip Chatterjee1
1Park Clinic, India

SY5.4. Monitoring for Peripheral Nerve Surgery

Jorge E. Gutierrez1
1Potenciales Center of Electrodiagnosis, Cali, Colombia

Injuries or diseases of peripheral nerves can have severe impact on function, severe disability
(inability to work), social and psychological effects or chronic pain.
The main reason to perform Intraoperative monitoring (IOM) during peripheral nerve surgery
are to prevent nerve injuries during surgery, to identify peripheral nerves or fascicles in cases
of variable anatomy, scar tissue or tumors, to determine axon continuity across a lesion, and to
guide surgical decisions regarding the type of neural reconstruction to perform.

Nerve action potential (NAP) recording is considered a standard method for peripheral nerve
monitoring given its ability to determine the presence or absence of functional axons in a
segment of nerve, it also localizes functional and non-functional nerve segments. A NAP
recording requires 4.000 to 5.000 intact or regenerating axons to be obtained and its preferred
to compound muscle action potential (CMAP) recordings. The presence or absence of a NAP
is more important than its latency, amplitude or shape. If a NAP is recorded in a nerve or
fascicle, neurolysis is performed, and nerve grafts are required in no-responding fascicles.
Some technical difficulties of NAP recording are related to very short stimulation/recording
distances, stimulus artifact, volume conduction from another nerve, low temperature, use of
tourniquet and use of local anesthetics.

CMAP recording is like that used in triggered EMG, surface or subcutaneous needle
electrodes are placed over desired muscles and the exposed peripheral nerve is stimulated.

Free EMG is useful to detect, in real time, trains or bursts of abnormal discharges that indicate
possible nerve damage. It´s easy to use, not affected by anesthesia and has high sensitivity
but low specificity. Bipolar intramuscular electrodes are preferred for EMG recordings.

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SY5.5. IONM of Brainstem - Focus on Corticobulbar MEP and Brainstem
Reflexes
Kyung-Seok Park1
1Department of Neurology, Seoul National University Bundang Hospital, Seoul National

University College of Medicine, Seoul, Korea

Neurosurgery in the brainstem is one of the most challenging procedures and requires higher
level of intraoperative neurophysiological monitoring (IONM) methodologies to prevent severe
postoperative neurological deficits. Some of them are specialized for the monitoring of the
functional integrity of the brainstem during surgery. Among these, this talk will be specifically
focused on IONM of the corticobulbar motor evoked potential (CoMEP) and brainstem
reflexes.
CoMEP can monitor functional integrity of the corticobulbar motor pathway continuously from
the motor cortex to the cranial nerves. Also, brainstem reflexes can be reliable methodologies
to monitor neural structures involved in brainstem reflex arcs, both sensory and motor
pathways. These two methodologies can be promising and powerful to evaluate the functional
integrity of the brainstem and cranial nerves.

Key Words: intraoperative neurophysiological monitoring (IONM), brainstem, corticobulbar


motor evoked potential (CoMEP), brainstem reflexes

SY5.6. Intraoperative Neurophysiological Monitoring in Cervical Spine


Surgery
Yew Long Lo1
1National Neuroscience Institute, Singapore General Hospital

Intraoperative monitoring (IOM) has been utilized for surgery of degenerative, neoplastic and
traumatic causes of cervical spine disease. However, controversy still exits with regards to its
clinical efficacy. Most studies to date have analyzed spinal or scoliosis surgery, while few
focusing solely on cervical decompression are available.
Early studies have described good sensitivity and specificity in the IOM of cervical spondylosis
surgery with (Kim et al, 2007) or without myelopathy (Xu et al, 2011). However, this has been
challenged by 2 subsequent ones which (Ajiboye et al, 2017; 2017) contradict the earlier
findings. A systematic review conducted recently concluded that IOM is useful for detection of
intraoperative neural damage in cervical spine surgery involving degenerative canal stenosis
(de Martino et al, 2019).

IOM has been reported to be efficacious for early identification of intraoperative C5 palsy after
cervical myelopathy surgery, despite its rarity and cryptogenic nature.

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There is a current lack of systematic studies addressing IOM for cervical tumour surgery, and
further research is needed.

A recent study on motor evoked potentials (MEP) during IOM of cervical spondylotic
myelopathy suggested that a 9 pulse stimulation train may be more efficacious than a 5 pulse
train, whereby intraoperative improvement of MEP is more frequently observed in the former
(Lo et al, 2020). The new findings may be relevant for future research into false positive and
negatives during IOM of diseased spinal cords (Iorio, et al, 2015; Lee et al, 2016).

SY5.7. Neurophysiologic Intraoperative Monitoring for Pain Surgeries


Aatif M. Husain1
1Department of Neurology, Duke University Medical Center; Neuroscience Medicine, Duke

Clinical Research Institute; Neurodiagnostic Center, Veterans Affairs Medical Center, Durham,
North Carolina, USA

Pain is a very common symptom that is most often treated medically. However, when medical
treatment fails, surgical options for pain management become necessary. Many types of pain
surgeries involve destruction or modulation of various parts of the nervous system. In this
presentation, pain surgeries that have used neurophysiologic intraoperative monitoring (NIOM)
to help optimize outcomes will be discussed. Initially a brief description of pain pathways will
be presented. This will be followed by a brief description of a few of the common types of pain
surgeries and the type of NIOM that can be used in these procedures. Data for value of using
NIOM in these surgeries will also be presented.

34
Symposium 6. Epilepsy: Advanced EEG in Clinical Practice

SY6.1. Infraslow, DC Shift and High Frequency Oscillations: Established


Tool or Research Topic?
Akio Ikeda1
1Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate

School of Medicine, Shogoin, Sakyo-ku, Kyoto, Japan

Since 1990s digital EEG has been rapidly introduced and almost replaced analogue EEG, and
currently furthermore wide-band EEG is clinically available. It ranges from infraslow or DC
shifts (since 1990s) to high frequency oscillation (HFO) such as over 300Hz or even 1kHz
(since 2000s) depending on the degree of sampling rate. Therefore, currently clinical EEG may
belong to the 2nd generation of digital EEG as “wide-band EEG era”. Therefore, it is very
important to differentiate those digital EEG features as to whether they are already established
tools or still research topics.
1) Research topics can be divided into biological research topics. It includes infraslow or DC
shifts. Ictal infraslow or DC shifts has been extensively investigated as the research topics
in basic and clinical fields, and at least a part of them (invasive ictal DC shifts) is very close
to a tool (Nakatani et al, 2018). Among pathological HFO, interictal, invasive HFO is at least
partly regarded as a tool. Physiological HFO such as language and memory are actively
investigated as the research topics.
2) Mathematical EEG data analysis with clear hypothesis or condition is also regarded as
methodological research topics (3D source analysis, Granger causality analysis, etc).
3) Clinical tools are also listed as follows; quantitative EEG analysis such as frequency band
mapping, time-frequency analysis and voltage map. Those are strong tools for clinical
practice and for clinical research for CNS function and its pathological state.

In the clinical filed, we could often apply novel EEG technology as the research topic in the
clinical situation, and then often and usually immediately, the outcome, feedback or the degree
of clinical correlation is obtained. Therefore, nevertheless, we should be cautious not to
obscure the boundary between research topic and clinical tool until it is at least partly
established appropriately.

SY6.2. Source Dipole Modelling: Established Tool or Research Topic?


Yotin Chinvarun1
1Neurology Division, Department of Medicine, Phramongkutklao Royal Army Hospital and

Medical College, Bangkok, Thailand

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SY6.3. MEG in Epilepsy: Established Tool or Research Topic?

Shozo Tobimatsu1
1Department of Clinical Neurophysiology, Neurological Institute, Faculty of Medicine

Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan

Both electroencephalography (EEG) and magnetoencephalography (MEG) measure the same


underlying brain activities. The greatest advantage of MEG is that source estimation
techniques are easier to apply for MEG than for EEG. Thus, MEG is one of the best ways to
assess the brain activity in patients with epilepsy, because it is noninvasive and can be used
multiple times for the same patient. Magnetic source imaging (MSI) of interictal spikes as part
of presurgical evaluations is one of the most successful clinical applications of MEG. A single
tight cluster of MEG spike dipoles is well correlated with ictal onset zone.

Conventional EEG analysis is not always able to define the epileptogenic area. To this end, the
single-dipole analysis tools can resolve localized epileptic MEG discharges and demonstrate
equivalent current dipoles (ECDs) in cerebral cortex. Epileptic spikes arose from the mesial
temporal lobe epilepsy are sometimes not estimated by MSI. However, MEG-positive spikes
with EEG-negative spikes are often found by MEG. Jerk-locked back averaging of the
myoclonic discharges in a patient with epilepsia partialis continua is useful for the precise
location of the epileptogenic zone.

In this talk, an overview on MEG for epilepsy will be presented. Then, minimum norm
estimates and beamformer analysis will be introduced. These methods are sometimes
beneficial for determining the propagation of epileptic discharges and the epileptic activities
located in the deep structures.

I will also introduce the concept that hippocampal sclerosis modulates the central auditory
processing (CAP) in patients with mesial temporal lobe epilepsy (mTLE). CAP dysfunction
provides us information on the lateralization of mTLE. Especially, altered neural
synchronization may provide useful information about possible functional deterioration in
patients with unilateral mTLE.

In conclusion, MEG is useful for the diagnosis of epilepsy. It also provides us an insight into
the pathophysiology of epilepsy.

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SY6.4. EEG and fMRI recordings: Established Tool or Research Topic?

John Archer1
1The University of Melbourne, Austin Health, Florey Institute of Neuroscience and Mental Health;

Melbourne, Australia

EEG with functional MRI combines the spatial resolution of MRI with the temporal resolution of
EEG, to reveal an epileptic focus or network. It images blood flow changes in the brain in
response to the epileptic discharges of interest, providing a temporally smoothed, average
response to a series of epileptic discharges, displayed as a statistical ‘activation map’. Key
advantages of EEG-fMRI include that it is non-invasive and offers whole brain coverage,
allowing visualisation of deep epileptic foci, and distributed epilepsy networks. Group EEG-
fMRI studies have been particularly useful in revealing the brain networks responsible for
epileptic syndromes, such as Benign Epilepsy with Centro-Temporal spikes, Genetic
Generalised Epilepsy, and Lennox-Gastaut Syndrome, providing important mechanistic
insights.

When used as a tool for presurgical localisation in individuals, results have been mixed, with
around 50% of studies providing helpful localisation. Practical limitations include difficulty
detecting discharges due to the harsh recording environment of the MRI, sensitivity of fMRI to
patient motion and insufficient epileptic discharges during the fMRI session. Even with optimal
recordings some studies show no significant activation, possibly due to frequent epileptic
discharges that are too low in amplitude to penetrate the scalp, contaminating the ‘reference’
periods used in analysis.

Many EEG-fMRI studies show networks of brain regions are involved, rather than a single
region, frustrating interpretation when seeking a surgical target. However, these patterns likely
reflect real network recruitment by interictal discharges, and hence indicate the network
through which epilepsy is being expressed. There is some evidence that when there is a
network of spike related activation, seizure freedom rates are affected by the extent of removal
of the region of greatest fMRI activation. Even when not providing definitive localisation, EEG-
fMRI activation maps can provide a starting point for subsequent investigations such as
additional structural imaging, or invasive studies.

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SY6.5. Temporal Plus Epilepsies

Manjari Tripathi1
1All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Temporal plus epilepsies are characterized by the epileptogenic foci outside the temporal
lobe and a mix of temporal and extratemporal features. They have a wide spectrum
of semiological presentation depending on the extent of network. They can arise from
frontal, parietal, occipital lobes along with temporal lobe networks. We discuss in this review
the semiology of different types. Lateralizing and localizing features are mentioned in the
table below the review is not exhaustive and it is recommended to read large descriptive
semiology’s in journals. Temporal plus epilepsies will have red flags for temporal lobe in that
there will be extratemporal auras or signs. These would suggest wider networks more than
temporal. So if a typical temporal lobe seizure has amnesia, arrest, lack of awareness,
aphasia, etc. Presence of a visual aura would favor a temporal plus network.

Seizure Sub type Symptomatogenic zone Lateralization Epilepsy


type syndrome
Auras Somatosensory Primary somatosensory CL PLE
cortex (areas 1,2 and 3b)
Secondary
somatosensory areas
(parietal operculum/ssb)
SSMA
Simple Primary visual cortex CL OLE
(areas t7, t8 and t9)
Complex visual Temporo- CL OLE
occipital junction
Olfactory Orbitofrontal region NonLAT FLE
Fear Ventnomedial prefrontal, NonLAT FLE
mesial frontal lobe
Multisensorial Temporo-parieto- NonLAT FLE
occipital junction
Cephalic/whole body SSMA NonLAT FLE
Myoclonic/negative Primary motor cortex ( CL (if FLE
area 4) and premotor unilateral)
Myoclonus Cortex (area 6)/ primary
somatosensory area
Simple Clonic Primary motor cortex, CL FLE
motor premotor cortex, and
SSMA
Tonic Primary motor CL (If FLE
cortex and SSMA unilateral)

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Hypermotor Anterior NonLAT FLE
cingulum, mesiofrontal,
orbitofrontal
Complex Automotor Anterior cingulum NonLAT FLE
motor
Gelastic Hypothalamus, NonLAT FLE
anteromesial frontal
region
Mesial frontal, cingulum, NonLAT FLE
intermediate frontal
(area 8)
Autonomic Tachycardia/hyperventilation Insula, anterior NonLAT FLE
cingulum, and medial
prefrontal cortex

CL = Contralateral, IL = ipsilateral, NonLAT = Nonlateralizing, FLE = Frontal lobe epilepsy, PLE = Parietal lobe epilepsy, OLE = Occipital lobe epilepsy, SSMA
= Supplementary sensorimotor area, IPSI = Ipsilateral

SY6.6. Parietal Lobe Epilepsy


Chong Wong1
1Staff Specialist Neurologist, Westmead Hospital and The Children’s Hospital at Westmead

Parietal lobe plays a key role in higher sensory, perceptual and cognitive processing. It is
highly integrated and interconnected to multiple brain regions. Although parietal lobe epilepsy
is uncommon, parietal seizures are varied and complex reflecting spread and overshadowing
the focal origin. In this session, I will review the literature and the parietal lobe surgical patients
at Westmead Hospitals and discuss the semiology and investigations in parietal lobe epilepsy.

SY6.7. "Multiple Lesions": Seizures from Cytoarchitectonically Different


Subdivisions of the Cerebral Cortex

Sinclair Liu1
1Shanghai Deji Hospital Comprehensive Epilepsy Center, Qingdao University Affiliated,

Shanghai, People’s Republic of China

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Symposium 7: Peripheral Neuropathy

SY7.1. Inflammatory Neuropathies and Diabetes

Yusuf Rajabally1
1University Hospitals Birmingham & Aston University, Birmingham, United Kingdom

Inflammatory neuropathies may occur with diverse presentations in subjects with diabetes.
Separating these neuropathies from typical diabetic polyneuropathy is important as prognosis
and treatment differ. Diabetic lumbosacral radiculoplexus neuropathy and diabetic cervical
radiculoplexus neuropathy are common and need to be readily recognized as require pain
relief but no immunotherapy, and as are of generally good prognosis. Prompt diagnosis is
important in vasculitic neuropathy in diabetes which requires similar diagnostic and treatment
strategies as in non-diabetic subjects. Chronic inflammatory demyelinating polyneuropathy
(CIDP) in diabetes has been the subject of controversy and uncertainty in recent years with
divergent results from different non-population based and epidemiological studies. Some
suggested an increased risk of CIDP with diabetes, while others did not. The clinical features
take priority when considering CIDP in diabetic patients. Electrophysiology, although helpful for
diagnostic confirmation, may however in practice pose problems with regards to technique and
interpretation. This may result both in under- and overdiagnosis of CIDP in subjects with
underlying diabetic polyneuropathy. Recent studies from different the U.K., Serbia and Italy, of
650 subjects with “definite” or “probable” CIDP, appear to confirm a higher prevalence of
diabetes in CIDP populations, which is of later onset compared to subjects without diabetes.
Data from these recent studies also suggest typical CIDP may be the predominant form in
diabetic subjects, with however persistent uncertainty remaining with regards to disability
levels and treatment response compared to subjects without diabetes. This implies that
treatment strategies for subjects with CIDP and diabetes should not differ from those in
subjects without diabetes, except for precautionary measures with regards diabetic control and
vascular risk.

SY7.2. Electrophysiological Features in GBS and CIDP

Satoshi Kuwabara1
1Chiba University, Chiba, Japan

GBS is currently classified into axonal and demyelinating categories. Acute motor axonal
neuropathy (AMAN), an axonal subtype of GBS, was widely recognized in the 1990’s.
Whereas in Europe and North America, acute inflammatory demyelinating polyneuropathy
(AIDP) is considered the major subtype of GBS, in East Asia, and Central and South America,
AMAN is found for 30~80% of GBS cases. Over the past 20 years, major advances have been

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made in understanding the immunopathogenesis and pathophysiology of AMAN. Clinically,
AMAN is characterized by pure motor involvement, frequent antecedent Campylobacter jejuni
enteritis, and serum anti-ganglioside antibodies. Electrophysiological studies frequently reveal,
as well as axonal degeneration, rapidly reversible nerve conduction block/slowing (resolved
within days to a few weeks); the time-course suggests functional or microstructural changes at
the nodes of Ranvier. It is now established that disrupted nodal sodium channel clusters, and
paranodal myelin detachment is responsible for conduction block in AMAN.

CIDP is currently classified into “typical CIDP” and other variants such as “multifocal acquired
demyelinating sensory and motor neuropathy (MADSAM)”. Typical CIDP is a classic form,
clinically characterized by symmetric proximal and distal muscle weakness and motor-
dominant manifestation. In typical CIDP, demyelination predominantly affects the distal nerve
terminals and nerve roots, presumably because of the lack of the blood-nerve barrier in these
regions, and this could be responsible for the “non-nerve length-dependent” distribution of
muscle weakness. These findings suggest an importance of humoral immunity in typical CIDP.

In contrast, MADSAM is characterized by multifocal demyelination in the intermediate nerve


trunks. In MADSAM neuropathy, cellular immunity may be predominantly involved in
breakdown of the blood-nerve barrier at the site of conduction block. Among the CIDP
spectrum, typical CIDP and MADSAM are the major subtypes, and their pathophysiology
appears to be distinct. The understanding of the pathophysiology of each CIDP subtype is
important for appropriate immune treatments.

SY7.3. Evaluation of Small Fibre Neuropathy and Therapeutic Progress


Sung-Tsang Hsieh1
1National Taiwan University, Taipei, Taiwan

Small fiber neuropathy causes 2 major symptoms: (1) reduced sensation to painful and
thermal stimuli due to a loss-of-function deficits and (2) neuropathic pain attributed to a gain-of-
function hyperexcitability. Over the last 2 decades, there have been substantial progresses in
terms of evaluation and therapy, in particular, a comprehensive and integrated assessment
platform to examine the underlying pathology, physiology, and neuroimaging for the negative
and positive sensory symptoms. These include skin biopsy for the pathology of nociceptive
nerve terminal degeneration, corneal confocal microscopy for quantifying corneal nerves,
evoked potential activated with laser, contact heat, and electric stimulations for the physiology
of small-diameter nociceptive nerves, quantitative sensory testing for the psychophysics of
thermal sensation, and nociception-activated functional MRI for the brain perception.

Various phenotypes of small fiber neuropathy can be objectively and quantitatively evaluated.
During the recent decade, the exploration of etiology for small fiber neuropathy has yield new
molecular and therapeutic targets, in particular, sodium channel mutations in small fiber

41
neuropathy. The presentation will review and summarize the current status of evaluation and
therapy of small fiber neuropathy.

SY7.4. Evaluating Peripheral Neuropathy In Resource Poor Settings


Thirugnanam Umapathi1
1National Neuroscience Institute, Singapore

The earth is neither flat nor round, but rather a pyramid with an apex occupied by the top 10%.
At the base are 4 billion people making <8 USD/day. Business, economies and, by
extrapolation, medical activities largely cater to the upper sections of the pyramid. The
circumstances at the bottom are unique; and the strategies employed in the apex seldom work
for the base. Using illustrative cases, I shall discuss the key lessons I have gleaned from my
limited exposure to peripheral neurology in resource poor settings.
“These unhappy times call for the building of plans that rest upon the forgotten, ...that put faith
once more in the forgotten man at the bottom of the economic pyramid” – Franklin D
Roosevelt.

SY7.5. Diagnosis and Treatment of Paraproteinemic Neuropathies


Sonoko Misawa1
1Chiba University School of Medicine, Chiba, Japan

There are several neuropathies associated with paraproteinemia: AL-amyloidosis, anti-myelin


associated glycoprotein (MAG) neuropathy, and POEMS syndrome. They can be sometimes
diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), but their therapeutic
strategies are clearly different from those for CIDP. Appropriate diagnosis and early treatment
are essential to improve prognosis of paraproteinemic neuropathies.

AL-amyloidosis is a systemic and fatal disorder associated with plasma cell dyscrasia. Its
neuropathy is characterized by length-dependent axonal polyneuropathy and autonomic
neuropathy. Because diffuse mild conduction slowing and/or prolonged distal latency in the
median nerve, which is caused by deposition of amyloid in the carpal tunnel, are sometimes
found, AL-amyloidosis can be initially diagnosed as CIDP. Therapeutic approach to myeloma
with autologous stem-cell transplantation or proteasome inhibitors can be effective. AL-
amyloidosis usually deteriorates subacutely and delay in starting treatment can progress to
fatal outcome.

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Similar to AL-amyloidosis, POEMS syndrome is a systemic and fatal disorder based on
monoclonal plasma cell proliferation. POEMS neuropathy can be summarized as diffusely
distributed demyelinating polyneuropathy and it can be frequently diagnosed as CIDP. It is not
usually associated with dysautonomia. Disease progression leads to multiple organ failure and
long-lasting neurological sequelae. Early therapeutic intervention is a key to improve its
prognosis. As with AL-amyloidosis, treatments for myeloma can be used for POEMS syndrome.
The efficacy of autologous stem-cell transplantation, immunomodulatory drugs, and
proteasome inhibitors have been shown so far.

Anti-MAG neuropathy is accompanied by IgM monoclonal gammopathy. Distal dominant


demyelinating neuropathy is a common feature of its neuropathy. Anti-MAG neuropathy is
often refractory to conventional treatment such as steroid and immunoglobulin. Rituximab
might be effective in some cases.

SY7.6. HIV Associated Neuropathy in the Non-Stavudine Era


Fitri Octaviana1
1Neurology Department, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo

Hospital

HIV-associated sensory neuropathy (HIV-SN) remains common in HIV patients receiving anti-
retroviral therapy (ART). Neuropathies resulting from HIV infection itself and toxic side effects
of ART have similar presentations and include neuropathic pain, tingling and numbness. Some
nucleoside analogue reverse transcriptase inhibitors used to treat HIV were strongly
associated with HIV-SN. The prevalence of HIV-SN in patients receiving stavudine at Cipto
Mangunkusumo Hospital was 34% and had strong association with age and height. As
stavudine has been phased out recently, some patients still have HIV-SN. In 2016, the
prevalence of HIV-SN had been significantly reduced became 14.2% and associated factors
was viral load >500 copies HIV RNA/mL. It is an evidence that lack of stavudine exposure is
central to the lower neuropathy prevalence.

One of clinical manifestation of HIV-SN is neuropathic pain. Neuropathic pain can be found as
solely symptom without other sensory neuropathy symptoms (numbness) and signs
(decreased physiological reflex and vibration). Prevalence of neuropathic pain (NP) of HIV
patients was 6.6% at Cipto Mangunkusumo Hospital, smaller than HIV-SN. Our study showed
that HIV patients with NP had shorter duration of ART than patients with HIV-SN, and may be
an early symptom of small fiber neuropathy in HIV patients.

Some evidences indicate that HIV-SN is immune-mediated. Intra-epidermal fiber density of


HIV-SN patients is lower than HIV-noSN and healthy control. Low fiber density in HIV-SN
seems to be corelated with low nadir CD4+ counts. CD14+ macrophages were evident at

43
surrounding intra-epidermal nerve of HIV-SN and HIV-noSN patients. Some chemokine
receptors such as CX3CR1, CCR2 and CCR5 was more common in HIV-SN patients. These
evidences showed that inflammatory macrophages expressing chemokine receptors may
participate in peripheral nerve damage leading to HIV-SN in HIV patients treated without
stavudine.

SY7.7. Neuropathy due to Nutritional Deficiencies


Fu-Liong Hiew1
1Neurology Department, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia

Populations vulnerable for nutritional deficiencies are at risk for developing neuromuscular
complications. Patients with diseases causing malabsorption, elderly, chronic alcohol users
and women in pregnancy are recognized as high risk groups. Besides, displaced populations,
frequently impoverished, similarly have high prevalence of neurological disorders. In majority
of the neurology manifestations associated with nutritional deficiency, neurological damage is
reversible. Therefore, prompt identification and treatment are important. The focus of this talk
is on neuromuscular presentation of a subgroup of refugee population seen in Malaysia
associated with nutritional deficiencies. Clinical manifestation ranges from mild peripheral
neuropathy to overt encephalopathy and in some, respiratory failure. We discuss challenges
and strategies in management from early identification of symptoms to engagement of relevant
authorities.

44
Workshops (WS)
Workshop 1: Electroencephalogram (EEG)

WS1.1. The Origin of EEG, Recording Techniques and Quality

John Dunne1
1School of Medicine, University of Western Australia, Perth, Western Australia

EEG records the electrical activity of the cerebral cortex, mainly from large pyramidal neurons
close to the surface, and this activity is influenced by subcortical structures. At least 6 cm2 of
synchronised cortical activity is required to be recorded by scalp EEG.
Surface electrodes are placed equidistantly over the scalp according to an international
convention (10–20 System), ensuring good electrical contact, with electrode impedances
<5000 ohms. Each electrode is influenced by both physiologically relevant activity and high
amplitude environmental electrical noise, so pairs of electrodes are connected to differential
(common-mode rejection) amplifiers to hopefully cancel out the environmental noise and
record any differences between the two electrodes.

Digital EEG amplifiers then use analogue-to-digital converters to translate the recorded voltage
differences into numerical values, requiring an amplitude resolution of at least 12 bits (usually
≥16 bits), and with the number of measurements per second (sampling rate) at least three
times the highest frequency being recorded (≥256Hz).
Filtering highlights activity that is biologically relevant, using a bandpass of 0.5–70 Hz for
routine diagnostic EEG, but filtering distorts raw data and is no substitute for good technique.

Digital EEG recording uses a system reference electrode separate from the 10-20 recording
electrodes and stores the voltage measurements for each recording electrode relative to this
system reference electrode. This allows reformatting of EEG channels and montages after the
recording simply by combining any electrode pair to create the channel. At least longitudinal
bipolar, transverse bipolar and referential montages should be used to read EEG.

Unfortunately, EEG is commonly misinterpreted, leading to misdiagnosis. Age and state


dependency dramatically influence the normal EEG, and there are many variations of normal,
especially during drowsiness and sleep. ECG, careful clinical observation and video recording
(routine if possible) are required throughout the recording to recognise artefacts and other
important changes.

For EEG to be useful, comprehensive knowledge, training and experience are required under
the supervision of a skilled mentor. When in doubt, it is best to assume that background
slowing is related to drowsiness/sleep, and every sharply contoured wave is an artefact or
normal variant until proven otherwise.

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WS1.2. Normal EEG: Awake (Children and Adults), Including Activation
Procedures

Chong-Tin Tan1
1University of Malaya, Malaysia

The normal EEG pattern in adult and children during awake tracing, and the typical changes
during activation procedure are well known and are easily available in the various EEG
textbooks. In the clinical reading of EEG, the common reasons of false reading are failure to
appreciate the range of normality, artefacts and normal variants. The skills to different these
“normal” EEG often require guided reading of hundreds of EEGs. The examples for range of
normality as masquerade are the alpha slowing and wide distribution during drowsiness,
temporal theta seen in the elderly, posterior slow wave of youth and hypnopompic
hypersynchrony seen in the young. The next group of misreading is to mistake biological
artefacts as cerebral activities. Examples are eye flutter for frontal slow waves; ectopic ECG,
lateral rectus spikes and lambda waves for interictal spikes and sharp waves. The non-
physiological artefacts from mechanical (patting, pulse, respiration), environment, electrode
and recording equipment are also common masquerade. The normal variants present with a
rhythmic pattern or an epileptiform morphology. The common examples are wicket spikes, and
14 & 6 Hz positive bursts.

WS1.3. Normal EEG: Drowsiness and Sleep, and Normal Variants

Manjari Tripathi1
1All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

The EEG in the normal awake child and adult is well known and needs to be known for proper
reading and reporting and identifying an abnormal transient or pattern. The following are points
of emphasis: Alpha rhythm seen after age of 5 years, Significant theta activity (4 to 7 Hz) is
present in the EEG of children and adolescents. Delta activity in the awake tracing is rarely
seen after the age of 5 years. Beta rhythm is seen anteriorly. All these will be discussed in
details with traces. In the evaluation of abnormalities in the EEG, one needs to be constantly
aware that there are many EEG transients that morphologically resemble epileptiform
discharges and that need to be distinguished from diagnostically crucial epileptiform
abnormalities to avoid overdiagnosis or misdiagnosis. These include: Artifacts: for example,
electrode pop, muscle potentials, eye movements, electrocardiogram (EKG), chewing,
electrical etc.

46
Normal components of ongoing background activity: for example, vertex sharp transients of
sleep, f waves, slow wave sleep, POSTs, mu rhythm, lambda waves, drowsy activity during
sleep in children that may often be associated with sharp components, etc.
Epileptiform variants of dubious clinical significance: there are a large number of benign
epileptiform variants that must be recognized, lest they be misinterpreted. Although
morphologically similar, they are nonepileptogenic as they have no established relationship
with the process responsible for generating epileptic seizures. Such sharp transients include
14 to 6 per second positive spikes, small sharp spikes or benign epileptiform transients of
sleep, 6 Hz spike wave or phantom spike wave, wicket spikes, psychomotor variant pattern or
rhythmic midtemporal discharges, breach rhythm, etc. Sleep not only activates diagnostically
useful epileptiform EEG patterns, but also unmasks several types
of nonepileptogenic sharp transients.

It is necessary that anyone dedicating a career in epilepsy be well aware of these. Often
reading EEG requires eyeballing and identifying patterns. The more the number of EEGs
reviewed the more the possibility of not missing out normal patterns and rhythms. These will be
demonstrated in various EEG epochs in the workshop.

WS1.4. Generalized Epileptiform Patterns – Interictal and Ictal: Defining the


Clinical Syndrome

Raymond Azman Ali1


1National University of Malaysia Medical Centre, Kuala Lumpur, Malaysia

Generalised epileptiform patterns (GEPs) are bilateral and synchronous epileptiform


discharges that appear over corresponding parts of both hemispheres, or the whole brain.
Their morphology, amplitude and timing should be the same or similar over both hemispheres.
The distinction between ictal and interictal GEPs is generally less clear compared with focal
patterns. Ictal patterns often represent longer repetitions of interictal patterns. Clinical signs are
more likely to be apparent during GEPs if the bursts are longer, the spike component is more
persistent, and tests of responsiveness are adequately and repeatedly tested by the EEG
technician. GEPs include 3Hz spike-wave discharges, polyspike-wave discharges, slow spike-
wave discharges, generalised paroxysmal fast activity, electrodecremental response,
photosensitive responses and ictal patterns. 3Hz spike and wave complexes are characteristic
of idiopathic (genetic) generalised epilepsy, which includes childhood absence, juvenile
absence and juvenile myoclonic epilepsy as well as epilepsy with generalised tonic-clonic
seizures only. In children with 3Hz spike and wave, spontaneous remission is more likely if
there is co-existent OIRDA and less likely if there is co-existent photoparoxysmal responses.

Polyspike and wave complexes, either single or repetitive, can be seen in both idiopathic
(typically in JME) and symptomatic generalised epilepsies. Slow spike and wave complexes

47
occur at a frequency of 1.5-2.5 Hz and usually arise from an abnormal background; they, along
with generalised paroxysmal fast activity (GPFA-bilaterally synchronous10-25 Hz activity) are
typically seen in symptomatic and cryptogenic generalised epilepsies, notably Lennox-Gastaut
syndrome. GPFA is typically seen in tonic seizures, but may also accompany atonic and
generalised tonic-clonic seizures. Electrodecremental responses are seen in the atonic and
tonic seizures of symptomatic and cryptogenic generalised epilepsies. Photoparoxysmal
responses are bursts of generalised spike or polyspike and wave discharges that appear at
certain flash frequencies and may outlast the stimulus.

WS1.5. Focal Epileptiform Patterns – Interictal: Utility and Limitations

Yotin Chinvarun1
1Neurology Division, Department of Medicine, Phramongkutklao Royal Army Hospital and

Medical College, Bangkok, Thailand

WS1.6. Focal-Onset Seizures: Surface Ictal EEG Recordings and


Localization

Byung In Lee1, MD
1Department of Neurology, Inje University Haeundae Paik Hospital, Busan, Korea

Ictal EEG can be identified by sustained electrographic discharges showing onsets and offsets
being clearly distinguished from background rhythm, consisting of rhythmic discharges of
variable frequencies and morphology showing gradual variations in frequency and amplitude.
Morphology of ictal onset patterns in focal epilepsies are quite diverse and varies even in a
same patient. For localizing purposes, onset patterns of ictal EEG are most important than
following ictal discharges and they are topographically divided into focal, regional, lateralized,
and diffuse.

From the electrophysiological point of view, scalp ictal EEG in focal epilepsy requires a source
area of synchronized EEG activities spreading over at least 10 cm2, indicating that an ictal
onset usually reflects already spreaded activities from the EZ, thus the localization of EZ
should not be based upon ictal EEG onset alone but should require its correlation with other
investigations. Considering that scalp EEG reflects electrical activities from the cortical surface,
ictal discharges arising from the EZ locating in deep sulci or deep cortical structures are not
readily picked up by scalp EEG until they spread a significant distance to the cortical surface

48
under the recording electrodes. Another caveat of ictal EEG is that the degree of
synchronization is difficult to recognize from the surface EEG. In cases of poor synchrony,
scalp EEG may not detect any clear focal or regional discharges but showing widely spread
arrhythmic activities, thus fail to carry any localizing information.

The lateralizing value of ictal scalp EEG seem to be higher in temporal lobe epilepsy than
extratemporal lobe epilepsy, which were reported 76% to 83% and 47% to 65%, respectively.
The probability of false lateralization was around 1% to 7% with remaining proportion of ictal
EEG being unhelpful due to contamination by severe artifacts or diffuse discharges without
focal features.

The source localization of ictal onset rhythm by high-density EEG is an emerging technique
that seems to perform similarly well to the source localization of interictal spike, which has
been proved in large patient cohorts to be accurate and clinically relevant, with positive and
negative predictive values rivaling those of structural MRI, although there remain questions
regarding the processing of signals for reliable results. A further promising development in
seizure onset localization is the inclusion of directed connectivity analysis: thus, not only the
most active source is taken into consideration when localizing the onset of a seizure, but also
how strongly each source broadcasts its activity to other brain regions (the so-called outgoing
connections). Such an approach was shown to be more accurate than merely considering the
most active source.

WS1.7. Localization of Seizures – Seizure Semiology

Chong Wong1
1Staff Specialist Neurologist, Westmead Hospital and The Children’s Hospital at Westmead

Seizure semiology is the first step in understanding a seizure disorder. Careful clinical
observation of the epileptic seizures provides valuable clues of the brain region from which the
seizures are arising and guide subsequent investigations. This talk will discuss important
semiologic characteristics of various seizures for lateralising and localising epileptic zones.

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WS1.8. Invasive Monitoring: Understanding the Chronological Occurrence
of Seizure Semiology

Sinclair Liu1
1Shanghai Deji Hospital Comprehensive Epilepsy Center, Qingdao University Affiliated,

Shanghai, People’s Republic of China

The percentage of patients considered for epilepsy surgery in need for invasive evaluation
ranges 30% and 40% in tertiary epilepsy centers (Kovac, et. al., 2017). It will become
unavoidable when mapping epileptogenic zone for the patients with “MRI-negative” study and
when mapping eloquent cortex for the patients subject to functional deficit following resection
surgery.

Invasive evaluation includes various type of intracranial electrodes, such as stereotactically


implanted intracerebral (depth) electrodes (SEEG), subdural electrodes (SD-EEG) and
intraoperative electrocorticography (ECoG).

Stereoelectroencephalography (SEEG) was designed and developed by Talairach and


Bancaud in France in 1960s. It is an invasive method of exploration for drug-resistant focal
epilepsies, offering the advantage of a tridimensional and temporally precise study of the
epileptic discharges (Isnard, et al., 2018). Differently from SD-EEG in part, it does not need
craniotomy.

The basic principle of Bancaud and Talairach’s approach to epilepsy surgery was to study the
seizures themselves, through what they named the “anatomo-electro-clinical correlations”
(Kahane, 2006).

A group of selective cases, studied with assistance of SEEG for refractory focal epilepsies, will
be presented in the workshop to illustrate significance of the anatomo-electro-clinical
correlation approach. All the cases had motor seizures which generated respectively from
allocortical and isocortical subdivisions of the frontal, temporal and parietal lobes. The
different systems of hierarchical organization activated during seizures gave rise to the
chronology of semiology dissimilarly. It is more likely for the complex motor behaviors (i.e.
automatism) to occur if the motor seizures generate from phylogenetically older or less
differentiated cerebral cortex (i.e. histologically defined allocortex). On the other hand, the
more phylogenetically recent or the more differentiated cortex (i.e. histologically defined
isocortex), from which the motor seizures generate, the more likely the simple motor (i.e. tonic
or tonic-clonic) signs develop.

This is to imply that meaningful categorization of motor seizures in terms of semiology is


possible and correlates with anatomic organization along a histological differentiation, in
keeping with current hypotheses of hierarchical organization in the human brain.

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WS1.9. Advances in EEG Analysis – Wide-Band EEG, Dense-Array EEG and
Quantitative EEG

Akio Ikeda1
1Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate

School of Medicine, Shogoin, Sakyo-ku, Kyoto, Japan

1) Wide-band EEG
As the faster frequency extreme of wide-band EEG, high frequency oscillation (HFO) was
originally described by means of 1) micro-electrode recording in animal model, and ictal HFO
occurred before clinical onset. Recently, 2 more types of HFO were well described; 2) invasive
electrodes recording in epilepsy surgery (macro-invasive electrodes such as depth-, subdural
ones), and 3) scalp electrodes or MEG in epilepsy patients. The multiple mechanisms of HFO
generation are considered such as synchronized post-synaptic potentials with sparse
pyramidal cell firing, or principal cell action potentials.
As the lower frequency of extreme of baseline shifts in the chronic epilepsy focus on epilepsy
patients, ictal DC shifts occurred earlier than or as early as ictal HFO significantly (Imamura et
al, 2011; Kanazawa et al, 2015). It was newly endorsed by the pathological abnormality, i.e.,
decreased Kir4.1, selectively seen in the cortices generating ictal DC shifts (Kobayashi et al,
2017). It could suggest more active role of glia before seizure generation, and thus called as
active DC shifts.
These both findings could represent activity of both epileptic neurons and astrocytes,
respectively, mainly as the local field potentials.

2) Quantitative EEG
Once digital EEG era has started since 1989, first generation EEG (1989-1996/2000) could
provide us with data acquisition of ordinary sampling rate (i.e., 500 Hz) and band pass filter
(i.e., 0.5 Hz - 200 Hz). Data display has been much flexible by means of reformat of montage,
filter change, sensitivity change, voltage mapping, power spectrum analysis, and 3D source
analysis, and so on. It is user friendly, and regarded as the established tool or method.
Second generation EEG (1996/2000-present) has been much advanced and complicated tool
such as data acquisition such as wide-band EEG, i.e., 0.016 Hz - 600 Hz. It contains DC shifts,
infraslow, HFO. Increased sampling rate, i.e., 2000 Hz could widen the extremely faster
activitty. Data display such as wide-band display, i.e., 100 ms/page to 5 min/page, time
frequency analysis, DSA (Density Modulated Spectral Array), and Ganger causality further led
us to the interactive sophisticated circumstances in the clinical and scientific field.

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3) Dense-array EEG
The more electrodes are placed on the scalp, the better spatial resolution is expected. Once it
also could cover the so-called “south hemispheres” of the head, it could delineate the any
activity arising from the basal temporal and mesial temporal areas. However, since scalp EEG
is attenuated in amplitude and is blurred with wider distribution by means of cortical bone and
different soft tissues, the degree of localization map may not be proportionally precise in spite
of involvement of many electrodes. In this regard, whole MEG data may overcome of the 2
important factors.

WS1.10. EEG in the ICU: Indications and Recording Techniques

Aatif M. Husain1
1Department of Neurology, Duke University Medical Center; Neuroscience Medicine, Duke

Clinical Research Institute; Neurodiagnostic Center, Veterans Affairs Medical Center, Durham,
North Carolina, USA

In the last two decades much has been learned about the frequency with which seizures occur
in critically ill patients. The use of continuous EEG (cEEG) monitoring in the intensive care unit
(ICU) has made it possible to detect seizures in these patients. Many studies have shown that
up to 20-30% of critically ill patients may have seizures or be in status epilepticus. In the
majority of cases, these are nonconvulsive, making their detection without EEG impossible. In
this presentation, the rationale for monitoring critically ill patients will be discussed. CEEG
monitoring utilizes a lot of resources, and a discussion about the duration of monitoring for
each patient will also be presented. The selection of patients who undergo cEEG monitoring is
important as well, and this will be discussed. Finally, a brief summary of the types of
abnormalities that can be seen with cEEG monitoring will be presented.

WS1.11. EEG in the ICU: Status Epilepticus and the Ictal-Interictal


Continuum

John Dunne1
1School of Medicine, University of Western Australia, Perth, Western Australia

Epileptic seizures and nonconvulsive status (NCSE) are common in the critically ill, but non-
epileptic movements and posturing are even more common. EEG can provide clarification
when seizures are suspected.

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Different EEG patterns form a continuum of non-seizure to seizure activity, and over-
interpretation of these patterns may lead to inappropriate advice and treatment.
Recurrent seizures are easiest to recognise, with rhythmic discharges having spatiotemporal
evolution in frequency (>4Hz), morphology and distribution.

Periodic patterns are the most difficult. Generalised periodic discharges (GPDs) may or may
not be ictal, and the specific EEG findings combined with the clinical context determines the
approach. Severe myoclonic encephalopathy may occur after cardiac arrest, and the EEG may
show burst suppression, unreactive GPDs separated by isoelectric intervals, ± electrographic
seizures. The prognosis is extremely poor irrespective of treatment. Lateralised periodic
discharges (i.e. PLEDs) may be an interictal finding in epilepsy and after any acute cerebral
insult such as stroke or herpes encephalitis, and usually indicates a predisposition to seizures
rather than ongoing seizure activity.

Generalised triphasic waves are mainly seen with metabolic and toxic encephalopathies and
are usually not an ictal finding. Whilst they can be sharply contoured, they are recognised by
their other characteristics including morphology, state-dependent reactivity, and lag in phase
and/or morphology changes from front to back. Triphasic waves (and almost all periodic
patterns) are suppressed by benzodiazepines, along with consciousness and respiration. Such
EEG suppression, in the absence of clinical improvement, does not diagnose NCSE.
Ambiguous patterns can also be seen, with stimulus-induced rhythmic, periodic or evolving
discharges. Whilst non-convulsive seizures and NCSE may be detected in comatose patients,
prognosis is largely determined by the underlying aetiology and its complications.

Unresolved questions include what particular EEG patterns should consistently prompt
aggressive treatment. It is not always possible to be certain that an EEG pattern is ictal or non-
ictal, and in the correct clinical context (especially patients presenting with seizures or with a
history of epilepsy) a treatment trial may be indicated to see if temporally associated clinical
improvement occurs. EEG interpretation needs to be made within the clinical context by an
expert reporter.

WS1.12. EEG Coma Patterns and Prognosis

Kheng-Seang Lim1
1University of Malaya, Kuala Lumpur, Malaysia

Identify the EEG coma patterns is just one of the steps in clinical management of those with
coma. This lecture will discuss on the importance of EEG in diagnosis, prognosis,
management and monitoring of disease progress, but also the limitations of EEG. There are
various EEG patterns in coma including both focal and generalised EEG patterns. It is

53
essential to correlate the EEG with clinical findings, in order to determine the aetiology,
although in certain cases the EEG patterns will guide the diagnosis e.g. triphasic waves in
metabolic encephalopathy and periodic discharges in someone suspected to have Creutzfeldt-
Jakob disease. A clinical-based approach to differentiate epileptiform and non-epileptiform
patterns will be presented based on clinical history, EEG patterns, neuroimaging and response
to antiepileptic drugs. An attempt to determine the severity and prognosis based on EEG
patterns will be made, in correlation with the underlying aetiology and other clinical findings.
Lastly, EEG patterns in coma secondary to autoimmune encephalopathy will be discussed.

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Workshop 2: Transcranial Magnetic Stimulation (TMS)

WS2.1. Introduction to TMS

Walter Paulus1
1Department of Clinical Neurophysiology, University Medical Center Göttingen, Germany

Transcranial magnetic stimulation (TMS) transports a short (about 100 µs) high intensity (~ 2
Tesla) magnetic field painless through the skull into the brain and induces an electric current
flow. This in turn excites neurons or fiber tracts. TMS can only induce tangential current flow.
Its intensity declines exponentially; thus mainly cortical areas closed to the surface are
targeted. The best available data are those obtained at the motor cortex (M1). M1 stimulation
allows easy quantification of effects by recording electromyographic muscle activity. When
stimulating the visual cortex phosphenes can be induced. Computational models allocate the
lowest threshold elements into terminals of the axon collaterals. The dependence of threshold
and polarization on spatial and temporal stimulation parameters are strongly affected by
morphological features of the axonal arbor, including myelination, diameter, and branching.
The thresholds for current flow are directionally sensitive, with lower threshold from the back to
the front as vice versa. Intensity dependent a D-wave and several I-waves with 1.5 ms
intervals are induced, which can be recorded in man invasively at the cervical spinal cord. In
psychology it allows to induce short virtual lesions which provide data on cortical functions
arising in the area stimulated. TMS is clinically used to quantify central motor conduction time
in particular in patients with multiple sclerosis. Reliable information on amplitude changes e.g.
in cervical myelopathy can be obtained by the triple stimulation technique.

WS2.2. Parameters of TMS

Ulf Ziemann1
1University of Tübingen, Tübingen, Germany

Transcranial magnetic stimulation (TMS) is a non-invasive painless technique to stimulate the


human brain. This presentation will introduce basic physiological principles of how TMS excites
human motor cortex. In addition, the following single-pulse and paired-pulse TMS measures of
corticospinal and motor cortical excitability will be presented and discussed with respect to
underpinning physiological mechanisms: motor threshold, motor evoked potential recruitment
curve, cortical silent period, short-interval intracortical inhibition, intracortical facilitation, long-
interval intracortical inhibition, short-interval intracortical facilitation, short-latency afferent

55
inhibition. Pharmacological evidence will be summarized to explain the physiology of these
measures. In addition, clinical utility will be exemplarily discussed by demonstrating abnormal
TMS measures of corticospinal and motor cortical excitability in patients with epilepsy and
Alzheimer's disease.

WS2.3. TMS: Clinical Application of MEPs

Mark Hallett1
1Human Motor Control Section, NINDS, NIH, Bethesda, MD, USA

Transcranial magnetic stimulation (TMS) has utility for clinical assessment, studies of
physiology, and therapy. There are a number of purposes in the clinic, particularly in regard to
assessment of the corticospinal tract by central motor conduction velocity (CMCV). CMCV is
obtained by getting the motor evoked potential (MEP) latency and subtracting the peripheral
conduction time. Peripheral conduction time can be assessed with stimulation over the spine
or with the F-wave technique. In situations such as multiple sclerosis and cervical myelopathy,
central conduction time is prolonged. It can also be used to monitor corticospinal tract function
during surgery. TMS can be useful in proving normal function in patients with functional
paresis. Recently, it is also being investigated for evaluation of upper motor neuron function in
patients with ALS. Excitability of the motor cortex in this circumstance is best assessed with
threshold tracking. Another valuable use is for presurgical mapping to examine topography of
functional cortex. This is most commonly done for motor function and language. Guidelines for
optimal methodology for mapping have been published.

WS2.4. Introduction to rTMS

Ying-Zu Huang1
1Medical School, Chang Gung University, Department of Neurology, Chang Gung Memorial Hospital.

Taoyuan, Taiwan

Transcranial magnetic stimulation (TMS) is a non-invasive technique that stimulates the brain
with the eddy current generated by short of magnetic pulses penetrating through the scalp and
skull. Repetitive TMS (TMS) that give TMS pulses repetitively in a train or trains has been
noticed to change the cortical excitability outlasting the stimulation periods for minutes to more
than an hour. Starting with TMS pulses given at a regular frequency, several forms of rTMS
protocol have been developed and applied for research and clinical purposes in the past

56
decades. Different protocols have different effects for enhancing or suppression purposes and
different efficiency in time and energy consumption. In this talk, the basic principle, developing
strategies, unique features, research and clinical applications of rTMS will be introduced and
explained. The audience would have a clear concept of rTMS and knowledge for selecting a
suitable rTMS protocol based available equipment and the purposes after this talk.

WS2.5: Demonstration of TMS

Yoshikazu Ugawa
1Fukushima Medical University, Fukushima, Japan

57
Workshop 3: Nerve Conduction Studies (NCS) /
Electromyography (EMG)

WS3.1. Basic Principles and Pitfalls of NCS

Jun Kimura1
1Department of Neurology University of Iowa, Iowa City, IA, USA

We have come to recognize five simple principles in the practice of nerve conductions studies.
These include 1) take a careful clinical history and examine the patient before initiating the
study; 2) watch the muscle twitch and isolate the target under scrutiny before measuring the
response; 3) compare responses elicited by shocks given distally and proximally to the site of
lesion; 4) assess physiologic and pathologic changes by linearity and nonlinearity in segmental
studies and 5) select a short distance for a focal lesion and a long passage for a diffuse
process to improve sensitivity and reproducibility.

In the evaluation of a focal lesion, studies of a longer segment tend to lower the sensitivity of
the test because the inclusion of the unaffected segments in calculation dilutes the effect of
slowing at the site of lesion. With stimulation of a normal nerve in 1 cm increments, the
latency-changes approximately 0.2 ms/cm for both sensory and motor conduction. In patients
with an entrapment syndrome, a latency- increase exceeding 0.4 ms across a 1 cm segment
usually signals a focal abnormality. An abrupt nonlinear change in waveform of the recorded
response provides an additional, and perhaps more convincing evidence.

In contrast, a longer segment provides a better result in assessing a diffuse or multi-


segmental process such as polyneuropathies. A longer path accumulates all segmental
abnormalities, which individually might not show a clear deviation from the normal range.
Assume a nerve impulse conducting at a rate of 0.2 ms/cm (50 m/s). A 20% delay for a 10
cm segment yields only 0.4 ms, whereas the same change for a 100 cm segment amounts
to 4.0 ms, for easy detection. Thus, the longer the segment, the greater the delay in
assessing a diffuse process. Evaluating a longer, as compared to shorter, segment also
improves the overall accuracy because the same absolute measurement error constitutes a
smaller percentage of change in latency and distance.

In essence, short distances magnify focal conduction abnormalities despite increased


measurement error, and long distances, though insensitive to focal lesions, provide better
yields and reliability for a diffuse process.

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WS3.2. Electrophysiological Assessment of Brachial Plexopathies

Jorge E. Gutierrez1
1Potenciales Center of Electrodiagnosis, Cali, Colombia

The brachial plexus has more than 166,000 interconnected axons and it´s a very vulnerable
structure. Imaging studies do not provide functional information about the plexus and routine
electrodiagnostic studies are usually incomplete. The anatomy and pathophysiology of the
lesions must be known to improve the treatment outcomes.
The clinical and electrodiagnostic evaluations must be extensive but oriented. A root injury
must be suspected after traction injuries, proximal wounds, in the presence of impairment of
muscles innervated directly from roots like anterior serratus, rhomboids, diaphragm and in the
presence of dysfunction of sympathetic preganglionic fibers from C8-T1. A trunk injury must be
suspected after supraclavicular or retro-clavicular injuries, in the presence of suprascapular
nerve involvement (upper trunk) or selective impairment of median or radial nerves.
No branches arise from middle or lower trunks and isolated middle trunk lesions are very rare.
An injury at the cord level must be suspected after infra-clavicular injuries, in presence of
involvement of the medial brachial and antebrachial cutaneous nerves, involvement of
latissimus dorsi, pectoralis and median nerve selective compromise.

In motor nerve conduction studies (MNCS), the distal amplitude represents an estimate of the
number of viable axons. For uncommon MNCS the side to side amplitude comparison is the
best indicator of nerve damage. No motor NCS is available for C7. Some MNCS are helpful to
localize (e.g.: radial over EIP, suprascapular). The sensory nerve conduction studies (SNCS)
are helpful to differentiate a pre vs postganglionic lesion (after > 10 days). Detailed sensory
studies help to define injured structures even better than motor conductions. No sensory NCS
is available for C5. The needle EMG studies are useful to detect mild axonal loss. There is no
correlation between number of Fibs/PSWs and severity of the axonal loss. The recruitment
reduced in conduction block. Localizing muscles must be evaluated and the muscle domains
must be well known. The utility of late responses like F wave and H reflex is limited in this
context. Somatosensory Evoked Potentials are useful to determine continuity from the
peripheral to the central nervous system.

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WS3.3. The Values of H-Reflex and Other Late Responses in Disease

David Burke1
1Department of Neurology, Royal Prince Alfred Hospital, and University of Sydney, Australia

It is relatively easy to record the H reflex from soleus, and many authorities still teach that it
can be obtained only from this muscle. Inability to record the soleus H reflex has three possible
causes: (i) poor technique; (ii) low central excitability; (iii) loss of afferent fibres due to, e.g.,
peripheral neuropathy or nerve root pathology. If the reason is (ii), all reflexes, in upper and
lower limbs, will be difficult to elicit, and the reflex response should then become apparent
during a steady voluntary contraction. A voluntary contraction not only potentiates the reflex
but it also decreases the stimulus intensity needed for the reflex, ensures that the reflex
involves the contracting muscle, and greatly reduces the attenuation of the response that
occurs with higher stimulus rates. It reduces the latency of the reflex responses only slightly
(<0.5 ms). During a steady voluntary contraction of the test muscle, H reflexes can be recorded
for most limb muscles, and provide information about conduction across the most relevant
spinal segments (e.g., C5/6, C6, C6/7, C8/T1, L3/4, L4/5 and S1). In routine diagnostic
practice, I assess reflex conduction in perhaps 60% of patients because this provides insight
into conduction in afferent and efferent axons over the entire peripheral nerve pathway. In
diagnostic practice we focus on pathology that causes loss of reflex function. However, if you
can record H reflexes at rest from muscles that normally have no H reflex unless they are
contracting voluntarily, there is presumptive evidence of hyperreflexia. If there is EMG
evidence of denervation in that muscle, this suggests UMN and LMN changes for a single
muscle, and this is support for a diagnosis of motor neurone disease. F waves can provide
complementary information about motor axons because the H reflex preferentially involves
slowly conduction motor axons from small low-threshold motoneurones, but the F wave
measures conduction in larger axons that are not discharged reflexly by the intense afferent
volley produced by the supramaximal stimulus.

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WS3.4. Electrodiagnostic Approach to Motor Neuron Disease: Split Hand in
ALS

Satoshi Kuwabara1
1Chiba University, Chiba, Japan

Amyotrophic lateral sclerosis (ALS) is characterized by involvement of both upper and lower
motor neurons. Electrophysiologic approach includes EMG (denervation potentials and
fasciculation potentials) and nerve conduction studies (NCS; low CMAP, decreased motor unit
number, and axonal hyperexcitability on nerve excitability testing) for lower motor neuron
involvement, and transcranial magnetic stimulation with threshold tracking (decreased short-
latency intracortical inhibition) for upper motoneuronal hyperexcitability. Of these, routine NCS
can detect the “split hand” pattern, which is a very important indicator for diagnosis, because
this symptom has been recognized as being specific to ALS; the thenar and first dorsal
interosseous (FDI) muscles are preferentially affected. In contrast, the hypothenar muscle
(abductor digiti minimi, ADM) is relatively preserved. FDI and ADM muscles are innervated by
the same C8/Th1 spinal segments and the same ulnar nerve. The split hand indicates a
potentially specific pathological mechanism, suggesting hyperexcitability of both upper and
lower motor neuron/axons.

Along with this symptom, wide-spread fasciculations are prominent clinical features in patients
with ALS, specifically seen in ALS among disorders with neurogenic muscle atrophy.
Fasciculations frequently arise from the motor nerve terminals, and the hyperexcitability of
motor axons appears to constitute the pathophysiology of the disease. Neurophysiologic
investigations of the upper and lower motor neurons/axons have shown increased excitability.
The altered excitability is supposed to relate to motor neuron death.

WS3.5. Idiopathic Inflammatory Myopathies

Renato J. Verdugo1
1Department of Neurology and Psychiatry, Faculty of Medicine, Clinica Alemana-Universidad del

Desarrollo, Santiago, Chile.

WS3.6. Overview of Routine EMG (with Demonstration)

Masahiro Sonoo1
1Teikyo University School of Medicine, Tokyo, Japan

61
WS3.7. Repetitive Nerve Stimulation Testing

Donald B. Sanders1
1Duke University Medical Center, Durham, NC, USA

Repetitive nerve stimulation (RNS) is the most frequently used electrodiagnostic test to
diagnose neuromuscular junction disorders. Although seemingly simple, attention to technique
is essential to avoid technical errors.

Stimulation at low rates (2-5 Hz) (LF-RNS) depletes acetylcholine stores at diseased motor
endplates, causing failure of neuromuscular transmission. A decrement of at least 10% to
trains of 2-3 Hz stimulation at rest or immediately after a period of maximal muscle contraction
(“activation”) is the usual criterion of abnormality; 5% can be used if technical artifacts can be
excluded. Take care to deliver adequate stimulation and prevent movement of the tested
muscle throughout nerve stimulation; peripheral muscles should be kept warm during testing.
RNS is reportedly abnormal in up to 90% of generalized myasthenia gravis (MG) patients and
in up to 60% of ocular myasthenia – sensitivity is strongly dependent on the muscles selected
for testing. RNS is more often abnormal in proximal, facial and clinically weak muscles; even
so, RNS is frequently normal in mild or purely ocular disease. For maximal diagnostic yield,
test several muscles, including those that are weak.

The characteristic findings in Lambert-Eaton myasthenia (LEM) are: small CMAPs at rest, a
decrement to LF-RNS, and marked facilitation. In MG, activation for 30 - 60 seconds is used to
unmask a mild decrement. In LEM, activation is used to demonstrate facilitation; the simplest
procedure is to elicit a single CMAP from the rested muscle, have the patient contract the
muscle for 10 seconds, then immediately elicit a single CMAP. Facilitation of at least 60% is
evidence of a pre-synaptic disorder, but may not be seen in all muscles. A decrement in at
least one hand muscle is the most frequent RNS abnormality in LEM and may be seen even
when CMAPs are not small and diagnostic facilitation cannot be demonstrated.

In MG the decrement is usually greatest in the 4th or 5th response and is less during
subsequent responses, producing a so-called ‘‘U-shaped” pattern. A continually progressive
decrement is usually seen in LEM. The pattern in MuSK MG resembles that in LEM.

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WS3.8. Cranial Nerve Assessment (with Demonstration)

Jun Kimura1
1Department of Neurology University of Iowa, Iowa City, IA, USA

The cranial nerve condition commonly tested in an EMG laboratory comprise mono or
polyneuropathies affecting the accessory, trigeminal or facial nerve. A posterior triangle tumor or
surgical procedures can damage the spinal accessory nerve. Other causes include stretch induced
injury, carotid endarterectomy, and ligature injury during surgical exploration. The paralysis of the
sternocleidomastoid causes weakness when the face is rotated toward the opposite shoulder.
Stimulation of this nerve behind the sternocleidomastoid elicits a compound action potential
(CMAP) recordable from the upper trapezius muscle.

The differential diagnosis of facial palsy, which constitutes the most common disorder of the cranial
nerve seen in an EMG laboratory, deserves a special mention. Weakness of the orbicularis oculi
and frontalis suggests a peripheral type of facial palsy, which may herald other symptoms of
multiple sclerosis, Lyme borreliosis and AIDS. Patients with Guillain Barre Syndrome and Charcot
Marie tooth disease type 1 may develop bilateral, or rarely unilateral, involvement. An acoustic
neuroma may compress the facial nerve. Bell's palsy develops sporadically, 80% showing a good
prognosis with demyelination, and 20%, a less favorable outcome with axonal degeneration as in
herpes zoster infection.

The conduction study of the facial nerve and blink reflex reveal various degrees of abnormality in
most of these patients. Stimulation of the facial nerve evokes CMAP usually recorded from the
nasalis. Blink reflex elicited by supraspinal nerve consists of ipsilateral R1 and bilateral R2
components. The latency of R1 represents the conduction time along the trigeminal and facial
nerves and poutine relay. Inherent latency variability makes R2 less reliable for diagnostic
purpose.

During the session, participants will observe various electrodiagnostic techniques currently in use
for the evaluation of the cranial nerves, and learn pit falls which may lead to an erroneous
interpretation of the acquired results. All these should lead to a better understanding of the use of
electrodiagnostic studies in attending patients with a disorder of cranial nerves in the clinical
practice. In patients with a diffuse process such as polyneuropathy, examination of the cranial
nerves complements the routine studies of the upper and lower limb nerves.

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Workshop 4: Advanced Neurophysiology Techniques

WS4.1. My Approach to Motor Unit Number Estimation (with


Demonstration)

Sanjeev Nandedkar1,2
1Natus Medical Inc, Hopewell Junction, NY, USA

2Medical College of Wisconsin, Milwaukee, WI, USA

In motor neuron disease such as amyotrophic lateral sclerosis, the motor neurons die from the
disease process. They cannot be replaced. The loss of motor units (MUs) is partially
compensated by reinnervation. As a result, the number of MUs is reduced and their size is
increased. Reinnervation cannot sustain and may not be sufficient as the MU loss continues.
This leads to progressive weakness.
As we look for treatments, we need a biomarker to track the disease progression. This had led
to the methods of “motor unit number estimation (MUNE)” over the last 5 decades. If a
treatment is effective, the rate of MU loss should decrease.
In this workshop I will review MUNE methods that were based on recording and measuring
individual surface recorded motor unit potentials (SMUPs). They are called “incremental
stimulation”, “multiple point stimulation”, etc. The methods are time consuming due to low
amplitude signals and have been used to study small distal muscles. Furthermore, they are not
automated.
In the last 15 years we have seen new methods based on mathematical models. Hence the
calculation is described as “motor unit number index (MUNIX)”. In MUNIX the compound
muscle action potential (CMAP) and surface EMG interference pattern are analyzed. The
procedure is automated, requires less than 5 minutes per muscle, and can be used for large
muscles such as tibialis anterior. By adding results from several muscles, one can better track
the disease progression. The methods also provide a measure for the amplitude of SMUP. The
motor unit size index (MUSIX) increases with reinnervation.
Another set of methods is based on ‘CMAP scan’. The stimulus intensity is reduced in fine
steps from supramaximal level to low intensity where no response is recorded. The amplitude
of 500 responses is analyzed using different algorithms. This method is also automated for
recording the data.
All methods have their advantages and disadvantages. They provide different numerical
results. However, each method shows disease progression when serial investigations are
performed. Such assessment requires careful measurements of the CMAP regardless of the
method.

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WS4.2. My Approach to Quantitative EMG (with Demonstration)

Sanjeev Nandedkar1,2
1Natus Medical Inc, Hopewell Junction, NY, USA

2Medical College of Wisconsin, Milwaukee, WI, USA

The routine needle EMG examination is usually performed by observing the waveforms on the
display screen and listening to their sound. It is subjective. Quantitative analysis (QA) is
considered time consuming and not used.
In this workshop, I will describe the “objective and interactive” approach. It adds elements of
QA to record and measure EMG signals. It does not add time for analysis, and provides
quantitative data to document abnormalities. The techniques of recording and measurements
will be demonstrated. This includes use of dual time base, amplitude trigger, cascaded traces,
etc. The “quality” control of the recording will be emphasized. The various signal displays help
better assessment of amplitude, duration, phases, turns, stability, and firing rate of motor unit
potentials (MUPs). It is also useful to assess the fullness of the interference pattern and its
amplitude. The additional information captured by this approach will improve the quality of the
needle EMG examination.
Finally, I will demonstrate the fully automated methods for MUP analysis (Multi-motor unit
analysis) and IP (Turns & amplitude). This requires just a few minutes per muscles. Using
modern algorithms, QA is not time consuming. It should be included in the laboratory routine.

WS4.3. Overview of Axonal Excitability Studies

Kazumoto Shibuya1
1Chiba University, Chiba, Japan

Nerve excitability testing is a non-invasive neurophysiological technique, which investigates


nerve functions in vivo. While nerve conduction studies deliver supramaximal stimuli to
measure conduction velocities and the number of large myelinated fibres, nerve excitability
studies use weaker stimuli and measure strength of stimuli, which evoke
target compound muscle action potential or sensory nerve action potential amplitudes. The
combination of conditioning and test stimuli, with various strength and
duration, can examine functions of various types of ion channels and ion pumps and
exchangers, which are located in peripheral nerve axons, and membrane
potential. Several paradigms, such as strength-duration properties, threshold electrotonus,
current-threshold relationship and recovery cycle, are assessed in this testing, and alterations

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of parameters in these paradigms indicate changes in those functions. This technique
has disclosed various pathophysiological alterations mainly in genetic and acquired
channelopathies. A famous study, using this technique, disclosed an effect of puffer fish
poisoning for nerve excitability. Tetrodotoxin is known to block sodium channels, and
salient reductions of strength-duration time constant and refractoriness in recovery cycle,
makers of persistent and transient sodium channel functions, respectively, were disclosed in
patients with tetrodotoxin intoxation. This study firstly visualized an effect of
tetrodotoxin for the human peripheral nerve. In another study, increased strength-duration time
constant, i.e. increased excitability, was revealed in patients with neuropathic pain and muscle
cramps. Mexiletine treatment, a sodium channel blocker, for these patients relieved those
symptoms and decreased strength-duration time constant. As those studies, recent studies,
using this testing, have disclosed an impact of altered ion channel functions for neurological
diseases and utility of this technique as a treatment biomarker. In this session, I plan to
introduce the principle of this testing and recent advances in clarification of pathophysiology in
neurological diseases and treatment developments, using this testing.

WS4.4. Overview of Muscle Membrane Excitability

Karl Ng1
1Royal North Shore Hospital, University of Sydney, Australia

The technique of muscle membrane excitability, as assessed by Bostock’s QTracs software, is


explained along with its application and interpretation. This relatively new method has afforded
an insight into many properties of normal and diseased muscle, including membrane
polarization. The premise of the technique is that sarcolemmal excitability changes will alter
the conduction velocity of spreading depolarization along the muscle membrane. The studies
behind important influences such as and including age, muscle selection and serum K+ are
discussed, and the results of application of technique to conditions such as inclusion body
myopathy and myotonic dystrophy are reviewed.

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Workshop 5: Intraoperative Neurophysiological Monitoring
(NIOM)

WS5.1. SEP Monitoring

Sandip Chatterjee1
1Park Clinic, India

WS5.2. Intraoperative Monitoring of Motor Evoked Potentials: Physiology


and Applications

Yew Long Lo1


1Department of Neurology, National Neuroscience Institute, Singapore General Hospital

Intraoperative monitoring (IOM) of motor evoked potentials (MEP) represents a significant


advance for evaluation of descending motor tract integrity. For spinal decompression, there is
increasing evidence of its efficacy, but ongoing research is performed to improve sensitivity
and specificity. To that end, there is also ongoing debate for warning criteria of MEP changes
from transcranial electrical stimulation (TES). It is presently unclear if a cutoff for ensuing
neurological deficit should be MEP disappearance or a value between this and 50% amplitude
reduction. Interim adjunctive strategies include use of ipsilateral MEPs, modified scalp
stimulating positions and multimodality monitoring. Some investigators have proposed a more
directed motor threshold to obtain supramaximal MEP voltage in an individual subject.

The indications of MEP monitoring have expanded from spinal surgery to central nervous
system procedures involving cortical, subcortical and brainstem regions. Corticobulbar
MEPs can provide localization and evaluate functional integrity of cranial nerve
nuclei. Awake craniotomy can combine monopolar MEP mapping with other modalities to
preserve speech and motor functions.

The evidence of MEP monitoring for scoliosis to date has been encouraging for the prevention
of neurological compromise, as a result of deformity correction. IOM of MEPs is pertinent here
as a non-invasive surgical adjunct for these neurologically intact patients. Recently, a study
has suggested that in susceptible individuals, intraoperative suppression of MEPs may rarely
occur unpredictably, independent of surgical or anesthetic intervention, but anesthetic factors
may be overall more significant.
Side effects of TES are rare, but seizures, bite injury, lip laceration, mandibular fracture, scalp
burns and cardiac complications should be anticipated. .

Improved knowledge in the use of intravenous anesthetic agents and pulse train cortical stimuli
has allowed for more efficacious MEP monitoring. However, recent studies suggest

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that often, suboptimal MEPs were elicited, especially during IOM of diseased spinal cord.
Various central and peripheral facilitatory techniques will be discussed.

WS5.3. Brainstem Auditory Evoked Potential Monitoring

Aatif M. Husain1
1Department of Neurology, Duke University Medical Center; Neuroscience Medicine, Duke

Clinical Research Institute; Neurodiagnostic Center, Veterans Affairs Medical Center, Durham,
North Carolina, USA

Brainstem auditory evoked potentials (BAEP) are obtained by stimulating the vestibulocochlear
nerve. As the stimulation volley ascends in the brainstem, BAEP waveforms can be obtained
from various neurologic structures. BAEPs are frequently used during surgery to monitor the
integrity of the distal auditory pathways. It is useful when the integrity of the cochlea,
vestibulocochlear nerve, or brainstem auditory structures is at risk. In this presentation, the
basics of the anatomy of the auditory pathways will be discussed. This will be followed by a
discussion of the methodology and technical challenges of obtaining BAEPs in the operating
room. The types of changes in BAEP waveforms that may occur in the operating room will be
described. Finally, a summary of the clinical applications of BAEP monitoring will be reviewed.

WS5.4. EMG Monitoring: Free-Run and Triggered

Tsui Fen Yang1


1Department of Physical Medicine and Rehabilitation, Taipei Veterans General Hospital,

Taipei, Taiwan

The recording of compound muscle action potentials (CMAPs) in response to spontaneous or


electrically stimulation of the cranial, spinal, peripheral nerves or nerve root activation is known
as intraoperative electromyography (EMG). EMG monitoring is widely used in surgeries when
the aforementioned neural structures might be at risk. It allows early detection of surgery-
related nerve injury so that proper intervention can be accordingly adopted; it also allows
confirmation of the functional status of the nerve. The intraoperative monitoring team must
thoroughly understand the nervous pathways that require monitoring and mapping in order to
develop optimal stimulation and recording strategies for each surgical procedure.

Intraoperative EMG testing includes triggered EMG (tEMG) for mapping and free-run EMG
(fEMG) for monitoring purposes. Triggered EMG is used to identify the nerves and nerve root,
to define a safe entry pathway to access the lesion, or to assess the functional integrity of the
nerve so that the surgical plan can be modified accordingly. Clinical applications of tEMG also

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include confirmation of the placement of pedicle screws during spine surgery and guiding the
sectioning of the lumbosacral rootlets during selective dorsal rhizotomy. The following three
kinds of stimulation electrodes are available: monopolar, bipolor, and tripolar electrode. Each
of the aforementioned electrodes are associated with certain strengths and weakness; thus,
they can be used under different circumstances and meets most of our clinical needs.

fEMG involves recording spontaneous or evoked muscle activity without interfering with the
surgery. It is used as a monitoring tool to detect surgically driven mechanical irritation of the
peripheral nerves and motor cranial nerves, hopefully before permanent damage occurs. It
also can be applied to detect muscle activity evoked by mechanical irritation of the
corticospinal tract and/or alpha motor neurons during brain tumor or intramedullary spinal cord
tumor resection. There are three types of fEMG activity, referred to as the A, B and C train.
Among them, the A train has been more associated with poor postoperative outcomes,
although false-positive and false-negative findings do exist. Thus, the concomitant use of
somatosensory and motor evoked potentials will certainly increase the sensitivity and
specificity of fEMG.

\WS5.5. Electroencephalographic Monitoring

Aatif M. Husain1
1Department of Neurology, Duke University Medical Center; Neuroscience Medicine, Duke

Clinical Research Institute; Neurodiagnostic Center, Veterans Affairs Medical Center, Durham,
North Carolina, USA

Electroencephalographic (EEG) monitoring was one of the first neurophysiologic techniques to


be used in the operating room. EEG helps assess cerebral function during surgical
procedures. It is different than evoked potential monitoring in the operating room as there is no
stimulus used to elicit the waveforms, rather ongoing “spontaneous” activity is assessed. One
of the earliest applications of EEG in the operating room was to determine adequacy of
cerebral perfusion during carotid endarterectomy surgery. Subsequently, many other uses
have been realized for EEG monitoring. In this presentation, the specifics of EEG recording
techniques in the operating room will be discussed. Anesthetics can greatly affect the EEG,
and these effects will be reviewed. How and why the EEG changes during surgery will be
discussed as well. Finally, the clinical applications for EEG monitoring will be reviewed.

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WS5.6. Intraoperative Monitoring and Mapping of Visual Pathways

Kunihiko Kodama1
1Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan

Background and Objective:


Neurosurgical procedures along the visual pathways carry a risk of visual dysfunction. A
reliable method for real time visual function monitoring assists in intraoperative decision
making regarding radicality of excision and intermittent maneuvers near the optic apparatus.
We present our experience of intraoperative flash visual evoked potential (VEP) monitoring.

Methods:
1) Anesthesia: Under total intravenous anesthesia (TIVA), the VEP shows larger
amplitude and stable latency with less variability. Inhaled anesthetics, such as sevoflurane and
isoflurane, markedly decrease the amplitude, and these should be avoided.
2) Stimulation: Flash VEP and ERG are recorded by flush stimulation on the closed
eye lids. At least 1-minute preconditioning by flash stimulation should be done to obtain a
steady VEP waveform before starting averaging. The stimulation intensity is decided by the
supramaximal stimulation to the retina. As the evoked ERG is easy to record and the
waveform is stable, it is utilized for checking the supramaximal value and inadequate
stimulation to retina due to stimulator dislocation.
3) VEP Recording: Reference electrodes are set at A1 and A2, and these are
electrically connected. Recording electrodes are set near Oz, O1, and O2. Simultaneous ERG
recording is recommended as described above. Warning sign was defined as 50% decrease of
baseline amplitude.
4) Optic nerve action potential: In cases of parasellar lesions such as
craniopharyngiomas and giant aneyrysms, optic nerves, chiasm and tracts are sometimes
streched around the lesion, and it is difficult to identify the location of the optic apparatus. In
these situations, flash stimulation is deliverd as same manner as VEP, and action potentials
recoeded from the operative fields facilitates to identifiy the optic apparatus.

Results:
VEP can be monitored in a patient with visual acuity greater than (0.03). Fifty percent decrease
of VEP amplitude as warning sign detected postoperative visual aggravation in sensitivity of
99%. Reversible change in VEP means visual function to be preserved. Sudden VEP change
correlates with visual acuity impairment and/or hemianopsia. The flat VEP indicates
postoperative severe visual disturbance (nearly blindness).

Conclusions:
Intraoperative flash VEP monitoring is essential and reliable method for preserving visual
function.

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WS5.7. Mapping Methodologies
Kyung-Seok Park1
1Department of Neurology, Seoul National University Bundang Hospital, Seoul National

University College of Medicine, Seoul, Korea

There are two main methodologies which can be used to assess the functional integrity of
nervous system during intraoperative neurophysiological monitoring (IONM). Monitoring
provides continuous real-time information about the functional integrity of the nervous tissue,
whereas mapping provides rapid anatomical identification and localization of neural structures.
Mapping of the primary motor cortex and subcortical corticospinal tract (CST) can be
performed with direct cortical and subcortical electrical stimulation with a hand-held probe.
Mapping of the central sulcus can be achieved by using phase reversal technique of
somatosensory evoked potentials (SEP).
This talk will be specifically focused on basic principles and application of these mapping
methodologies and their usefulness mainly in brain tumor surgeries.

Key Words: intraoperative neurophysiological monitoring (IONM), cortical mapping, subcortical


mapping, central sulcus mapping

WS5.8. Anaesthesia and Intraoperative Neurophysiological Monitoring

Sitaram Raman1
1Singapore General Hospital, Singapore

There has been a growth of monitoring in the operating room today. From a “finger on the
pulse” as a standard to ECGs and pulse oximeters. Now neurological monitoring in also used
extensively in the OR. There are three types of neuro monitoring in the OT which will be
discussed today.

Firstly, single nerve monitoring, eg the facial nerve during parotid surgery. Next, we have the
monitoring of the depth of anaesthesia via the bispectral index and density spectral array. This
helps us understand the depth of anaesthesia especially in total intravenous anaesthesia.

BIS monitoring is good at indicating an “alert” state, which is why it can reduce the incidence of
intraoperative awareness. However, its algorithm does not always accurately predict an
“asleep” state. This means an unknown percentage of patients who are already asleep will not
be identified because of falsely elevated BIS values. These patients will receive unnecessary
dosage of anesthetics resulting in a deep hypnotic state.

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Given that there may be falsely elevated values, we have to consider when it is best to use the
monitors and if it is cost effective. Lastly, we monitor the spinal cord using SSEPs and MEPs
during spine surgery especially scoliosis surgery.

Today it is standard practice to conduct some form of monitoring when performing any spinal
operation that is associated with a high risk of spinal injury. Generally, operations are
considered to carry such a risk when corrective forces are applied to the spine, the patient has
preexisting neurological damage, the cord is being invaded, or an osteotomy or other
procedure is being carried out in immediate juxtaposition to the cord.

In this presentation we will look at the latter two monitoring modalities and whether they are
needed and are cost effective in the OR.

WS5.9. Artefacts in Neurophysiologic Intraoperative Monitoring

Ahmad Yanuar Safri1


1Universitas Indonesia-Cipto Mangukusumo General Hospital, Jakarta, Indonesia

Intraoperative neurophysiology monitoring (NIOM) is based on neurophysiological


examinations that are usually carried out in daily clinical practice. However, there are
fundamental differences in NIOM than in clinics due to the environment and the need for
accuracy and speed of interpretation.

The operating room is a hostile place for neurophysiology recording such us EEG, EMG, and
evoked potentials because of there are many sources of electricity that can cause interference
and artefacts during recording and will ultimately reduce the accuracy and speed of
interpretation of NIOM.

Technical error can be happening during NIOM and often cannot be corrected because of
aseptic and antiseptics practices has been performed in the field of surgery, therefore one
should have troubleshooting strategy and preparation in NIOM to overcome this problem.

The knowledge of the recognition of artefacts and trouble-shooting during NIOM becomes very
important to maintain patient safety during surgery. More than half of the work on NIOM is to
overcome NIOM's artefacts and trouble-shooting to get accurate recording results.

This topic will discuss practical ways to deal with artefacts and troubleshooting that are often
found in NIOM based on experience and the basics of electricity, including how to reduces
signal noise, get better recording results and strategy to get reliable and accurate interpretation
of NIOM and overcome technical error.

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This topic will also discuss the safety of NIOM to prevent iatrogenic injury such us tongue
laceration in the use of Trans-cranial MEP, induction of seizure in cortical stimulation and
prevent neural tissue injury due to direct stimulation.

Keywords: Neurophysiology Intra Operative Monitoring (NIOM), artefacts, troubleshooting.

WS5.10. IONM in Asia-Oceania: Current Status and Vision

Kyung-Seok Park1
1Department of Neurology, Seoul National University Bundang Hospital, Seoul National

University College of Medicine, Seoul, Korea

A demand for intraoperative neurophysiological monitoring (IONM) during various


neurosurgical procedures is increasing rapidly in Asian-Oceanian region. Therefore, IONM-
related local organizations have been established in several countries and doing their
academic activities in this field. Also, Asian-Oceanian Society of Intraoperative
Neurophysiology (AOSIN) was first established in 2018 for the purpose of education and
dissemination of knowledge of IONM in this region, and has held its annual congress since
then.

This talk will be specifically focused on a review of the current status of IONM in this region
and visions of AOSIN.

Key Words: intraoperative neurophysiological monitoring (IONM), Asia-Oceania, Asian-


Oceanian Society of Intraoperative Neurophysiology (AOSIN)

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Workshop 6: Single-Fiber EMG

WS6.1. Voluntary SFEMG: Principles and Parameters

Donald B. Sanders1
1Duke University Medical Center, Durham, NC, USA

SFEMG is a selective recording technique that identifies action potentials (APs) from individual
muscle fibers. Selectivity results from the 25 µm diameter recording surface that exits on the
side of the electrode, 3 mm from the tip, and is further enhanced by using a 500Hz high pass
filter. SFEMG measures fiber density and neuromuscular jitter.
Fiber density is measured during slight voluntary muscle activation. Position the electrode to
record with maximum amplitude the AP from one muscle fiber, which triggers the display, and
count the number of time-locked acceptable APs (amplitude >200 μV, rise time <300 μs). The
FD is the mean number of APs, including the triggering AP, counted at 20 sites throughout the
muscle. The FD differs among different muscles and increases with age, especially after age
70 and in distal limb muscles.

Jitter is measured either during axonal stimulation or voluntary activation. Stimulate facial
nerve branches proximal to their entry into the muscle or anterior to the ear. In limb muscles,
stimulate intramuscular axons via a monopolar needle electrode inserted near the endplate
zone. Stimulate at 2 to 10 Hz (10 Hz is preferred) and adjust the stimulus intensity to produce
a slight twitch. Measure jitter between the stimulus and individual APs. Analyze at least 50
stimuli for each AP and aim to measure at least 30 APs at different sites throughout the
muscle.

Jitter measured during voluntary activation is less subject to technical problems. Position the
electrode to record at least two time-locked acceptable APs. Aim to measure jitter from 20 AP
pairs from different sites throughout the muscle.
Calculate jitter as the Mean value of Consecutive Differences (MCD) of successive
interpotential intervals. A study is abnormal if the mean MCD exceeds the muscle upper limit,
or if more than 10% of AP pairs or endplates have increased jitter. With more pronounced
disturbances, individual muscle fibers fail to respond to nerve activation, blocking.

Jitter can be measured with small concentric needle electrodes if careful attention is given to
signal quality and appropriate reference values are used. Fiber density can only be measured
with SFEMG electrodes.

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WS6.2. Stimulation Single-Fibre EMG: Methods and Pitfalls (with
Demonstration)

Masahiro Sonoo1
1Teikyo University School of Medicine, Tokyo, Japan

Single-fiber electromyography (SFEMG) is a technique introduced by Prof. Stålberg and his


colleagues in 1970’s. SFEMG electrode has the smallest uptake area (0.3 mm in radius)
among EMG electrodes, and therefore can identify action potentials of individual muscle fibers
(single fiber action potentials; SFAPs) comprising the motor unit by applying a very high low-
cut filter (500 Hz). Recently, disposable concentric needle is commonly used in place of
reusable SFEMG needle (CN-SFEMG). By setting an even higher low-cut filter (1 or 2 kHz),
individual SFAPs can be identified even using the concentric needle.

There are two recording methods of SFEMG, during voluntary contraction (v-SFEMG) and
following axonal stimulation (s-SFEMG). Both methods have merits and demerits, and specific
pitfalls. Patient cooperation is necessary for v-SFEMG but not for s-SFEMG, which is therefore
applicable for comatose patients or small children. Examination time is usually believed to be
shorter in s-SFEMG (I don’t think that this is always true). This is because only one SFAP is
necessary in order to measure jitter in s-SFEMG, although two SFAPs recoded simultaneously
are necessary in v-SFEMG. However, v-SFEMG is probably more sensitive in detecting
neuromuscular junction (NMJ) dysfunction in myasthenia gravis (MG). Since SFEMG is
expected to be more sensitive than repetitive nerve stimulation test, the sensitivity must be the
most crucial issue. Therefore, I prefer v-SFEMG and conduct only v-SFEMG in daily practice.

For s-SFEMG, monopolar stimulating needle is inserted close to the motor nerve or terminal
nerve twigs (facial nerve branch lateral to the external canthus for frontalis recording). CN
needle is inserted within the target muscle. We search sharp SFAPs during 5 to10 Hz test
stimulation. When good SFAPs are identified, jitter is measured during 10 Hz stimulation.
Submaximal stimulation is the greatest pitfall in s-SFEMG. Therefore, the threshold current is
determined for each SFAP and an intensity 20% over the threshold is used for recording jitter.
Very often, the target SFAP is blurred by the contamination of other SFAPs that have been
newly-recruited while increasing the current intensity. This Is the reason why s-SFEMG takes
longer time than usually considered.

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WS6.3. Measuring Jitter with Concentric Needle Electrodes

Donald B. Sanders1
1Duke University Medical Center, Durham, NC, USA

Although it is preferable to use single fiber electrodes (SFEs) to measure jitter, concentric
needle electrodes (CNEs) are increasingly used instead because of concern about
transmissible diseases and the cost of SFEs.

Increasing the low frequency filter to 1 kHz or higher produces CNE signals that appear
identical to SF action potentials (APs), but such CNE signals frequently represent summation
of many SFAPs - to emphasize this, simple spikes recorded with CNEs that resemble SFAPs
have been called “apparent single fiber action potentials” (ASFAPs).

Jitter in summated signals recorded with CNEs is somewhat less than the jitter in SFAPs.
Thus, although increased jitter in CNE studies indicates abnormal neuromuscular
transmission, mild abnormalities may not be detected as well as with SFEs unless appropriate
reference values are used. Note - fiber density measurements can only be made with SFEs.
The following precepts should be followed if CNEs are used for jitter measurements:
• Use the CNE with the smallest recording surface. Currently this is the DCF 25
electrode, which is available from several vendors. Note - because of their large pickup
area, monopolar needle electrodes are even more likely to record superimposed APs
and should not be used for jitter analysis.
• Use a high pass filter setting of 1kHz. Because this will reduce the amplitude of
recorded signals, spikes with amplitude as low as 50 μV are acceptable for jitter
measurement if they have a rise time less than 300 μsec.
• Measure jitter only from simple spikes that resemble SFAPs (ASFAPs). Do not
measure jitter from spikes with a notch or “shoulder,” as these are definitely produced
by more than one AP.
• Use reference values for the specific CNE that you are using.

We recommend that each laboratory obtain results from a small group of control subjects and
compare these to published reference values, using the same techniques that were used to
acquire those reference values, i.e., the specific type of electrode, filter settings, and criteria of
acceptable APs.

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WS6.4. Demonstration of SFEMG Technique

Erik Stålberg1
1Uppsala, Sweden

A healthy subject will be examined with SFEMG in the frontalis muscle. First slight voluntary
activation and then with electrical stimulation. Concentric needle electrodes will be used.
Potential pairs of good and non-acceptable quality will be shown and comments will be made
on pitfalls particularly in stimulation SFEMG. Remarks on criteria for signal acceptances will be
made and a few examples of post-processing will be given.

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Workshop 7: Neuromuscular Ultrasound
WS7.1. Introduction to Neuromuscular Ultrasound

Einar Wilder-Smith1
1Kantonsspital Luzern, University of Berne, Switzerland

The rapid technical development of high-resolution ultrasound imaging over the past 20 years
has resulted in Neuromuscular Ultrasound becoming firmly established in everyday clinical
neurological practice. Using the latest available 18–6 MHz
transducers, high quality images of both nerve and muscle are generated. Current high-
resolution imaging using 20 MHz linear array probes whilst being restricted to skin penetration
of 1 cm, is at just under 10 µm. For nerves the most important classification parameters are
cross sectional area, echogenicity and Power Doppler, the latter representing a non-directional
measure of the volume of blood present in low flow tissues.

The original and most widely used clinical indication for NUS represents entrapment
neuropathies. Elaborate comparison between cadaver nerves and nerve ultrasound studies
have firmly established the accuracy of the method. Furthermore, studies on musculoskeletal
ultrasound have shown agreement between senior and non-experienced ultrasonographers
after joint examinations. Cost effectiveness of NUS is excellent.

Increasingly important is the Nerve Ultrasound (NUS) work-up of polyneuropathies, in


particular, the field of inflammatory neuropathies. Emerging differences in patterns of nerve
pathology are increasingly helpful in disease classification. In the evaluation of nerve trauma,
NUS is more and more useful for presurgical planning.

Muscle Ultrasound is helpful in a variety of clinical settings. As a screening technique for


detecting Myopathies/myositis the technique of quantitative ultrasound has good sensitivity but
is still highly dependent on the settings and image post processing of individual ultrasound
machines. For detecting the fasciculations associated with Motor Neuron Diseases,
ultrasonography has been shown more sensitive than EMG.

With further technical advance and standardization of muscle US techniques, disease specific
advances are likely to take place.

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WS7.2. Guidelines for Neuromuscular Ultrasound Training

Eman Tawfik1
1Department of Physical Medicine & Rehabilitation Faculty of Medicine, Ain Shams University, Cairo,

Egypt

‘Without standards, there can be no improvement’ This quote by Taiichi Ohno applies to every
domain including medical practice and summarizes why are we going to talk about the
guidelines for neuromuscular ultrasound training.
Currently, neuromuscular ultrasound has become a fundamental part of the diagnostic workup
of neuromuscular disorders. Thus, there is a growing interest among physicians to acquire the
skill. However, there is no consensus on what constitutes adequate training or the curriculum
of different neuromuscular ultrasound training levels. In this lecture, Dr. Tawfik is going to talk
about why do need guidelines for neuromuscular ultrasound training, how did a group of
experts develop the guidelines, the general guidelines as well as the learning objectives & the
curriculum of different levels of neuromuscular ultrasound training. Dr. Tawfik will end up the
lecture by highlighting how the audience can benefit from the guidelines and apply them as a
neuromuscular ultrasound tutor/or as a learner.

WS7.3. Neuromuscular Ultrasound in Upper Extremity

Byung-Jo Kim1
1Korea University Medicine, Seoul, Korea

Application of high-resolution ultrasonography in neuromuscular disorders has been rapidly


expanding. Traditionally, clinical assessment of patients with the neuromuscular disorders
starts with obtaining the history, clinical examination and is followed by an electrodiagnostic
test, which is a golden standard to assess the functional status of the peripheral
neuromuscular system. Nowadays, in addition to the assessment of neuromuscular function
using the traditional electrodiagnostic test, evaluation of the morphology of involved nerves
and muscles using US is performed in patients with a wide spectrum of neuromuscular
disorders. To get a better diagnosis, we need to increase our understanding of physiology and
morphology of peripheral nerve and muscle as assessed by electrodiagnostic test and US. In
this lecture, we will discuss about ultrasound images of nerves and muscles in upper extremity
according to the cross-sectional anatomic knowledge. In the extremities, nerves frequently run
with vessels and small arteries and can mimic nerve fascicles. Cross-sectional anatomy is a
key to locate target structure during ultrasound. 3- dimensional spatial relationship of nerve,
muscle, and vessels has specific pattern. The anatomic knowledge will help you to be an
expert in NM US.

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WS7.4. NMUS of the Brachial Plexus

H. Stephan Goedee1
1Brain Center Rudolf Magnus, University Medical Center Utrecht, Department of neurology,

Utrecht, The Netherlands

The brachial plexus can be involved in a variety of neuromuscular disorders, such as


inflammatory and hereditary demyelinating neuropathies, neurofibromatosis, traumatic injuries,
neoplasms, nerve sheath tumors. Traditionally, the evaluation of brachial plexus involvement
has relied on combination of clinical features, electrodiagnostic testing and MRI imaging.
Nerve imaging is an important and powerful add-on strategy, that can help to localize site of
lesion and uncover nature and extend of nerve involvement. The apparent lack of clear
association between nerve morphology and function further supports complementary role of
imaging modalities in routine diagnostic work-up of peripheral nerve disorders. Although MRI
has dedicated sequences that allow refined tissue discrimination, lack of objective cut-off
values hamper it’s used in the routine clinical setting.

Nerve ultrasound is an emerging practical bedside alternative to MRI, which the advantage of
flexible field of view, superior image resolution for superficial structures, broad availability and
relatively low cost. This neuromuscular ultrasound workshop will focus on main anatomical
targets for sonographic evaluation of brachial plexus, including important landmarks and notes
for discrimination. The workshop will also address standardized assessment and reference
values for abnormality, including sonographic examples of neuromuscular disorders.

WS7.5. Neuromuscular Ultrasound of the Lower Extremities

Joy Vijayan1
1Consultant, Division of Neurology, Department of Medicine, National University Hospital, Singapore.

Neuromuscular ultrasound is rapidly evolving as an excellent diagnostic tool in the evaluation


of peripheral neuropathies, especially so in focal neuropathies. It can be of use in
understanding the underlying pathological mechanism of neuropathies. Focal neuropathies are
more likely to be associated with structural abnormalities that can be easily recognized by
imaging modalities. Certain subtypes of polyneuropathies are also associated with
morphological changes. Point of care ultrasound helps with rapid examination of long
segments of multiple nerves in the out-patient setting. This can result in early triaging of
patients and rapid management decisions. Other advantages include easy patient tolerability
when compared to nerve conduction studies and lower cost. The sonographic abnormalities
that are commonly associated with neuropathies include focal or diffuse enlargement, hour-

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glass constriction, change in neural architecture, decreased mobility of the nerve and
increased vascularity of the nerves. It can also be of use in detecting extra-neural
abnormalities like tenosynovitis, soft tissue tumors, entrapment or compression from metallic
implants.

Peripheral nerves of the lower extremities that can be commonly assessed by sonographic
studies include the sciatic, peroneal and tibial nerve. Other less common nerves that are
assessed include the lateral femoral cutaneous nerve, the superficial and deep peroneal
nerves and the sural nerves. It can also be of value in the evaluation of the digital nerves in the
feet. Clinical conditions where sonographic studies can be of paramount importance include
evaluation of the peroneal nerve at the fibular head in patients presenting with foot drop and
assessment of the tibial nerve in the tarsal tunnel in those presenting with unilateral foot
numbness. Its use as a diagnostic tool has extended beyond focal neuropathies to include
polyneuropathies secondary to metabolic, inherited and inflammatory causes. Other uses of
lower extremity ultrasound include its role as a tool in guiding with therapeutic interventions like
steroid injections and detection of morphological changes within cutaneous nerves prior to
consideration of nerve biopsy.

WS7.6. Approach to US of Tongue and Cranial Nerves

Yu-ichi Noto1
1Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of

Medicine, Kyoto, Japan

Bulbar muscle (including the tongue muscle) ultrasound is used as an alternative tool for
detecting fasciculations in the diagnosis of ALS because the judgment of the presence of
fasciculation potentials in needle EMG examination is always difficult in the tongue muscle.
Also, bulbar muscle ultrasound is useful for the assessment of bulbar dysfunction and
dysphagia.

Nerve ultrasound can detect some cranial nerves. Sonographic optic nerve sheath assessment
may be useful in the diagnosis of intracranial hypertension and optic neuritis. Sonographic
facial nerve enlargement can be observed in patients with Bell’s palsy. Sonographic
identification of glossopharyngeal nerve is useful for glossopharyngeal nerve block. Vagus
nerve hypertrophy is seen in immune-mediated neuropathy and atrophy is observed in
Parkinson’s disease. The spinal accessory nerve also can be detected by nerve ultrasound.
Cranial nerve imaging by ultrasound can be a supplementary tool in the diagnosis and
assessment of some neurological diseases.

81
WS7.7. Overview of Quantitative Muscle Ultrasound

Nens van Alfen1


1Department of Neurology and Clinical Neurophysiology, Radboud university medical center,

Nijmegen, the Netherlands

The diagnosis and treatment of a patient with suspected neuromuscular disease requires an
integrated approach that utilizes clinical, electrodiagnostic, pathologic and genetic data.
Ultrasound complements the clinical and electrophysiology exam by showing the degree of
abnormalities in myopathies, as well as spontaneous muscle activities in motor neuron
diseases and other disorders.

US is particularly well suited for evaluation of neuromuscular disorders as it can be performed


at the bedside, has no need for sedation, can rapidly evaluate multiple body regions, and is
well tolerated by both children and adults including patients in wheelchairs or those who
cannot lay flat or still. Muscle ultrasound can be used as a biomarker for Duchenne and FSH
muscular dystrophy and spinal muscular atrophy, and the combination of electromyography
and ultrasound increases the diagnostic certainty of amyotrophic lateral sclerosis. Qualitative
visual analysis of muscle can be graded using the Heckmatt scale, based on the degree of
echogenicity and visualization of the deep boney structures.

Quantification of the gray scale levels can be performed by importing the ultrasound image into
an image analysis software program such as ImageJ, selecting a region of interest within the
muscle belly, and measuring the mean gray scale pixel values within the region of interest.
Quantitative analysis allows for more sensitive and specific determinations of muscle
pathology than qualitative analysis, with reported sensitivities for neuromuscular pathology
around 95%.

A drawback of quantitative muscle ultrasound is that it requires that imaging settings be held
constant and that normative values have been obtained using identical imaging parameters. A
strong advantage of ultrasound over other imaging modalities is that evaluation in real time
permits the identification of pathologic muscle movement, particularly fasciculations.

82
WS7.8. Neuromuscular Ultrasound Demonstration

Nens van Alfen1


1Department of Neurology and Clinical Neurophysiology, Radboud university medical center,

Nijmegen, the Netherlands

In this demonstration, Dr Nens van Alfen will demonstrate the basic approach to
neuromuscular ultrasound.

WS7.9. Imaging as a Natural Complementary Test in Neuromuscular


Medicine

Einar Wilder-Smith1
1Kantonsspital Luzern, University of Berne, Switzerland

Advances in the understanding of peripheral nerve disorders occur when new techniques allow
a better insight into the underlying pathophysiology. Functional tests have to date led the way
in furthering our understanding of peripheral nerve disorders. EMG was rapidly followed by
nerve conduction studies and further technical refinements enabled the use of evoked
potentials, intraneural microneurography and threshold tracking. Similar to what happened in
the exploration of the central nervous system, there was a gap in the field of Neuromuscular
medicine until clinically useful imaging of the peripheral nerves emerged.

Because of its ready availability, excellent resolution and relative low cost, peripheral nerve
Ultrasound has rapidly become part of clinical routine. A particular domain of peripheral nerve
MRI scanning is the depiction of neural structure lying deep and behind bone such as the
lumbosacral plexus. In this review, we will be looking at how imaging techniques compliments
functional tests and provides a comprehensive evaluation of the peripheral nervous system.

WS7.10. Neuromuscular Ultrasound in Focal Neuropathies

Joy Vijayan1
1Consultant, Division of Neurology, Department of Medicine, National University Hospital, Singapore.

Neuromuscular ultrasound is rapidly evolving as an excellent diagnostic tool in the evaluation


of focal neuropathies. It can also be of use in understanding the underlying pathological
mechanism of focal neuropathies. Unlike length-dependent polyneuropathies, focal
neuropathies are more likely to be associated with structural abnormalities that can be easily

83
recognized by imaging modalities. Point of care ultrasound helps with rapid examination of
long segments of multiple nerves in the out-patient setting. This can result in early triaging of
patients and rapid management decisions. Other advantages include easy patient tolerability
when compared to nerve conduction studies and lower cost. The sonographic abnormalities
that are commonly associated with focal neuropathies include focal enlargement, hour-glass
constriction, change in neural architecture, decreased mobility of the nerve or increased
vascularity.

Neuromuscular ultrasound is particularly helpful with the correct localization of focal


entrapment neuropathies. It can help also in the detection of dynamic compression. Its role as
a diagnostic tool has extended beyond entrapment neuropathies to include traumatic
neuropathies, infective and non-infective inflammatory neuropathies and peripheral nerve
sheath tumors. It can also be of use in detecting extra-neural abnormalities like tenosynovitis,
soft tissue tumors, entrapment or compression from metallic implants. Other lesser recognized
uses include the detection of anatomical variants like bifid median nerves, persistent median
artery, anomalous muscles and ulnar nerve subluxation at the elbow. Pathologies of the
brachial plexus like inflammatory plexitis and thoracic outlet syndrome can also be detected by
sonographic studies.

The use of neuromuscular ultrasound can be complemented when used in conjunction with
nerve conduction studies. Neural changes can be detected by sonographic studies very early
into the disease at sites of focal pathologies like conduction block or temporal dispersion.
Recent uses of ultrasound include its role as a tool in guiding with the therapeutic interventions
for focal focal neuropathies. These include administration of steroids or local anesthetics in
entrapment neuropathies or in those patients with neuralgic pain. The latter include carpal
tunnel syndrome, meralgia paresthetica or other lesser known painful neuralgic disorders.

WS7.11. NMUS in Inherited and Inflammatory Neuropathies (Encore)

H. Stephan Goedee1
1Brain Center Rudolf Magnus, University Medical Center Utrecht, Department of neurology,

Utrecht, The Netherlands

Traditionally, the evaluation of inflammatory and hereditary neuropathies has mainly relied on
combination of clinical features and electrodiagnostic testing. Although MRI has previously
been suggested as potential helpful add-on strategy in neuropathy diagnosis, the lack of
objective cut-off values hamper it’s used in the routine clinical setting. Nerve ultrasound is an
emerging and powerful diagnostic tool, that has proven to be highly sensitive and offer an
important complement to routine electrodiagnosis. It has several advantages over MRI, such
as the availability of reference values, flexible field of view, superior image resolution, broad
availability and relatively low cost. Since the first descriptions of nerve ultrasound findings in
neuropathies, several sonographic studies have been published. This course will review the
available literature and experience of nerve ultrasound in inherited and inflammatory

84
neuropathies. The use and implementation of different sonographic protocols and scoring
systems will be discussed in detail, including a practical sonographic protocol and
improvement in diagnostic yield. Also, the apparent lack of clear association between nerve
morphology and function in neuropathies will be addressed. The course will also highlight
several pitfalls and relevant imaging mimics.

WS7.12. The Role of Quantitative Muscle Ultrasound in Disease

Nens van Alfen1


1Department of Neurology and Clinical Neurophysiology, Radboud university medical center,

Nijmegen, the Netherlands

Quantitative Muscle Ultrasound (QMUS) is a valid and reliable diagnostic imaging technique
for the evaluation of nerves and muscles. Muscle ultrasound is currently used for both
diagnostic screening and biomarker follow up purposes. A recent systematic review based on
publications from 2000 to 2014 confirmed the utility and acceptability of US for the diagnosis of
pediatric skeletal muscle disorders, with high sensitivity and specificity of around 90%-95%. In
comparison to electromyography (EMG), muscle US is better tolerated. In addition, the
distribution of US abnormalities as well as the pattern of muscle involvement can aid in the
diagnosis of NMD.

In QMUS both echogenicity and muscle diameter are routinely quantified. Echogenicity
increase is the most robust and reproducible measure of muscle pathology, while muscle
atrophy alone has a low diagnostic accuracy for detecting neuromuscular disease and
discrimination between neurogenic disorders and myopathies. However, a combined cut-off
based on echogenicity and muscle thickness can detect neurogenic disorders with a sensitivity
of 67%, a specificity of 94%, a PPV of 86%, and a NPV of 84%

QMUS is routinely supplemented with dynamic screening of muscle movements, and


ultrasound is better at detecting fasciculations than using needle EMG screening or clinical
examination. The combination of US and EMG increased the diagnostic category of probable
or definite ALS from 48% to 79%. In ALS patients who had fasciculations, a score using the
US results of 9 muscles can discriminate between ALS and ALS-mimics with 92% sensitivity
and 100% specificity.

In practice the author advocates the use of muscle US as a first line screening tool when a
neuromuscular disorder is suspected. Muscle ultrasound is not a replacement to the
electrodiagnostic examination. As EMG is a measurement of function, and ultrasound is
concerned with structure, the use of both techniques is complementary, similar to EEG and
MRI or CT. Like the electrodiagnostic exam, neuromuscular ultrasound yields the best
information when it is informed by the history, examination, and by an examiner with

85
knowledge of the patterns of neuromuscular disease. When used together, ultrasound and
EMG provide a more satisfactory and complete evaluation than either alone.

86
Meet-The-Expert (ME)
ME1. Sleep Disorders in Parkinson’s Disease
Claudia Trenkwalder
1University Medical Center Göttingen, Germany

Sleep disorders are among the most frequent non-motor symptoms in parkinsonism. As many
as 60% patients with Parkinson’s disease (PD) suffer from insomnia, 15-59% from REM sleep
behavior disorders and 30% from excessive daytime sleepiness. Further nocturnal problems in
PD patients include insomnia, painful dystonia, restless legs syndrome, dysuria, anxiety,
depression or nocturnal hallucinations. REM sleep behavior disorder (RBD), a pre-
symptomatic feature of neurodegeneration, with abrupt movements during REM periods,
vocalizations and sometimes violent, enacted dreaming exposes the patients or their bed-
partners to night-time injuries and is probably caused by lesions in the locus coeruleus and
pedunculi-pontine area. Daytime sleepiness and a narcolepsy-like phenotype are also part of
sleep-wake dysregulations in Parkinsonism. Sleep disordered breathing and pharmacological
effects of dopaminergic therapy may furthermore disturb the initiation and maintenance of
sleep, enhancing excessive daytime sleepiness. Thus, sleep disorders represent an important,
multifactorial problem of the disease. A thorough diagnostic workup including interviews with
the patient and his/her bedpartner as well as video-supported polysomnography can establish
the specific sleep diagnosis. Sleep scales developed for PD as the Parkinson Disease Sleep
Scale (PDSS-2) may further explore the various domains affected and thus facilitate
therapeutic decisions. Treatment strategies include sufficient nocturnal dopaminergic
stimulation such as levodopa sustained release or rotigotine patch for treating nocturnal
akinesia, zopiclone ort melatonin for insomnia, clozapine against nocturnal hallucinations, and
dopaminergic agents or even opioids for restless legs syndrome. RBD may be treated with
clonazepam, although a current RCT did not provide evidence for efficacy. CPAP treatment
can be applied in PD patients with moderate to severe obstructive sleep disorders breathing.

87
ME2. My Practical Approach to the Electrodiagnosis of Peripheral
Neuropathies
Jun Kimura1
1Department of Neurology University of Iowa, Iowa City, IA, USA

Polyneuropathy consists of the triad of sensory changes in a glove and stocking distribution, distal
weakness, and hyporeflexia. Certain types of neuropathy may show widespread sensory
symptoms, and others may begin with more prominent proximal weakness. Positive sensory
symptoms result from ectopic impulse generation and auto excitation of myelinated afferent fibers.
In general, but not always, normal muscle stretch reflexes speak against peripheral neuropathy.
Acute pandysautonomic neuropathy characteristically shows severe postganglionic sympathetic
and parasympathetic dysfunction, with relative or complete sparing of motor and sensory function.
Milder autonomic dysfunction also accompanies most peripheral neuropathies, but manifests
clinically detectable symptoms only in a few conditions, such as diabetes, amyloidosis, Guillain-
Barre syndrome, porphyria, and familial dysautonomia.

A detailed history often reveals general medical conditions such as diabetes, alcoholism, renal
disease, malignancies, sarcoidosis, polyarteritis nodosa, amyloidosis and infectious processes
such as diphtheria and leprosy. Inflammatory neuropathies include the Guillain-Barre syndrome
and chronic inflammatory demyelinative neuropathy. Metabolic neuropathies result from nutritional
deficiencies or the toxic effects of drugs or chemicals. The family history is essential in establishing
the type of inherited conditions associated with polyneuropathy. Sometimes a patient's own
account may not provide enough information, necessitating independent examination of family
members. For some patients with an unequivocal diagnosis of polyneuropathy, extensive studies
may fail to uncover the exact etiology. Hereditary and immune mediated polyneuropathy account
for most such cases.

Anatomic diagnosis depends on clinical and electrodiagnostic evaluation, but few specific patterns
of peripheral nerve involvement characterize a given disorder. Nerve conduction and
electromyographic studies delineate the extent and distribution of the lesions and differentiate two
major pathologic changes in the nerve; axonal degeneration and demyelination. An index based on
multiple electrophysiologic measures against standard norms may provide a better overall
estimation as reported in the assessment of diabetic polyneuropathy. Electrodiagnosis, as
discussed in detail during our workshop, helps delineate the locations and patterns of
abnormalities, but these studies alone rarely distinguish clinical types of neuropathies or establish
the exact etiology in a given case. Arriving at a specific diagnosis and establishing a course of
therapy depend heavily on clinical, electrophysiologic and histologic assessments.

88
ME3. First-onset seizures - An Update on the Concept and Review of Best
Practice
Margitta Seeck1
1EEG & Epilepsy Unit, University Hospital of Geneva, Switzerland

The diagnostic work-up of a first seizure or seizure-like event (FS) is extremely important to
determine rapidly its origin, the optimal therapy and prognosis. FS affect patients of all ages.
Together with stroke, seizures are one of the most frequent neurological emergencies,
associated with an increased morbidity and mortality. In order to obtain a proper diagnosis,
EEG, MRI, and according to clinical hypothesis, other exams need to carried out. It has been
shown in several studies that the yield is higher if exams are carried out as early as possible.
The prognosis and further adherence will crucially depend on the first contact. I will present
evidence for these assumptions and examples of cases.

The importance of precise diagnosis and efficient therapy has been recognized in prospective
and retrospective studies, leading to evidence-based guidelines by the Academy of Neurology
in 2015 (Krumholz Neurology 2015) as well as several commissions inside the ILAE and IFCN.
By 5 years or more, 50-70% present seizure recurrence, suggesting that antiepileptic drugs fail
in the majority of patients. Reasons for drug failures need to be examined as well, as careful
as the index event. In most centers, work-up in the emergency department (ED) includes often
only a medical/neurological consultation, standard EEG and CT, leaving the organization of
further exams to the discretion of the treating neurologist.

Hopefully, a comprehensive and swift work-up after FS will become standard in centers around
the world dealing with FS patients.

89
Oral Abstracts

90
Electroencephalogram (EEG)

EG001. STRATIFIED OUTCOME ANALYSES OF THE QUANTITATIVE


BURST SUPPRESSION RATIO IN REFRACTORY STATUS EPILEPTICUS

Neil Mehdiratta*1 ; Shenghua Zhu2 ; Marcus Ng1


1Internal Medicine (Neurology), University of Manitoba, Canada, 2Radiology, University of

Ottawa, Canada.
Email Address: mng2@hsc.mb.ca

Introduction: In refractory status epilepticus (RSE), seizures are generally managed by


anaesthetic sedation. Although sedation depth correlates with EEG suppression level, the
optimal degree of suppression is unknown. We sought to assess the relationship between the
‘quantitative burst suppression ratio’ (QBSR) as a marker of EEG suppression, and RSE
patient outcomes.

Methods: We used Persyst software to calculate QBSR/10-second EEG epoch from non-
anoxic adult RSE intensive care unit (ICU) patients over 15 months. QBSR was the
percentage of each epoch with EEG amplitude under 3 microvolts/millimetre. Between
unfavourable and favourable (defined as surviving to discharge from ICU) groups, we
compared median QBSR per-patient (multivariate regression), per-daily EEG recording
session (generalized estimating equation), and per-EEG epoch (quasi-least squared model)
using Stata 14 or MATLAB software to control for aSTESS (adjusted status epilepticus severity
score), sedative number, and gender.

Results: 135,765 QBSRs were calculated from 158 EEGs derived from 17 patients. There
were 7 favourable group patients who all survived 3 months post-discharge from ICU.
Stratifying analyses per-patient, per-session, and per-epoch, high aSTESS is consistently
associated with unfavourable outcome (per-patient OR 0.59-0.97, p=0.033; per-session OR
0.06-0.94, p=0.041; per-epoch OR 0.67- 0.71, p<0.001). On per-epoch analysis, greater
sedative use is associated with favourable outcome (OR 1.45-1.54, p<0.001) while gender
does not associate with outcome (OR 1-1, p<0.001). On all analyses, there was no significant
association between QBSR and outcome.

Conclusion: Our findings validate the prognostic value of aSTESS, but do not support an
association between RSE outcomes and EEG suppression depth measured by QBSR.
However, an association between more sedative use and better outcome on per-epoch
analysis may suggest that more aggressive treatment targeted against foreground transients
such as seizures may be more beneficial in RSE than quantitative background suppression.

91
EG002. 8-YEAR EXPERIENCE OF STEREOELECTROENCEPHALOGRAPHY
AT WESTMEAD HOSPITAL AND THE CHILDREN’S HOSPITAL AT
WESTMEAD

Thienan John Phamnguyen*1 ; Mark Dexter2 ; Andrew Bleasel1 ; Deepak Gill3 ; Sachin
Gupta3 ; Manori Wijayath1 ; Zebunnessa Rahman1 ; Melissa Bartley1 ; Chong Wong1
1Epilepsy, Westmead Hospital, Australia 2Neurosurgery, Westmead Hospital, Australia,
3Epilepsy, The Children's Hospital at Westmead, Australia

Email Address: j.thienan@gmail.com

Introduction: We report our surgical series of intractable epilepsies investigated with


stereoelectroencephalography (SEEG) at Westmead Hospital and The Children’s Hospital at
Westmead; evaluating the methodology, complication rate and seizure outcome from an 8-
year program.

Methods: 82 SEEG were performed in 80 patients (males, n= 45) at Westmead Hospital and
The Children’s Hospital at Westmead from 2012 to 2019. All SEEG were performed using
frame-based stereotaxy with CRW (Integra, Plainsboro, NJ, USA) frame systems. Clinical
demographics, methodology, outcomes and complications were collected and analysed.

Results: Mean age of patients was 22 years old (range 4-49 years old). Two patients had two
SEEG implantations because the initial implantation did not provide sufficient information to
inform resection. Fifty-six patients had normal MRI. A total of 1132 electrodes were inserted in
82 SEEG procedures (mean number of electrodes per SEEG = 14, range 8-21; total number of
electrode contact = 14611). Majority of patients had unilateral implantation (left = 24, right = 32,
bilateral = 24). Complications occurred in four patients (subdural haemorrhage = 3, fractured
electrode =1). All patients were able to proceed with VEEG monitoring after electrode
implantation. Seizures were recorded in 81 SEEG studies. One patient did not have any
seizures during the evaluation. 72% of patients underwent resective surgery following SEEG.
Two patients were offered resective surgery but had declined. 26% of patients were not
localised. 13 patients were awaiting surgery or pending conference decision. 74% became
seizure free after resective surgery.

Conclusion: SEEG can be a relative safe and effective methodology for defining the
epileptogenic zone

92
EG003. CAN SPIKE VOLTAGE TOPOGRAPHY PREDICT THE ICTAL
PATTERNS IN TEMPORAL LOBE EPILEPSY?

Ashalatha Radhakrishnan*1 ; Ramshekhar Menon1 ; Sanjeev V.Thomas1


1Epilepsy Division, Neurology, Sree Chitra Tirunal Institute for Medical Sciences and

Technology, India
Email Address: drashalatha@sctimst.ac.in

Introduction: To study whether voltage topographic analysis of interictal epileptiform


discharges (IEDs) in temporal lobe epilepsy (TLE) help to predict the type of scalp ictal onset
pattern.

Methods: IEDs from 74 patients with drug resistant TLE and unilateral mesial temporal
sclerosis (MTS) who underwent presurgical evaluation were categorized according to dipolar
fields into Types A and B temporal IEDs. Type A IEDs were defined as those with dipolar fields
(tangential/ vertical) with sharply defined negativity over the infero-lateral temporal scalp and
positivity over contra lateral centro-parietal scalp. Type B IEDs included all other IEDs other
than Type A. Scalp ictal electrographic patterns were classified into rhythms confined to
temporal lobe ipsilateral to MTS (“focal”), lateralized to the hemisphere (“regional”), and
diffuse, nonlateralized and non-localized rhythms (“diffuse”) and ictal onset to the contra lateral
side of MTS (“discordant”).

Results: A total of 5476 IEDs and 377 seizures were analyzed. They were divided into four
categories based on the side of MTS: 1) Type A IEDs ipsilateral to MTS (44 patients); 2)
bilateral Type A IEDs (16 patients); 3) Type B IEDs ipsilateral and Type A IEDs contra laterally
(7 patients); 4) Bilateral type B IEDs (7 patients). In 51 of 60 (85%) patients with Type A IEDs
(either ipsilateral and/or bilateral to MTS), the ictal pattern was either focal or regional and only
in 9 patients (15%) it was “diffuse”.12 of 14 (86%) patients with either ipsilateral or bilateral
Type B IEDs showed a diffuse ictal onset, whereas 2 (14%) showed a contralateral ictal onset.

Conclusion: In MTS, Type A IEDs are associated with clearly localized ictal onset and Type B
IEDs with diffuse ictal onset. A spikevoltage topographic analysis is sufficient for selection of
cases for antero-medial temporal lobe resection. Additional home-video recordings of seizures
can rule out psychogenic non-epileptiform events.

93
EG004. COMPARING THE ACCURACY OF SLEEP STAGING DATA FROM A
NEW WEARABLE SLEEP ELECTROENCEPHALOGRAPHY DEVICE VERSUS
THE FITBIT CHARGE 3 WITH POLYSOMNOGRAPHY AS REFERENCE

Yoko Suzuki*1 ; Takashi Abe1 ; Fusae Kawana2 ; Satomi Okabe3 ; Toshio Kokubo1 ; Kazuya
Hoshino4 ; Misao Baba4 ; Masaaki Fujiwara4 ; Masashi Yanagisawa1
1International Institute for Integrative Sleep Medicine (WPI-IIIS), University of Tsukuba, Japan,
2Graduate School of Medicine, Juntendo University, Japan, 3Graduate School of

Comprehensive Human Sciences, Kansei, University of Tsukuba, Japan, 4S’UIMIN Inc.,


University of Tsukuba, Japan.
Email Address: yoko.12.suzu@gmail.com

Introduction: Although sleep disorders are a common social problem, polysomnography


(PSG), the gold standard in sleep diagnostics, can be burdensome and may interfere with
natural sleep. We have developed a high-precision wearable electroencephalography (EEG)
device for objectively measuring sleep at home. Actigraphy devices with the capability of
measuring heart rate variability can also be used to evaluate sleep stages at home (e.g., Fitbit
Charge 3). However, the accuracy of these inhome devices has not yet been compared. We
hypothesized that the accuracy of our wearable sleep EEG device would be higher than that of
the Fitbit Charge 3.

Methods: We simultaneously recorded PSG, wearable sleep EEG, and data from the Fitbit
Charge 3 in 42 (mean ± SD: 23.5 ± 7.8 years, 14 women) healthy participants. A registered
polysomnographic technologist scored the PSG and wearable sleep EEG data, and we
evaluated the sleep stages captured by the Fitbit Charge 3 using the Fitbit automatic sleep
staging algorithm (stage W, N1+N2, N3, and R). We measured and compared the accuracy of
the wearable EEG device and Fitbit Charge 3 data with respect to the PSG data.

Results: The accuracy of the wearable sleep EEG device and Fitbit Charge 3 was 90.7 ± 2.8
% and 69.5 ± 8.3 %, respectively (mean ± SD). The wearable sleep EEG device had
significantly higher accuracy than the Fitbit (p < 0.001).

Conclusion: Compared with the Fitbit Charge 3 data, data from the wearable sleep EEG
device more closely mirrored the PSG data. Thus, the wearable sleep EEG device may be
superior to the Fitbit Charge 3 as an alternative to PSG. Our wearable sleep EEG device has
potential for use in sleep evaluations for multiple consecutive nights in an in-home setting.

94
Epilepsy

EP002. EPILEPTIC ADOLESCENTS AND RAMADAN FASTING: A


PROSPECTIVE COHORT STUDY

Amira Darwish*1
1Pediatrics , Tanta university, Egypt

Email Address: amira.darwish@med.tanta.edu.eg

Introduction: The prevalence of epilepsy in childhood is 3 to 5.5/1000 worldwide and 8/1000


in Arab countries, including Egypt. All healthy Muslims are obligated to fast the month of
Ramadan when they reach puberty as Ramadan fasting is one of the five pillars of Islam. Aim:
To evaluate the effect of Ramadan fasting on seizure control in epileptic adolescents.

Methods: A prospective cohort study that included 56 epileptic patients aged 11-18 years.
Patients were followed-up in the pediatric neurology clinic at Tanta University Children's
Hospital during Ramadan 2019. Seizures frequency and severity, measured by Chalfont
Seizure Severity Scale, during Ramadan fasting were compared to those in the month before
Ramadan.

Results: Before Ramadan, 43/56 patients were controlled and seizure-free. Meanwhile, 13/56
patients were uncontrolled and had seizure frequency of 1-6 seizures in the month before
Ramadan. There was no significant difference in seizure frequency during Ramadan compared
to before Ramadan (p=0.132), whereas seizure severity was significantly reduced during
Ramadan (p=0.028). Both seizure frequency and severity were significantly lower during
Ramadan in uncontrolled epileptic patients compared to before Ramadan (p= 0.02 & 0.005).

Conclusion: Ramadan fasting is safe in adolescents with epilepsy. Fasting is not a


precipitating factor for seizures in epileptic adolescents, provided compliance to anti-epileptic
drugs, and could be beneficial for seizure control in uncontrolled epilepsy.

95
EP003. EPILEPSY AND ALZHEIMER DISEASE

Abbasher Hussien*1 ; Amira Sidig2 ; Khabab Abbasher3 ; Hussien Abbasher4


1Internal Medicine, University of Khartoum Faculty of medicine, Sudan, 2Community Medicine,

Al Neilain University faculty of medicine, Sudan, 3MBBS student, University of Khartoum


Faculty of medicine, Sudan, 4MBBS student, AlYarmouk college, Sudan.
Email Address: Khabab9722@gmail.com

Introduction: An increased prevalence of epilepsy had been documented with dementia.


Alzheimer’s disease and epilepsy often coexist.

Methods: We flowed 480 patients aged more than 65 years with diagnosis of Alzheimer’s
disease between May 2006 and May 2019 looking for coexist epilepsy.

Results: Regarding Alzheimer’s disease female were affected more than male (60%).10 % of
our patients have epilepsy. Generalize epilepsy was the most common type (62%). Epilepsy
was more common with late onset Alzheimer’s disease. Abnormal EEG was detected in 20%
of our studied group. Abnormal Brain MRI in form of cerebral atrophy was observed in 60 % of
patients with Alzheimer’s disease and epilepsy.

Conclusion: Patients with Alzheimer’s disease have an increased risk of developing epilepsy.
There is strong relation between disease duration and development of epilepsy.

96
EP004. TARGETING EPILEPSY WITH NOVEL THERAPEUTIC ENTITIES TO
INVESTIGATE THE BIOCHEMICAL AND MOLECULAR MECHANISMS

Jitendra Kumar Sinha*1


1Amity Institute of Neuropsychology and Neurosciences (AINN), Amity University UP, India

Email Address: jitendrakumarsinha@gmail.com

Introduction: Analysis of anticonvulsant action of novel therapeutic entity (NTE) in kindling


(Pentylenetetrazole (PTZ)) and acute (Kainic Acid (KA)) seizure mice (male swiss albino)
models.

Methods: The NTE (NP-VM1) was screened from a class of 5 hydrazone derivatives of 2,4-
disubstituted thiazole through subcutaneous PTZ and neurotoxicity screening followed by log-
probit analysis for determining ED50, TD50 and protective-index (PI). Neuroprotective potential
of the NTE was analyzed in both models and was validated through behavioral screening tests
and oxidative stress estimation (MDA, GPx and SOD levels). Results were analyzed by
ANOVA single factor and Tukey’s post-hoc test.

Results: NP-VM1 showed significant biological activity in screening. It significantly reduced the
seizure activity in both models. Significant increase in the spatial learning ability in Morris
Water Maze test was further confirmed by increased recognition index in Object Recognition
Test. Reduced transfer latency and time spent in open arm in Elevated Plus Maze test along
with increased rearings and movement through the centre in Open Field Test signified
restoration of behavior normalcy and locomotion ability. Rotarod test showed that NTE
significantly negated the neurotoxic and ataxic effects which was further proved by enhanced
GPx and SOD levels and attenuated MDA levels in the hippocampus. The results of sucrose
consumption test were however not statistically significant.

Conclusion: NP-VM1 significantly augmented the ROS detoxification thereby fortifying the
neurons from chronic damage, which was evident by the restoration of behavioral normalcy
and learning, motor coordination and locomotion abilities.

97
Transcranial Stimulation (TCS)

TS002. COMBINING SICF AND ICF WITH QPS BOOSTS EXCITABILITY


INCREASE IN THE PRIMARY MOTOR CORTEX

Yuichiro Shirota*1 ; Martin Sommer2 ; Walter Paulus2


1Neurology, The University of Tokyo Hospital, Japan, 2Clinical Neurophysiology, University

Hospital Göttingen, Germany.


Email Address: you_shirota@yahoo.co.jp

Introduction: After-effects induced by repetitive transcranial magnetic stimulation (rTMS) at


the primary motor cortex (M1) are usually indexed by the amplitude of the motor evoked
potential (MEP). They depend on various parameters, in particular on the temporal pattern.
Quadripulse stimulation (QPS; high-frequency train of four pulses delivered every five seconds
for 30 minutes) was originally invented based on a repetitive paired-pulse TMS (ppTMS)
protocol, which utilized short-interval intracortical facilitation (SICF) as a building block. Here,
we tried to test whether after-effects of QPS can be increased by integrating either SICF,
intracortical facilitation (ICF) or long-interval intracortical inhibition (LICI).

Methods: Three conditions were compared using a cross-over design in 23 healthy


volunteers. The three conditions were SICF+ICF, SICF+LICI, and QPS-like. In SICF+ICF, two
pulses with a 1.5 ms inter-stimulus interval (ISI) were repeated twice, separated by 10 ms
(ICF). Likewise, in SICF+LICI two pulses with a 1.5 ms interval were repeated at 100-ms
interval (LICI). In the QPS-like condition, the four pulses were evenly delivered at 5-ms
intervals. In all three conditions the four pulses were repeated every five seconds for 10
minutes. Amplitude of MEP elicited by single pulse TMS was measured before the
intervention, and up to 60 minutes after it. The MEP amplitudes were normalized to the
baseline and served as the main outcome.

Results: The combination of two facilitatory ppTMS, SICF+ICF, resulted in significantly


increased MEP amplitudes over time whereas combining the facilitatory SICF with the
inhibitory LICI tended to decrease the MEP amplitude. QPS with 10-minute intervention time
had no effect in contrast to the 3-times longer original QPS paradigm.

Conclusion: The efficacy of the original QPS can be maintained with one third of stimulation
time when differently organized in time along with optimal values for SICF+ICF.

98
TS003. THE COMBINED EFFECT OF REPETITIVE TRANSCRANIAL
MAGNETIC STIMULATION (RTMS) AND TREADMILL TRAINING ON
WALKING ENDURANCE AND BALANCE IN PEOPLE WITH PARKINSON’S
DISEASE (PD) – A RANDOMISED PLACEBO-CONTROLLED TRIAL

Chloe lauha Chung*1; Margaret Kit Yi Mak2


1Rehabilitation Sciences, The Hong Kong Polytechnic University, Singapore, 2Rehabilitation

Sciences, The Hong Kong Polytechnic University, China.


Email Address: chloe.lh.chung@connect.polyu.hk

Introduction: Reduced walking endurance and balance is common among patients with PD.
Treadmill training and rTMS have been shown to be effective in improving walking
performance in patients with PD, but effects were modest and short-lived. Long-term effect of
combined rTMS and treadmill training on walking endurance and balance performance in
patients with PD is not known.

Methods: 34 participants with PD were randomised into 2 groups. Participants received 12


sessions of either 25 Hz rTMS and treadmill training (25Hz-TT) or sham rTMS and treadmill
training (1Hz-TT) conducted over 3 weeks. For 25Hz-TT group, 600 TMS stimuli at 80%
resting motor threshold were delivered to leg area of motor cortex of each hemisphere. Two-
minute walk test (2MWT) and mini-Balance Evaluation System Test (mini-BEST) were used to
evaluate the efficacy of interventions on walking tolerance and balance performance.
Participants were tested before, after, 1 month and 3 months after intervention ended. Two-
way repeated measure ANOVA was used to evaluate the outcome data. SPSS 23.0 was used
for all statistical analysis.

Results: Significant interaction effect was found in mini-BEST (F (3,32) =3.247, p=0.041).
Post-hoc analysis shows that only 25-Hz group improved mini-BEST at Post (p=0.001), 1-
month (p=0.006) and 3-month post intervention (p=0.036). Between-group analysis of change
from baseline showed that 25Hz-TT had a greater change than sham-TT (by 1.96, p=0.01) in
improving mini-BEST. Both 25Hz-TT and sham-TT groups improved 2MWT at all
measurement time points (p<0.001).

Conclusion: Our results showed that combining 25-Hz rTMS with treadmill training improved
walking endurance and balance performance in people with PD in both short-and long-term.

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TS004. SITE-SPECIFIC DECREASE IN CORTICAL REACTIVITY DURING
SENSORY TRICK IN CERVICAL DYSTONIA PATIENTS

Nivethida Thirugnanasambandam*
Department of Neurology, National Institute of Mental Health and Neuro Sciences, India
Email Address: dr.nivethida@gmail.com

Introduction: Sensory tricks (SeT) are various maneuvers that can alleviate dystonic
contractions and are a characteristic feature of cervical dystonia (CD). The neurophysiology
underlying SeT, however, still remains largely unknown. Reducing the abnormal cortical
facilitation and modulating the abnormal cortical and subcortical oscillatory activity are likely
mechanisms that have been proposed. The supplementary motor area (SMA) and primary
sensorimotor cortices are thought to be involved in this phenomenon. In the current study,
using concurrent EEG recording during transcranial magnetic stimulation (TMS) of the SMA
and primary motor cortex (M1), we aimed at determining the changes in cortical reactivity and
oscillatory activity induced by SeT.

Method: We recruited thirteen patients with CD who exhibited SeT and equal number of age-
and gender-matched healthy controls. Single TMS pulses were delivered over the SMA and
M1 either at rest or during SeT. 32-channel EEG was recorded and TMS-evoked potentials
were obtained. Further, time-frequency analysis was performed on the induced data.
Correlation analysis for significant neurophysiological parameters was done with clinical
measures.

Results: In the current study, we found that SeT induced a significant decrease in the
amplitude of TEP elicited from M1 stimulation at ~210-260ms in patients, which correlated with
symptom duration. Post hoc analysis of EMG activity in the neck muscles revealed that this
effect on TEP was present only in the subset of patients with effective SeT.

Conclusion: Our results suggest that SeT reduces cortical reactivity over M1 approximately
200ms after stimulation. This adds support to the idea that reduced cortical facilitation
underlies the phenomenon.

100
TS005. SIGNIFICANT EFFECTS ON FUNCTIONAL INTEGRATION WITHIN
THE PARIETAL MEMORY NETWORK AFTER HIGH-FREQUENCY RTMS
OVER THE L-DLPFC IN PATIENTS WITH ALZHEIMER’S DISEASE

Xin Liu*1 ; Houcang Yue2 ; Yu-jun Li2 ; Xiaoming Wang3


1Neurology Department, Affiliated Hospital & Clinical Medical College of Chengdu, China,
2Neurology Department, North Sichuan Medical College, China, 3Neurology Department,

Affiliated Hospital of North Sichuan Medical College, China.

Introduction: High-frequency repetitive transcranial magnetic stimulation (rTMS) improves


cognitive function in patients with Alzheimer's disease (AD), although the underlying
mechanism is unclear. This study aimed to investigate the effects of rTMS treatment on brain
networks in patients with AD using fMRI.

Methods: Twenty-two patients with mild–to-moderate AD were recruited and randomly


assigned to two groups matched for gender and age: the treatment group (TG,12 subjects),
which received real therapy with 10Hz rTMS over the left dorsolateral prefrontal cortex (L-
DLPFC) for two weeks , and the sham control group (SG,10 patients), which received sham
stimulation therapy. All patients were assessed using cognitive measurements and fMRI at
baseline and after treatment. A data-driven method, functional connectivity density (FCD)
mapping, was used to evaluate the effects of rTMS on the brain in patients with AD. Additional
areas with significantly different FCDs were selected as seeds to calculate functional
connectivity.

Results: Compared with baseline values, TG showed significantly increased FCD in the
bilateral precuneus (PCu), left inferior parietal gyrus (LIPG) and left triangular part of the
inferior frontal gyrus (LIFG) after rTMS, but FCD in SG remained unchanged. The FCD in the
left superior frontal gyrus (LSFG) significantly decreased in TG after rTMS. The bilateral PCu
demonstrated increased FC with LSFG in TG. In addition, increased FC between RPCu and
LIPG was also observed after treatment. TG also showed improved performance on MMSE
and DST and NPI, ADL and ADAS-cog after treatment (p<0.05).

Conclusion: These findings demonstrated that 10Hz rTMS over L-DLPFC had significant
effects on functional integration within the parietal memory network (PMN) and its coupling
with the site of stimulation in AD. The results suggest that rTMS can improve cognitive function
in AD. Moreover, our study supported the usefulness of rTMS and functional connectivity as a
potential measure to investigate brain networks in AD.

101
Nerve Conduction Studies (NCS) and Electromyography
(EMG)
NE001. CORRELATION OF NERVE CONDUCTION VELOCITY WITH BODY
FAT MASS AND OXIDATIVE STRESS MARKERS IN TYPE 2 DIABETIC
NEUROPATHY PATIENTS

Anwar Hasan siddiqui*1 ; Faique Rahman2 ; Sangeeta Singhal1 ; Ahmad Faraz1 ; Hamid
Ashraf3
1Physiology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, India,
2MBBS IInd Professional, Jawaharlal Nehru Medical College, Aligarh Muslim University,

Aligarh, India, 3Rajiv Gandhi Centre for Diabetes and endocrinology, Aligarh Muslim University,
India.
Email Address: only.anwar@gmail.com

Introduction: Diabetic peripheral neuropathy (DPN) is the most common but least recognised
and understood long term complication of type 2 diabetic patients (T2DM). There is a close
association between obesity and type 2 diabetes as well as its complications. Nerve
conduction study is an important non-invasive diagnostic modality used in the detection of
diabetic peripheral neuropathy. Obesity is associated with increased oxidative stress and lipid
peroxidation is thought to play a crucial role in the development of diabetic neuropathy.The aim
of this study is to see the correlation of nerve conduction velocity parameters viz nerve
conduction velocity and nerve conduction amplitude with the total body fat mass and oxidative
stress markers.

Methods: The study included 90 type 2 diabetes patients with clinically suggestible symptoms
of diabetic peripheral neuropathy (DPN). 50 type 2 diabetic subjects without peripheral
neuropathy served as control. Total body fat mass was measured using bioelectric impedance
analysis. Oxidative stress markers viz total antioxidant capacity and malondialdehyde were
measured using elisa kit. Nerve conduction study both motor and sensory was carried out in
relevant nerves.

Results: Serum levels of total antioxidant capacity and super oxide dismutase were
significantly decreased in type 2 diabetic with neuropathy as compared to the ones without
neuropathy. Serum levels of malondialdehyde, on the other hand, was significantly elevated.
Motor nerve conduction velocity (MNCV) of ulnar and common peroneal nerve and sensory
nerve conduction velocity of ulnar and sural showed a significantly decreased conduction
velocity as compared to control. The total body fat mass showed a significant negative
correlation with the motor conduction velocity of median, posterior tibial and sensory
conduction velocity of median and sural nerve.

102
Conclusion: The nerve conduction velocity test and total body fat mass evaluation is a cost-
effective tool for earlier detection of neuropathy in type 2 diabetes patient.

103
NE004. DISTINGUISHING GBS WITH TREATMENT-RELATED
FLUCTUATIONS FROM ACUTE-ONSET CIDP: A NEUROPHYSIOLOGICAL
AND ULTRASONOGRAPHIC PERSPECTIVE

Toh Tsun Haw*1 ; Nortina Shahrizaila1 ; Mohd Azly Yahya1 ; Goh Khean Jin1 ; Tan Cheng Yin1
1Neurology, University Malaya Medical Centre, Malaysia

Email Address: yuy-987@hotmail.com

Introduction: Distinguishing Guillain-Barré syndrome (GBS) with treatment-related


fluctuations (TRF) from acute-onset chronic inflammatory demyelinating polyneuropathy (A-
CIDP) in the early phase can be challenging. Distinguishing features in clinical aspect have
been reported previously. We aim to identify the neurophysiological and ultrasonographic
features that might differentiate between patients with GBS-TRF and A-CIDP.

Methods: Patients with GBS-TRF and A-CIDP were identified from an existing cohort of GBS
patients presenting to University Malaya Medical Centre, Kuala Lumpur, Malaysia from 2011 to
2019. The clinical, neurophysiological and nerve ultrasound data were recorded and analysed.

Results: Five GBS-TRF (mean age 42±23 years old) and five A-CIDP (mean age 66±13 years
old) patients were included. The mean time to first neurological deterioration was significantly
longer in the A-CIDP patients compared to GBS-TRF (11 vs 5 weeks; p=0.028). Based on two
sets of nerve conduction studies (NCS), both GBS-TRF and A-CIDP patients fulfilled the
electrodiagnostic criteria for demyelination. At first study, A-CIDP patients had significantly
prolonged ulnar minimal F-wave latencies (40.7 vs 28.6 ms; p=0.020) and slower tibial nerve
motor conduction velocities (32.1 vs 38.0 m/s; p=0.050) when compared to GBS-TRF. The
sural nerve conduction velocities were slower in A-CIDP compared to GBS-TRF during the
second study (26.3 vs 41.4 m/s; p=0.015). The sural potentials were also more likely to be
unrecordable in A-CIDP patients in the second study. On nerve ultrasound, A-CIDP patients
had significantly larger cross-sectional area of the ulnar nerve at the wrist (7 vs 5 mm 2;
p=0.039), forearm (8 vs 5 mm2; p=0.025), below elbow (9 vs 6 mm2; p=0.031) and above
elbow (9 vs 7 mm2; p=0.037) compared to GBS-TRF patients.

Conclusion: Other than distinguishing clinical features, we found that there were
neurophysiological and ultrasonographic features that can be useful in differentiating between
GBS-TRF and A-CIDP.

104
NE005. ELECTRONEUROMYOGRAPHY AS A PREDICTOR OF PERIPHERAL
NEUROPATHY SYMPTOMS AFTER TWO YEARS FOLLOW-UP IN FARMERS
EXPOSED TO PESTICIDE: A COHORT STUDY

Andre Stefanus Panggabean*1 ; Kusumo Dananjoyo1 ; Rusdy Ghazali Malueka1 ; Subagya1 ;


Ahmad Asmedi1
1Neurology Department, Faculty of Medicine, Public Health and Nursing Universitas Gadjah

Mada, Yogyakarta, Indonesia


Email Address: andre.stefanus.p@mail.ugm.ac.id

Introduction: Farmer is one of the most common occupation in Indonesia. To increase crop
production, they tend to use organophosphate or carbamate pesticides. These extensive
pesticide uses could cause low cholinesterase levels in blood, and cause injury in peripheral
nerve and neuropathy symptoms. With electroneuromyography (ENMG), subclinical
neuropathy preceding its symptom could be found. The purpose of this study is to find
association between any abnormality measurement in ENMG with neuropathic symptoms after
two years.

Methods: This was cohort prospective study. Farmers with history of pesticide exposure,
without neuropathic symptoms in 2017, had low blood cholinesterase levels, and gave consent
to have ENMG examination were recruited. Motor and sensory aspect of nerve was examined.
For motor, we examined distal latency (DL), nerve conduction velocity (NCV), proximal
amplitude (PA) and distal amplitude (DA) of median, ulnar, and tibial nerves. For sensory, we
examined DL and PA of median, ulnar, and sural nerves. Neuropathic symptoms in 2 years
follow up were determined from Total Neuropathic Symptoms Scale, with score > 0 diagnosed
as having neuropathic symptoms.

Results: There were 26 farmers included in this study. After statistical analyses, there were
association between neuropathic symptoms after 2 years with motor NCV, motor PA and
sensory DL of median nerve; motor NCV and motor PA of ulnar nerve (p-value > 0.05). After
age, gender, working hour, pesticide spray duration adjustment, ENMG markers which had
significant and independent association with neuropathy symptoms after two years were ulnar
motor DL (p-value: 0.032), median motor NCV (p-value: 0.035), median motor PA (p-value:
0.043), and ulnar sensory DL (p-value: 0.010).

Conclusion: From this study, we concluded that ENMG can be used as predictor for
neuropathic symptoms in 2 years for asymptomatic farmers exposed to pesticide with low
blood cholinesterase levels.

105
Autonomic Dysfunction
AD001. RELATIONSHIP BETWEEN ELECTROCHEMICAL SKIN
CONDUCTANCE AND CARDIOVASCULAR AUTONOMIC FUNCTION IN
PARKINSON’S DISEASE

Chih-Cheng Huang*1 ; Yun-Ru Lai1 ; Cheng-Hsien Lu1


1Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Taiwan.

Email Address: hjc2828@gmail.com

Introduction: Electrochemical skin conductance (ESC) of the hands and feet can be recorded
by SUDOSCAN. Several reports have confirmed that ESC can serve as an indicator of
sudomotor function. Some studies done in patients with diabetes mellitus showed that ESC
can be a predictor of cardiovascular autonomic neuropathy. The autonomic pathology in
Parkinson’s disease (PD) is primarily postganglionic. This study tested the hypotheses that
ESC measured by SUDOSCAN has high correlation with cardiovascular autonomic function in
patients with PD, and can serve as a predictor of cardiovascular autonomic neuropathy (CAN)
for such patients.

Methods: Sixty-three patients (male/female: 28/35) with PD were recruited. The severity was
assessed using Unified Parkinson’s Disease Rating Scale (UPDRS) and Hoehn and Yahr
stage. They had ESC measurement of hands and feet by SUDOSCAN, and underwent a
standardized evaluation of cardiovascular autonomic function, including heart rate response to
deep breathing, Valsalva ratio (VR), baroreflex sensitivity, 5-min head-up tilt test and
frequency-domain analysis of heart rate variability. Composite Autonomic Scoring Scale were
coded accordingly and these patients were then divided into two groups, with and without
CAN.

Results: There was weak but significant correlation between hand ESC and UPDRS scores.
As for the cardiovascular autonomic parameters, the significant correlation only exited between
hand ESC and VR. There was no significant difference of ESC between patients with CAN and
without CAN.

Conclusion: In spite of the significant correlation between hand ESC and UPDRS scores, the
ESC measured by SUDOSCAN may not be good enough in predicting CAN for patients with
PD.

106
AD002. CORRELATION OF GLYCAEMIC STATUS WITH HRV IN PATIENTS
OF DIABETIC PERIPHERAL NEUROPATHY: A CROSS SECTIONAL STUDY

Ahmad Faraz*1 ; Hamid Ashraf2 ; Sangeeta Singhal1 ; Fareha Hussain1 ; Jyoti Jaiswal1
1Physiology, J.N Medical College, Aligarh Muslim University, India, 2Rajiv Gandhi Centre for

Diabetes and Endocrinology, J.N Medical college, India.


Email Address: afaraz83@gmail.com

Introduction: Diabetes has become one of the leading causes of morbidity and mortality
across the world. Cardiac Autonomic Neuropathy (CAN) has increased the chances of silent
myocardial infarction and thus has shortened lifespan resulting in death in 25 - 50 % patients
within 5 - 10 years of CAN. Further patients of Diabetic Peripheral neuropathy (DPN) often
have associated Autonomic Neuropathy. Our study aims at identifying the correlation of
glycemic parameters with HRV parameters in patients of DPN.

Methods: 95 patients of diabetes were assessed for the inclusion and exclusion criteria. 60
patients of diabetes were included in the study who gave valid written consent. They were
assessed for the signs of neuropathy by evaluating ankle reflex, pinprick, vibration sense, and
monofilament testing. Patients were assessed for neuropathy by MNSI Scoring. Heart rate
variability was performed by PHYSIO-PAC SOFTWARE SYSTEM. Two–lead
electrocardiographic data were recorded for 5 minutes and analyzed by HRV software. The
time-domain analysis was done by Fast Fourier transformation. Statistical analysis of the data
was performed using SPSS version 21. Nominal variables are presented as the numbers and
percentages of cases and continuous variables as means ± standard deviation (SD). The
association between continuous variables was tested by linear correlation using Pearson's
coefficient. P-value of < 0.05 was considered significant.

Results: The mean age of T2DM with neuropathy was 49.17 ± 7.1. A correlation of Fasting
Plasma Glucose (FPG), Postprandial Glucose (PPG) and HbA1C with HRV parameters
(pNN50, RMSSD, HF power, and LF/HF ratio) showed the statistically significant negative
association.

Conclusion: Patients with uncontrolled diabetic peripheral neuropathy are more prone to
CAN. We conclude that patients having autonomic dysfunction should be treated with
extensive therapy, which will thereby lower mortality and morbidity. Screening by HRV should
be done periodically.

107
AD003. POSTURAL ORTHOSTATIC TACHYCARDIA SYNDROME (POTS): A
CASE SERIES FROM INDIA

Parthasarathy B*1; Arun Grace Roy1


1Neurology, Amrita Institute of Medical Sciences and Research Centre, Kochi, India.

Email Address: dr.parthasarathyb@gmail.com

Introduction: POTS (Postural Orthostatic Tachycardia Syndrome) is defined as the presence


of chronic symptoms of orthostatic intolerance (≥6 months) accompanied by an increased
heart rate (HR) ≥30 bpm within 10 minutes of assuming an upright posture in the absence of
orthostatic hypotension. It is diagnosed by tilt table testing where the heart rate at base line
and on standing is recorded. The incidence and presenting complaints of this disease has not
been studied in the Indian subcontinent.

Methods: This is a retrospective study where we looked into the data of patients with
diagnosis of POTS over a period of 3 years (August 2014 – August 2017). We are describing
the baseline demographics, clinical presentations, investigations, treatment and follow up of
these patients. POTS was diagnosed based on inclusion criteria with a tilt table. Heart rate was
recorded in supine position and then in upright position. Continuous heart rate and BP
recording was done every minute for 10 minutes and if patient had symptoms it was noted.

Results: During the study period from August 2015 – August 2017 we had 42 patients with
POTS. This was 7.7% of all patients who had positive tilt table testing during this period. The
cohort had 24 males and 18 females. Commonest symptoms were postural giddiness in 18
patients, palpitation on standing and walking in 13 patients and postural syncope in 12
patients. 13 patients had improvement with excess fluid intake, while 29 patients required drug
therapy.

Conclusion: This is the only series of POTS from India. POTS is not a rare cause of
orthostatic intolerance as previously reported. High index of suspicion can help in early
identification and treatment of this syndrome.

108
AD005. DIABETIC CARDIOVASCULAR AUTONOMIC NEUROPATHY

Abbasher Hussien*1 ; Khabab Abbasher2 ; Alla Eldin3 ; Amira Sidig4 ; Khalid Hajnoor5
1Internal Medicine, University of Khartoum Faculty of medicine, Sudan, 2MBBS student,

University of Khartoum Faculty of Medicine, Sudan, 3Internal medicine, Al-shaab Teaching


Hospital, Sudan, 4Community medicine, Al Neilain University faculty of medicine, Sudan,
5Internal medicine, Mediclinic Hospital, United Arab Emirates.

Email Address: Khabab9722@gmail.com

Introduction: Diabetic neuropathies are thought to result from diabetic microvascular injury
involving small blood vessels that supply nerves. Cardiac autonomic neuropathy (CAN)
represents a serious complication associated with diabetic neuropathies.

Methods: This is a descriptive prospective cross-sectional hospital-based study. The study


population included type 1 and type 2 diabetic patients admitted to the cardiac care unit in
ELshaab Teaching Hospital with acute coronary syndrome over a period of two years, from
April 2017 to April 2019.

Results: A total of 49 males (65.33%) and 26 females (34.67%) were included in the study.
CAN was detected in 94.67% of the studied group.

Conclusion: An incidence of 94.67% cardiovascular autonomic neuropathy in our sample


indicates a high occurrence of this syndrome among our diabetic patients. Poor glycemic
control is a common feature among our studied group. Sub-clinical autonomic neuropathy can
be detected early using autonomic function tests.

109
AD006. HEART RATE VARIABILITY AS A TOOL FOR PREDICTING
EPILEPTIC SEIZURES

Akash Shridharani*1 ; Siby Gopinath1 ; Anand Kumar1 ; Mrudula G B2


1Department of Neurology, Amrita institute of medical sciences, India, 2Department of ECE,

Amrita School of Engineering, India


Email Address: aakashshridharani@gmail.com

Introduction: Epilepsy is a neurological disorder that affects the patient’s quality of life and
health care budget. Autonomic involvement, particularly respiratory and cardiac involvement is
extremely dangerous. A particularly life-threatening complication is SUDEP. HRV represents
ANS activity. We aim to compare the HRV in preictal, interictal and postictal period in patient
with various types of epilepsy.

Methods: Patients admitted for presurgical evaluation for epilepsy surgery and patients with
epilepsy admitted for prolonged EEG monitoring (> 8 hours) having various types of seizures
were selected. Amongst these 49 patients, 142 seizures with various semiologies were
recorded and were sent for processing. ECG signal was extracted from EEG data. Signal
processing and HRV extraction was performed using EDF browser based on MATLAB. Time
domain measurements like mean HR, RRI, RMSSD, SDNN and pNN50 were analyzed at
different times – 20 minutes before the seizure, 1 minute before the seizure, during the seizure
and 20 minutes after the seizure for each individual event.

Results: Mean age was 27.14 ± 12.15 years with a minimum age of 5 years and maximum
age of 58 years. Out of a total of 142 events recorded, 23 events were focal seizures with
preserved consciousness, 73 events were focal seizures with impaired consciousness, 45
events were generalized seizures and 1 was a psychogenic seizure. There was significant rise
in the mean HR and a drop in RRI, RMSSD, SDNN and pNN50 in the immediate preictal and
interictal period. A comparison of various time intervals compared with respect to type of event
showed that generalized event had a more autonomic dysregulation compared to focal
seizures.

Conclusion: HRV analysis is a valid method for quantifying central influences on autonomic
nervous system and its cardiac control, and can give important information when used as an
adjunct during clinical seizures.

110
Movement Disorders

MD001. THE ROLE OF NEUROPHYSIOLOGICAL TESTING FOR


DIFFERENTIAL DIAGNOSIS OF FUNCTIONAL TREMOR IN BUSY CLINIC

Sasivimol Virameteekul1* ; Pattamon Panyakaew1 ; Roongroj Bhidayasiri1


1Neurology, Chulalongkorn Hospital, Thailand

Email Address: svi@chulapd.org

Introduction: The presenting phenomenology of functional tremor (FT) is complex making


clinical diagnosis challenging. However, certain features from neurophysiological testing may
aid the differential diagnosis of FT. Aim of investigation: To study the clinical characteristics
and neurophysiological findings of FT and compare with organic tremor (OrgT).

Methods: 12 patients with suspected FT were recruited. Clinical characteristics and


neurophysiological features were reviewed and compared with OrgT.

Results: Of the 12 patients, 50% were neurophysiologically confirmed as having FT. There
were no differences in age of onset, disease duration or psychiatric comorbidity between FT
and OrgT, but FT is more common in female than OrgT (M:F: 5:1 vs. 2:4, p=0.24). Stressful
events were commonly reported as a precipitating factor in FT. Presenting symptoms of FT
were mostly unilateral (83%) and task-specific (67%) with a possible dystonic posture. The
aetiologies of OrgT mimicking FT were enhanced physiological tremor (4 patients), PD (1
patient) and orthostatic tremor (1 patient). Clinical signs and neurophysiological features that
can significantly differentiate between FT and OrgT were distractibility (83% vs 7%, p=0.02),
entrainment (67% vs 0%, p=0.01), distractibility/frequency shift with tapping (100% vs 0%,
p<0.01), pause of tremor during ballistic movement (67% vs 0%, p=0.01), coactivation at
tremor onset (67% vs 0%, p=0.01) and inconsistency of tremor (60% vs 0%, p=0.03)
respectively.

Conclusion: Our study demonstrates the role of neurophysiological testing in those with
suspected FT. In particular, distractibility appears to be an important clinical sign for diagnosis
of FT.

111
MD003. THE COMPLEXITY OF INVOLUNTARY HAND MOTION
DISTINGUISHES BETWEEN ESSENTIAL AND PARKINSONIAN TREMOR

Dongning Su*1 ; Zhu Liu1 ; Shuo Yang1 ; Ying Wang1 ; Huizi Ma1 ; Brad Manor2 ; Jeffrey M.
Hausdorff3 ; Lewis A. Lipsitz2 ; Hua Pan1 ; Tao Feng1 ; Junhong Zhou2
1Neurology, Beijing Tiantan Hospital, China, 2Hinda and Arthur Marcus Institute for Aging

Research, Harvard Medical School, United States, 3Center for the Study of Movement,
Cognition, and Mobility, Tel Aviv Sourasky Medical Center, Israel
Email Address: 18511886771@163.com

Introduction: Essential tremor (ET) and Parkinsonian tremor (PT) are often clinically
misdiagnosed. Involuntary hand motion depends upon numerous physiological interactions
over multiple temporal-spatial scales. The dynamics of the motion are thus “complex,” and the
pathophysiological changes in PT and ET may impact such complexity to different degrees.
We hypothesize that measures of complexity may be used to distinguish ET from PT.

Methods: Forty-eight participants with clinically-diagnosed PT and 48 age- and sex-matched


participants with ET completed this study. Participants performed two 30-second tests within
each of the following conditions: sitting while resting arms on chair arms or outstretching arms
horizontally. The involuntary motion was derived from accelerometers secured to the dorsum
of each hand. The complexity quantified using multiscale entropy and the amplitude and
dominant frequency of the motion were calculated. We also confirmed the accuracy of our
results using a separate dataset of 22 patients with PT and 22 with ET.

Results: Participants with ET had lower complexity of both hands across both conditions
(F>10.8, p0.15, p0.18, p<0.001) than those with PT, while no significant difference in the
amplitude and dominant frequency between groups was observed. Those with lower
complexity had longer disease duration (r2>0.15, p< 0.009) and greater UPDRS-III scores
(r2>0.18, p<0.009). Receiver-operating-characteristic curves revealed that the complexity
metric can distinguish ET from PT (resting: AUC=0.78, cut-off value=49; postural: AUC=0.88,
cut-off value=48). Using the cut-off score of 49 in resting condition, it showed an 80% accuracy
to identify ET from PT using the complexity metric in resting condition. Using the cut-off score
of 48 in postural condition, it revealed a high accuracy of 90% to identify ET from PT.

Conclusion: The complexity of involuntary hand motion captures pathophysiological


characteristics reflective of PT and ET. This metric may serve as a novel marker to help
distinguish ET from PT in clinical practice.

112
MD004. THERAPEUTIC EFFECT OF LOW DOSE BOTULINUM TOXIN TYPE A
INJECTION IN HEMIFACIAL SPASM PATIENTS: A PRELIMINARY STUDY

Retno Jayantri Ketaren*1 ; Evlyne Erlyana Suryawijaya2 ; Vinson Hartoyo2 ; Jennifer Simca3
1Department of Neurology, Siloam Hospitals Lippo Village - Faculty of Medicine Universitas

Pelita Harapan, Indonesia, 2Department of Neurology, Siloam Hospitals Lippo Village,


Indonesia, 3General Practitioner, Siloam General Hospital, Indonesia
Email Address: vinson.hartoyo@gmail.com

Introduction: Hemifacial spasm (HFS) is a common movement disorder characterized by


involuntary tonic or clonic contractions of muscles innervated by the facial nerve. Botulinum
toxin type A (BoNT-A) injection is currently the preferred symptomatic treatment for primary
hemifacial spasm. Low dose injection of botulinum toxin in patients with HFS is still
controversial. In this study, we evaluated the clinical characteristics and therapeutic effects of
HFS patients receiving low dose BoNT-A injection.

Methods: A descriptive study was conducted on hemifacial spasm patients in a referral


hospital in Tangerang, Indonesia since 2018- 2019. Patients with hemifacial spasm that had
consented to the procedure were included, and patients who had contraindications were
excluded. Demographic data, clinical data, duration of response, and side effects were
analyzed. 1-4 units of botulinum toxin injection were intramuscularly injected to muscles that
had spasm.

Results: A total of 26 patients with HFS were treated with 82 BoNT-A treatments, comprising
of 34,6% male and 65,4% female with doses injected 3.68 ± 1.68 U. Orbicularis oculi and
zygomatic major muscles were the most injected muscles (92.3% and 38.5% respectively)
while the least muscles injected were Frontalis and Risorius muscle (injected only in 3.8%).
Lagophthalmos was the most common experienced side effects (reported in 17.6% of cases).
Overall, 81.3% of patients reported moderate improvement after injection.

Conclusion: This study showed that low dose injection of BoNTA might have a promising
outcome. Majority of patients experienced moderate benefit with scanty side effects. Extensive
evaluation of response rate, complication rate and resistance effects of BoNTA with abundant
amount of samples are needed to further substantiate this study.

113
Motor Neuron Disease (MND)
MN001. ASSOCIATION OF SYMPATHETIC SKIN RESPONSE IN
AMYOTROPHIC LATERAL SCLEROSIS

SK Mahbub Alam*1 ; Dr. Mehedi Hasan1 ; Prof. Hasan Zahidur Rahman1


1Neurology, Bangabandhu sheikh mujib medical university, Dhaka, Bangladesh

Email Address: smalam09@gmail.com

Introduction: Amyotrophic lateral sclerosis (ALS) is the most common type of motor neuron
disease. General concept is autonomic disturbances are extremely rare in ALS. Some
hypotheses have put forward to see autonomic involvement in ALS. Previous studies have
found an association between sympathetic skin response (SSR) and ALS as a part of
autonomic involvement. The study was aimed to explore the association of SSR in ALS.

Methods: This case control study was carried out in the Department of Neurology, BSMMU,
Dhaka, from April 2018 to September 2019. Total 70 subjects were enrolled as study
population after fulfilling inclusion and exclusion criteria. Among them, 35 were grouped as
case and rest 35 were as control. All patients with ALS were diagnosed according to The
Revised El Escorial diagnostic criteria fulfilling definite, probable, probable lab-supported and
possible cases of ALS with no family history of ALS.

Results: The study included 35 ALS patients and 35 age- and sex-matched controls. SSR was
absent in 6 (17.1%) cases in upper limbs and 20 (57.1%) cases in lower limbs. In 4 patients,
SSR was absent in both upper and lower limbs. Among control group SSR response was
present in all population. There was significant difference in mean latency and amplitude of
SSR in case and control group (p-value<0.001). Absent SSR in ALS with bulbar palsy showed
obvious difference with ALS without bulbar palsy (p-value 0.040). There was an association of
SSR with severity of disease in ALS patient (p-value 0.030) but we could not establish it with
duration of disease.

Conclusion: In this study we found significant relationship between SSR and ALS. We noticed
an association of SSR with severity of disease in ALS.

114
MN002. ASSESSMENT OF UPPER MOTOR NEURON FUNCTION UTILIZING
THRESHOLD TRACKING TRANSCRANIAL MAGNETIC STIMULATION IN
AMYOTROPHIC LATERAL SCLEROSIS

Kazumoto Shibuya*1 ; Yo-ichi Suzuki1 ; Sonoko Misawa1 ; Suichi Tomoki1 ; Atsuko


Tsuneyama1 ; Keigo Nakamura1 ; Hiroki Kano1 ; Satoshi Kuwabara1
1Neurology, Chiba University, Japan

Email Address: kazumoto@net.email.ne.jp

Introduction: Upper motor neuron signs are mainly assessed by clinical examinations.
Threshold tracking transcranial magnetic stimulation (TT-TMS) can precisely investigate motor
cortical function and has been reported as a diagnostic marker in amyotrophic lateral sclerosis
(ALS). The correlation between TT-TMS and clinical and neurophysiological findings were
examined.

Methods: TT-TMS was performed in 30 ALS patients and 26 healthy controls (HC), measured
in the abductor pollicis brevis (APB) muscle. Clinical factors, such as age, gender, disease
duration, site of onset, upper motor neuron (UMN) sign scores and ALS functional rating scale
revised (ALSFRS-R) and a neurophysiological index, such as compound muscle action
potential (CMAP) amplitude, measured in the APB muscle, were analyzed with TT-TMS
findings.

Results: Of 30 patients, 13 were male, and 10 were bulbar onset. The mean age was 69 years
old, and the mean disease duration was 19 months. Averaged short interval intracortical
inhibition (SICI) (1-7 ms) was decreased in ALS (p < 0.01), compared with HC. Averaged SICI
(1-7 ms) was not related to age, gender, disease duration, site of onset, UMN sign scores,
ALSFRS-R and CMAP amplitudes. Decreased SICI was unveiled even in 4 patients, without
evident upper motor neuron signs.

Conclusion: TT-TMS found motor cortical dysfunction in ALS, but the relationship between
SICI and UMN signs was not evident. Progress of cortical hyperexcitability and pyramidal tract
lesion may not be parallel in ALS. Additionally, muscle atrophies might hamper detection of
UMN signs. Further studies are necessary to discuss usefulness of TT-TMS as a UMN sign
marker in ALS.

115
MN003. DECREMENTAL RESPONSE TO REPETITIVE NERVE STIMULATION
OCCURS IN EARLY STAGE OF AMYOTROPHIC LATERAL SCLEROSIS

Ma Jingyue*1 ; Liu Xiangyi1 ; Zhang Shuo1 ; Fan Dongshen1


1Neurology, Peking University Third Hospital, China

Email Address: mjyue1019@163.com

Introduction: This study aimed to clarify the features and clinical significance of decremental
response during slow-rate repetitive nerve stimulation (RNS) in amyotrophic lateral sclerosis
(ALS) patients of early stage.

Methods: 3-Hz RNS tests were performed in 175 nerve/muscle pairs from 22 ALS patients,
whose duration of illness was less than 1 year, and lower motor neuron signs were limited in
upper limbs. Both proximal and distal muscles were included. All patients were given a follow-
up evaluation by telephone every 3 months. 15 patients had an electromyography (EMG)
reexamination in 6 months. Besides, 81 nerve/muscle pairs in 37 normal controls were
collected.

Results: In ALS patients, compound muscle action potentials (CMAP) decrement percentages
to RNS in all tested nerve/muscle pairs were greater than in controls (p<0.01). Muscles
without EMG spontaneous activity (including fibrillation potentials and positive sharp waves)
showed higher decrement percentage than normal controls (p<0.05), but were significantly
lower compared to muscles with spontaneous activity (p<0.05). Additionally, in muscles
without EMG spontaneous activity, 14/58 pairs had RNS decrements over 5%, mainly in the
deltoid (38.5%) and trapezius (35.3%). Furthermore, nerve/muscle pairs with higher RNS
decrement percentage had greater drops in CMAP and higher increases in EMG spontaneous
activity 6 months later, but had no correlation with the changes of ALSFRS-R scores.

Conclusion: RNS decrements changed in the early stage of ALS, even before the denervation
of corresponding muscles. In ALS patients within 12 months, higher RNS decrement
percentage indicated faster low motor neuron injury and denervation during the progression of
the disease. RNS could be a possible electrophysiological biomarker to evaluate prognosis.

116
MN004. THE SELECTIVE AND INVERSE U-SHAPE CURVE ALTERATION OF
RETINAL NERVE FIBER LAYER IN ALS: A POTENTIAL MIRROR OF THE
DISEASE

Yixuan Zhang*1 ; Xiangyi Liu1 ; Ziyuan Liu2 ; Xue Yang2 ; Yuanjin Zhang1 ; Dongsheng Fan1
1Neurology Department, Peking University Third Hospital, China, 2Ophthalmology Department,

Peking University Third Hospital, China


Email Address: 1310301514@bjmu.edu.cn

Introduction: The alteration of the visual pathway involving retina has been reported in
amyotrophic lateral sclerosis (ALS), but lack of consistency and subgroup analysis. We aim to
assess the retinal nerve fiber layer (RNFL) alteration in different stages of ALS patients and its
association with ALS progression parameters.

Methods: Study population consisted of 70 clinically diagnosed ALS patients and 48 matched
controls. All of them underwent glaucoma screening tests and Optical Coherence Tomography
(OCT) imaging. Four quadrants of peripapillary RNFL (pRNFL) were observed and automated
measured. Early stage distal motor neuron axon dysfunction in ALS was detected by
compound muscle amplitude potential (CMAP) of distal limbs within 12 months. The ALS
disease parameters including ALSFRSR score and disease progression index (DPI, (48-FRSR
score)/months from symptom onset).

Results: Generally compared with controls, the nasal (p=0.00) and temporal (p=0.03)
quadrants of pRNFL were significantly thicker in ALS patients when controlling for age and
DPI. The pRNFL was significantly thickened as disease progressed (r=0.37, p=0.04) within 12
months, while declined with time (r=-0.47, p=0.02) after one year. ALS patients were sub-
divided into thicken-RNFL (T-RNFL, >control mean+SD) and impaired-RNFL (I-RNFL)

Conclusion: The alteration of retinal nerve was not consistent in ALS patients with diverse
phenotypes and progression rates. In our study, the pRNFL thickened during the first year and
then gradually declined. This inverse U-shape curve transformation might be due to the initially
priming and then inflammatory reaction of microglia intermingled between retinal nerve axons,
and was in accordance with early stage motor neuron inflammation and axonopathy.

117
Neuromuscular Junction Disorders (NMJ)

NJ001. ELECTRODIAGNOSTICS PROFILE IN MYASTHENIA GRAVIS

Pricilla Yani Gunawan*1 ; Ahmad Yanuar Safri2 ; Fitri Octaviana2 ; Winnugroho Wiratman2 ;
Manfaluthy Hakim2
1Neurology Pelita Harapan University, Indonesia, 2Neurology, University of Indonesia,

Indonesia
Email Address: pricilla.gunawan@uph.edu

Introduction: Repetitive Nerve Stimulation (RNS) has a sensitivity of 50-80% in predicting


Myasthenia Gravis (MG). This wide range is affected by type and severity of MG, muscle
tested, frequency of stimulation and exercise. There has been a previous study describing
these factors but did not confirmed it with the result of Single Fiber (SF) and Acetylcholine
Receptor antibody (AChR).

Methods: This was a cross-sectional study. Data was from RNS results of MG patients in
Electromyography laboratories, Cipto Mangunkusumo National Hospital January to December
2019. Muscle tested on RNS were abductor digiti minimi (ADM) muscle, trapezius (Tr) muscle
and orbicularis occuli (OrbOc) muscle. Slow RNS used 3, 5, 7 and 10 Hz stimulation followed
by 3 Hz stimulation immediate, 1 minute (min) and 2 min post exercise. Decrease of
Compound Muscles Action Potential (CMAP) amplitude > 10% is considered as positive RNS.

Results: As much as 47 patients were included in this study. The average age of patients is 43
years old. As much as 44.7% had a positive RNS test, and 70% of the case was positive from
OrbOc, followed by Tr (60%) and ADM (30%). Frequency of stimulation that obtained the
positivity the most is at 5 Hz, and the 4th wave revealed the lowest point of decreament,
followed by the 5th wave. Single Fiber was positive in 57.4% of the case, and AChR was
positive in 51.1%. There are 11 patients (23.4%) that has a mismatch results between SF and
AChR.

Conclusion: RNS without confirmation of SF and AChR may yield a false negative result.
Orbicularis Oculi shows the most sensitive muscle tested in RNS, with stimulation of 5 Hz and
the 4th waveform the lowest decrement. SF and AChR are still important in confirming strong
suspicion patients with MG despite negative results in RNS.

118
NJ002. BULBAR ONSET MYASTHENIA GRAVIS: DIAGNOSTIC
CHALLENGES AND MISSED OPPORTUNITIES

Ayatallah Farouk Hussein*1 ; Shereen Fathy1 ; Aya Shekhany1


1Clinical Neurophysiology Unit Kasraliany, Cairo University, Egypt

Email Address: aia.farouk@yahoo.com

Introduction: Myasthenia gravis (MG) is a frequently encountered neuromuscular junction


(NMJ) disorder in clinical practice with a prevalence of 5 to 15 cases per 100,000 Ocular
symptoms are the presenting sign of the disease in 50% to 75% of patients, bulbar plus ocular
symptoms in 23.17% & Myasthenic patients presenting with isolated bulbar symptoms are
mostly about 20.73% When a patient comes with acute bulbar symptoms especially with
dysphonia ,it will lead to diagnostic dilemma as there are good number of causes, apart from
laryngeal myasthenia which is an uncommon aetiology, as stroke, demyelinating neurological
diseases, toxicity, infection and other causes.

Results: This is a case series report of isolated bulbar onset myasthenia gravis which is rarely
found , all presenting with dysphonia as an initial manifestation of bulbar myasthenia we could
reach the diagnosis serologically and electrophysiologically (EMG, NCV, repetitive
supramaximal stimulation and single fiber EMG )and exclude other common causes of acute
bulbar weakness with marvellous improvement after therapy .

Conclusion: considering MG as one of the potential differential diagnoses among cases of


new onset or recurrent unexplained bulbar symptoms is of high importance giving a high
opportunity to almost complete recovery.

119
Intraoperative Monitoring (IOM)

IM001. INTRAOPERATIVE HYPOGLOSSAL NERVE MAPPING DURING


CAROTID ENDARTERECTOMY

Atsuhiro Kojima*1 ; Isako Saga1 ; Mami Ishikawa2


1Neurosurgery, Saitama City Hospital, Japan, 2Neurosurgery, Tachikawa Kyosai Hospital,

Japan
Email Address: akojima-nsu@umin.ac.jp

Introduction: Hypoglossal nerve deficit is a possible complication caused by carotid


endarterectomy (CEA). The accidental injury of the hypoglossal nerve during surgery is one of
the major reasons for permanent hypoglossal nerve palsy. In this study, we investigated the
usefulness of intraoperative mapping of the hypoglossal nerve to identify this nerve during
CEA.

Methods: A total of consecutive patients who underwent CEA for the treatment of symptomatic
or asymptomatic carotid artery stenosis were studied. A hand-held probe was used to detect
the hypoglossal nerve in the operative field, and the tongue motor evoked potentials (MEPs)
were recorded.

Results: The tongue MEPs were obtained in all the patients. The invisible hypoglossal nerve
was successfully identified without any difficulty when the internal carotid artery was exposed.
Intraoperative mapping was particularly useful for identifying the hypoglossal nerve when the
hypoglossal nerve passed beneath the posterior belly of the digastric muscle. MEPs were also
elicited when the ansa cervicalis was stimulated, although the resulting amplitudes were much
smaller than those obtained by direct stimulation of the hypoglossal nerve. Postoperatively,
none of the patients presented with permanent hypoglossal nerve palsy, although one patient
exhibited transient minor hypoglossal nerve paresis.

Conclusion: Intraoperative hypoglossal nerve mapping enabled us to locate the invisible


hypoglossal nerve during the exposure of the internal carotid artery accurately without
retracting the posterior belly of the digastric muscle and other tissues in the vicinity of the
internal carotid artery.

120
Evoked Potential (EP)

EV001. THE EFFECT OF WATCHING MOVIES ON BAEP

Muhammad Iqbal Basri*1 ; Sitti Rafiah1 ; Asty Amalia1 ; Muhammad Akbar2 ; Yudy Goysal2
1Department of Anatomy, University of Hasanuddin, Indonesia, 2Neurology, University of

Hasanuddin, Indonesia.
Email Address: iqbalbasri@gmail.com

Introduction: Previous researches on peripheral filtering at the cochlear and brainstem levels
toward auditory information by using BAEP measurement had been conducted. However, the
results were still varied. This study was aimed to find out the effect of selective visual attention
of watching movies towards the latency and amplitude of BAEP.

Methods: This study involved 12 healthy subjects (9 males, 3 females) with right-ear
dominance. The age of samples ranged from 21-52 years old, and the mean was 30.7 years.
BAEP measurement was done in two conditions, eyes were closed and watching a silent
action movie in 14 inches monitor with a view distance of 75 cm. Every subject was recorded
two times for reproducibility using 4 Ag-Cl electrodes (Cz, Fz, A1, and A2) that were placed
according to the International Electrode (10-20) Placement system. The stimulus was
monoaural click-sound at the right ear with the intensity of 70 dB nHL, while noise-masking
was given to the left ear. The measurements of latency and amplitude of wave I, wave V and
interval peak latency of wave I-V were done.

Results: This study showed that there were significant latency delay of wave I as much as
0.068 ms or 3.81% (p=0.006) and a decrease of amplitude as much as 0.043 µV or 26.34%
(p=0.001). But no significant different of latency and amplitude of wave V on two conditions -
eyes closed and during watched movie (p > 0.05). The interval peak latency wave I-V of these
two groups was not significantly different also with each value [4.022 ± 0.115 ms vs 3.989 ±
0.100 ms (p = 0.241)].

Conclusion: Selective visual attention by watching movies gave an inhibition effect toward the
auditory pathway at the cochlear level, but no effect at the brainstem level.

121
EV002. AUDITORY EVENT-RELATED POTENTIAL CHANGES OVER 6
MONTHS FOLLOWING ACUTE ORGANOPHOSPHATE INSECTICIDE
POISONING: A FOLLOW-UP STUDY

Tharaka L. Dassanayake*1 ; Vajira S. Weerasinghe1 ; Indika Gawarammana2 ; Nicholas


Buckley3
1Department of Physiology, Faculty of Medicine, University of Peradeniya, Sri

Lanka, 2Department of Medicine, Faculty of Medicine, University of Peradeniya, Sri Lanka,


3School of Medicine and Health, University of Sydney, Sri Lanka

Email Address: tlag23@yahoo.co.uk

Introduction: Ingestion of organophosphate insecticides (OPIs) is a common method of self-


harm and a major clinical problem is Asia. Apart from acute cholinergic effects, OPIs are also
claimed to be associated with long-term neurobehavioural deficits. However,
neurophysiological evidence for such long-term deficits is scarce in humans. We aimed to
assess long-term changes in the pre-attentive (N1) and attentive (P3b) components of auditory
event-related potentials (ERPs) following acute OPI self-poisoning.

Methods: We recruited 203 patients (147 men) hospitalised following OPI ingestion (OP
Group; all had significant red cell cholinesterase inhibition) and 50 patients (23 men)
hospitalised with paracetamol overdose (Control Group) as a means of self harm. We recorded
their EEG in an auditory oddball task and derived the averaged ERPs. The subjects underwent
three testing sessions: on discharge from hospital (around 14 days post-ingestion), 6 weeks
and 6 months post-ingestion. We compared the reaction time and ERP indices of the two
groups at each time point, adjusting for sex, age, education and psychiatric comorbidities in
multiple regression models.

Results: After adjusting for covariates, OP Group had significantly slower reaction time than
the Control Group on discharge (mean difference [SE] = 65[20] ms, p=0.001) and at 6 weeks
(mean difference [SE] = 45[20] ms, p=0.024), but not at 6 months. Parietal (PZ) P3b
amplitudes were significantly smaller in the OP group on discharge (mean difference [SE] =
2.23[0.84] V, p=0.009) and at 6 months (mean difference [SE] = 3.34[1.43] V, p=0.021). P3b
latencies or frontal (FZ) N1 component did not show significant intergroup differences.

Conclusion: Acute exposure to clinically significant doses of OPIs seem to cause impairment
in behavioural and neurophysiological indices of stimulus discrimination, that outlast the
cholinergic phase of intoxication. The behavioural impairment seems to disappear over
months, but the underlying neurophysiological deficits of attentive processing seem to last
even after 6 months post-exposure.

122
EV003. ROLE OF MULTIFOCAL ELECTRORETINOGRAM IN ASSESSMENT
OF EARLY RETINAL DYSFUNCTION IN HYPERTENSIVE PATIENTS

Ayatallah Farouk Hussein*1 ; Reem ElHadidy1 ; Radwa Azmy1 ; Eman Attia Abd ElSalam1 ;
Rasha Zedan2
Clinical Neurophysiology Unit Kasralainy, Cairo University, Egypt, 2Ophthalmology, Kasralainy
Cairo University, Egypt.
Email Address: aia.farouk@yahoo.com

Introduction: Hypertensive retinopathy is the second most common cause of retinopathy


following diabetes, probably caused by retinal ischemia as a consequence of atherosclerotic
irreversible changes of systemic hypertension. Non-uniform functional retinal abnormalities are
difficult to detect by full-field ERG and needs the disease to reach advanced changes to be
affected. mfERG offers an objective noninvasive and reliable method to measure retinal
function. It allows simultaneous recording of local ERG responses from different parts of the
retina. Also previous studies suggested that the abnormalities in retinal electrophysiological
studies usually precede fundus signs of retinopathy as mfERG is affected early by the
neurodegenerative process. our aim in this study was to investigate localized retinal
dysfunction in hypertensive patients using multifocal electroretinogram (mfERG) and to assess
its sensitivity as an early predictor for the development of retinopathy in hypertensive patients.

Methods: Ninety-eight eyes were included in this case-control study. Twenty-eight eyes of
healthy subjects served as a control group (group I). Seventy eyes belonged to patients with
systemic hypertension assigned into two groups; group II including 39 eyes of hypertensive
patients with normal fundus and group III including 31 eyes of patients with signs of
hypertensive retinopathy. All participants were subjected to complete ophthalmic and
electrophysiological examination using mfERG. N1 and P1 wave amplitudes and implicit times
from the central hexagon and four concentric rings across the visual field were analyzed.

Results: mfERG amplitudes were significantly reduced in hypertensive group with retinopathy
than in controls. N1 amplitude was significantly reduced in the most eccentric ring in eyes of
hypertensive patients with normal fundus.

Conclusion: mfERG is a sensitive objective tool for assessment of retinal dysfunction in


hypertensive patients. mfERG amplitude is a promising predictor for early development of
retinopathy in systemic hypertension.

123
Peripheral Nerve Disorders

PN001. COMPARISON OF NEUTROPHIL TO LYMPHOCYTE RATIO AND


PLATELET TO LYMPHOCYTE RATIO BEFORE AND AFTER PLASMA
́ YNDROME PATIENTS
EXCHANGE TREATMENT IN GUILLAIN-BARRE S

Desby Juananda*1 ; Ahmad Asmedi1 ; Rusdy Ghazali Malueka1 ; Indra Sari Kusuma
Harahap1
1Department of Neurology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah

Mada, Yogyakarta, Indonesia.


Email Address: desbyjuananda@gmail.com

Introduction: Plasma exchange (PE) was the first treatment in Guillain-Barre ́Syndrome
(GBS) proven to be superior to supportive treatment alone. Several studies have evaluated the
PE efficacy for GBS. Recently, neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte
ratio (PLR) are known to be widely used as a biomarker of inflammation and a predictor of
prognosis in various disorders. This study investigates whether the NLR and PLR were the
laboratory parameters that associated with the treatment of GBS.

Methods: Fifty-two adults with GBS were retrospectively analysed from the electronic medical
records of patients who attended to the Neurology Department of Dr. Sardjito General
Hospital, Yogyakarta, Indonesia from January 2016 to September 2019. The NLR and PLR
before and after PE treatment in all patients were calculated and analysed with Wilcoxon
signed-rank test (p<0.05).

Results: Mean platelet, neutrophils and lymphocytes count (x 1000/mm 3) before and after PE
were 313.81 ± 13.64 vs 260.27 ± 17.79, 13.00 ± 1.79 vs 10.51 ± 0.95 and 2.22 ± 0.53 vs 1.91
± 0.16, respectively (p<0.05). The result showed that there was a statistically significant
difference in PLR before and after PE treatment (p=0.003). Comparing those two parameters
in before and after treatment, PLR was significantly reduced after PE.

Conclusion: This study demonstrated that PLR was significantly reduced after PE treatment
compared to NLR. We suggest that PLR could be one of laboratory parameters in GBS
treatment.

124
PN002. ASSOCIATION OF SERUM VITAMIN D LEVEL WITH PERPHERAL
NEUROPATHY IN TYPE 2 DIABETIC PATIENTS ADMITTED IN A TERTIARY
CARE HOSPITAL

Noor-E-Jabeen*1 ; Manobendra Bhattcharjee2 ; Rezaul Karim Ranju2 ; Professor Jalal Uddin2 ;


Md. Shamim Chowdhury3
1Department of Neurology, United hospital Limited, Bangladesh, 2Department of Neurology,

Mymensingh Medical College, Bangladesh, 3Department of Cardiology, National Heart


Foundation Hospital & Research Institute, Bangladesh
Email Address: shamim_mmc41@yahoo.com

Introduction: Diabetic neuropathy is a long term complication of diabetes that can cause
considerable morbidity in many patients leading to a deterioration of their quality of life. A
deficiency of vitamin D is common in patients with diabetes and low concentrations are
associated with the presence and severity of neuropathy in diabetes. Vitamin D deficiency is
shown to be an independent risk factor for diabetic peripheral neuropathy (DPN).

Methods: This cross-sectional analytical study was conducted in the department of neurology,
Mymensingh Medical College Hospital from October 2017 to September 2018. Total 100 type
2 diabetic patients were included after considering inclusion and exclusion criteria then sample
population was divided into two groups; Group –I : Type 2 diabetic patients with peripheral
neuropathy, Group –II : Type 2 diabetic patients without peripheral neuropathy.

Results: In this study mean serum vitamin D value of group-I and


group-II were 17.50±5.08 ng/ml and 26.33±4.43 ng/ml respectively. It was statistically
significant (p < 0.05). Among the nerve conduction study parameters, in DPN group 82% was
sensory motor and 18% was pure sensory and in them, mean vitamin D level was 17.16 ± 4.59
and 19.03 ± 7.00 ng/ml respectively. In subgroup analysis 24% was axonal had secondary
demyelination,18% had axonal and 8% were demyelinating neuropathy and mean value of
serum vitamin D level was 18.14 ± 5.29,17.20 ± 5.40, and 16.25± 3.77 ng/ml, respectively.

Conclusion: Vitamin D deficiency is associated with diabetic peripheral neuropathy and


vitamin D status needs to be monitored in diabetic patients. Further trials are warranted to
clarify the mechanism leading to DPN in exploring the true role of vitamin D.

125
PN003. CORNEAL SUB-BASAL WHORL-LIKE NERVE PLEXUS: A
LANDMARK FOR EARLY AND FOLLOW-UP EVALUATION IN
TRANSTHYRETIN FAMILIAL AMYLOID POLYNEUROPATHY

Yixuan Zhang*1 ; Xiaoxuan Liu1 ; Yuanjin Zhang1 ; Xiangyi Liu1 ; Shuo Zhang1 ; Ziyuan Liu2 ;
Haikun Wang2 ; Dongsheng Fan1
1Neurology Department, Peking University Third Hospital, China, 2Ophthalmology Department,

Peking University Third Hospital, China


Email Address: 1310301514@bjmu.edu.cn

Introduction: To evaluate the quantification of corneal sub-basal whorl-like nerve plexus


parameters as a sensitive and static marker in early diagnosis of small fiber neuropathy (SFN)
and monitor disease progression in familial amyloid polyneuropathy (FAP) patients.

Methods: Fifteen FAP patients and fifteen healthy controls underwent neurological evaluation
(including clinical scales and electrophysiological tests) and corneal nerve observation.
Corneal nerve fiber density (CNFD), Corneal nerve fiber branch density (CNBD), Corneal
nerve fiber length (CNFL) were detected in the central cornea (conventional method) and the
whorl-like inferocentral cornea (new method). The Langerhans cells (LC) density in each
image was calculated.

Results: Corneal parameters were significantly reduced with disease progression. Only
inferior whorl length (IWL) was significantly reduced in preclinical patients (with normal SSR
and CHEPs results) comparing with age matched controls. IWL, CNFL, CNBD and CNFD were
significantly lower in early phase patients. LC density was significantly increased around the
central whorl in early phase patients than in healthy controls (p=0.009), and was lower in the
progressive patients. Both IWL and CNFL correlated with severity of neuropathy. IWL was
more significantly reduced with the progression of disease than CNFL. The area under the
ROC curve to distinguish FAP with CNFL and IWL was 88.0% and 89.3%, respectively,
exceeding other parameters. The coefficient of variation of the IWL value was significantly
(P<0.0001) smaller than that of the conventional method.

Conclusion: The observation of corneal sub-basal whorl-like nerve plexus can detect SFN in
preclinical FAP patients earlier than traditional electrophysiological methods. IWL is the most
sensitive and reliable parameters and it can best discriminate FAP patients from controls. The
clustering of immature LCs at the whorl area might reflect the inflammatory response of small-
fiber nerves at the very early stage.

126
Neuromuscular Ultrasound (NMUS)

NU001. FEASIBILITY OF SHEAR WAVE ELASTOGRAPHY AND HIGH


DEFINITION COLOR IN EVALUATION OF CARPAL TUNNEL SYNDROME

Joo Hye Sung*1 ; Seol-Hee Baek1 ; Byung-Jo Kim1


1Neurology, Korea University ANAM Hospital, Korea

Email Address: centertruth@naver.com

Introduction: Diagnosis of carpal tunnel syndrome (CTS) is based on clinical findings and
electrodiagnostic tests (EDT). However, EDT has high false negative rates, and the results
often do not reflect the severity of symptoms. The aim of this study is to investigate the value
of shear wave elastography (SWE) and high definition (HD) color in diagnosis and evaluation
of symptom severity in CTS.

Methods: The study prospectively enrolled 39 wrists from 23 patients with EDT-confirmed
CTS, 15 wrists from 12 patients who have CTS symptoms without EDT abnormality and 28
wrists from 14 healthy volunteers. Cross-sectional area (CSA) and longitudinal diameter of
median nerve were measured. SWE of median nerve at the wrist and midforearm were
performed, and the ratio between them was calculated. SWE of abductor pollicis brevis (APB)
and abductor digiti minimi (ADM) were also measured and the ratio was obtained. HD color
was conducted in the longitudinal plane at carpal tunnel.

Results: The CSA of the median nerve at the carpal inlet and longitudinal diameter were
significantly larger for the EDT-confirmed CTS group compared to control group
(13.21[11.28,17.23] vs 7.98[6.53,8.94]; p<0.001, 2.77[2.41,3.08] vs 2.07[1.79,2.24]; p<0.001).
It also had significantly higher median nerve elasticity ratio (2.59[1.77,4.09] vs 1.61[1.32,1.94];
p=0.007). HD color showed significantly higher grades in the former group (p=0.004). In ROC
analysis, a 1.97 cut-off value on SWE ratio revealed sensitivity, specificity, PPV, NPV of
74.4%, 78.6%, 82.9%, 68.8%, respectively (AUC: 0.796[0.687,0.905]). Inter-rater reliability was
excellent for SWE measurements (ICC: 0.908(0.761,0.967)). There was no significant
correlation of CSA, elasticity and HD color grade with clinical symptom scores. In comparison
between the group of CTS patients without EDT abnormality and control group, the former had
significantly larger CSA in carpal inlet and elasticity ratio (9.61[7.21,11.55] vs 7.98[6.53,8.94];
p=0.011, 2.59[0.94,4.65] vs 1.61[1.32,1.94]; p=0.009).

Conclusion: SWE and HD color are helpful supportive tools in diagnosing CTS, especially in
patients who have typical CTS symptoms but normal results in EDT.

127
NU002. SPECTRUM OF NERVE AND MUSCLE ANATOMIC VARIANTS
DETECTED DURING NEUROMUSCULAR ULTRASOUND EXAMINATION: A
RETROSPECTIVE STUDY

Eman Tawfik*
Physical Medicine & Rehabilitation, Faculty of medicine, Ain Shams University, Egypt
Email Address: Eman_Tawfik@med.asu.edu.eg

Introduction: Neuromuscular ultrasound has become an important diagnostic tool for


neuromuscular disorders. The sonographic scanning protocol varies according to the
provisional diagnosis but there are basic elements to assess like nerve and muscle size,
echotexture, vascularity, nerve mobility, dynamic muscle assessment, in addition to the
assessment of surrounding structures and anatomic variants. Recognizing anatomic variants is
important as it may impact treatment and diagnosis. The aim of this study was to
retrospectively analyze the frequency and the spectrum of anatomic variants detected during
neuromuscular ultrasound examination.

Methods: The medical records of 339 patients who were referred for neuromuscular
ultrasound examination during the period from December 2014 to June 2020 were reviewed.
The neuromuscular ultrasound reports and images archive of the patients were analyzed for
presence of nerve and/or muscle anatomic variants. The records which did not show anatomic
variants in the report and images archives were excluded from further analysis. The ultrasound
images which showed anatomic variants were furtherly analyzed for the type of the anatomic
variant, its detailed description, and its diagnostic or therapeutic impact if such information is
available.

Results: Nerve and/or muscle variants were found in 61 of 339 patients (17.9 %). The most
common muscle anatomic variants were accessory flexor digitorum superficialis within the
carpal tunnel, bifid median nerve, persistent median artery, and ulnar nerve
subluxation/dislocation at the ulnar groove. Uncommon anatomic variants were encountered
including abnormal traverse of median nerve within the pronator teres muscle, reversed
palmaris longus, and atypical position of tibial nerve at the medial ankle.

Conclusion: Various nerve and muscle variants were detected in 17.9 % of patients who were
referred for neuromuscular ultrasound examination. Putting the anatomic variants in
consideration when performing neuromuscular ultrasound examination is important as it may
impact diagnosis and treatment.

128
Basic Neuroscience

BN001. CALOTROPIS PROCERA (ROOT) AMELIORATES FUNCTIONS


REGAIN AND ATTENUATES OXIDATIVE STRESS IN A MOUSE MODEL OF
SCIATIC NERVE COMPRESSION INJURY

Ghulam Hussain*1 ; Azhar Rasul2 ; Haseeb Anwar3 ; Shoaib Ahmad Malik4 ; Shamaila Zafar1 ;
Syed Kashif Shahid Kamran1 ; Aroona Razzaq1 ; Tao Sun5
1Neurochemicalbiology and Genetics Laboratory (NGL), Department of Physiology,

Government College University Faisalabad, Pakistan, 2Zoology, Government College


University Faisalabad, Pakistan, 3Physiology, Government College University Faisalabad,
Pakistan, 4Biochemistry, Sargodha Medical College, University of Sargodha, Pakistan, 5School
of Medicine, Huaqiao University, China
Email Address: gh_azer@hotmail.com

Introduction: Peripheral nerve injury is the crucial and complicated health issue affecting the
life quality and capability. This situation not only compromises the physical functions but also
leads to the demise of the affected individual largely due to a lack of effective interventions.
Novel therapeutic strategies must be explored and evaluated to overcome the situation of
partial or complete loss of function. Here, we have inspected the role of Calotropis procera, a
famous medicinal plant, in accelerating the functional retrieval following the mechanically
induced sciatic nerve injury.

Methods: The study was performed in BALB/c mice (n = 16, weight = 30–32 g, age = 8–10
weeks) that were divided into two groups: Normal diet (n = 8) and C. procera diet group (n =
8). A compression injury was induced to the sciatic nerve at mid-thigh region and C. procera
was orally offered mixed in the diet at a dose of 100 mg/kg/day. Both motor and sensory
functions were assessed by muscle grip strength, Sciatic Functional Index (SFI), and hot-plate
tests. Biochemical analyses, haematology, and glucose tolerance were performed to observe
the effects of C. procera on the systemic indexes.

Results: We found a speedy recovery sensory and motor functions (p<0.05). It restored
muscle mass, diminished total oxidant status (TOS), and enhanced total antioxidant capacity
(TAC). The activity of the enzymes i.e., arylesterase and paraoxonase also increased in the
treated group. Similarly, platelet and granulocyte counts were augmented. In addition, the
treated group showed an improved capacity for glucose tolerance.

Conclusion: These results demonstrate that C. procera effectively accelerates the functional
retrieval following an injury to the peripheral nerve. Our findings provide very important
information that can help the pharmaceutical and food industries to figure out plant-based
interventions against traumatic peripheral nerve injuries to develop cost-effective therapeutic
products.

129
BN002. INTRAVENOUS ADMINISTRATION OF POLY(LACTIDE-CO-
GLYCOLIDE) NANOPARTICLE ENCAPSULATING BRAIN-DERIVED
NEUROTROPHIC FACTOR PROMOTES NEUROPROTECTION IN AN
ISCHEMIC STROKE MODEL IN THE RAT

Siti Norsyafika Kamarudin*1 ; Igor Iezhitsa1 ; Minaketan Tripathy2 ; Renad Alyautdin3 ;


Nafeeza Mohd Ismail4
1Pharmacology, University Teknologi MARA (UITM), Malaysia, 2Pharmaceutics,

Adichunchanagiri University, India, 3Scientific Centre For Expert Evaluation of Medicinal


Products, Centre for Expertise of Drug Safety, Russia, 4Pharmacology, International Medical
University, Malaysia.
Email Address: syafika84@yahoo.com / norsyafika7020@uitm.edu.my

Introduction: Poly(lactide-co-glycolide) (PLGA) nanoparticles (NPs) are biodegradable and


biocompatible drug carriers and competent at delivering neuroprotective agents to the brain
following peripheral administration. Brain-derived neurotrophic factor (BDNF) could provide
neuroprotection in brain insult particularly ischaemic injury. We tested the neuroprotective
effect of PLGA nanoparticle-bound BDNF (NPBDNF) on a permanent middle cerebral artery
occlusion (pMCAO) model of ischemic rats.

Methods: Sprague-Dawley rats were divided into 4 groups of 7 rats each. Group 1 was
subjected to sham operation, group 2, 3, and 4 were subjected to pMCAO. Four hours after
pMCAO, group 3 and 4 were intravenously (IV) treated with BDNF and NPBDNF, respectively
at 4 hours post-ischaemic induction. Functional outcome was assessed at 2 and 24 hours after
pMCAO, using the modified Neurologic Severity Score (mNSS) and grid walking. Following
functional assessments, rats were euthanized by terminal cardiac puncture, whereby blood
was taken to assess for neurobiomarker level, the neuron spesific enolase (NSE). The brain
was evaluated to measure the infarct volume.

Results: The NPBDNF treated group showed significant functional improvement in mNSS,
demonstrating a mild injury evidenced by a decreased in mNSS score by 2.0 and 2.1 times,
respectively, when compared with pMCAO and BDNF treated groups. Grid walking test
showed significant improvement by error reduction in contralateral foot slips in rats treated with
NPBDNF as compared with BDNF alone by 2.00 fold. The infarct volume in rats treated with
NPBDNF was significantly smaller by 1.91 fold compared to the pMCAO group and 1.95 fold
compared to the BDNF treated group. These results were further corroborated by the NSE
level estimates.

Conclusion: NPBDNF exhibit a significant neuroprotective effect in the pMCAO model of


ischemia in rats following intravenous administration during the critical period of
neuroprotective window.

130
E-Poster Abstracts

131
Electroencephalogram (EEG)

EG001. THE ROLE OF EEG IN HERPES SIMPLEX ENCEPHALITIS (HSE): AN


EXPERIENCE OF TWO DISTINCT EEG FINDINGS FROM DR SARDJITO
GENERAL HOSPITAL YOGYAKARTA INDONESIA

Agus Nur Salim Winarno*1; Sekar Satiti2; Desin Pambudi Sejahtera3; Atitya Fithri Khairani3
1Neurology Resident, Department of Neurology, Faculty of Medicine, Public Health, and

Nursing Universitas Gadjah Mada, Indonesia 2Neuroinfection Subdivison, Department of


Neurology, Faculty of Medicine, Public Health, and Nursing Universitas Gadjah Mada,
Indonesia, 3Epilepsy Subdivision, Department of Neurology, Faculty of Medicine, Public
Health, and Nursing Universitas Gadjah Mada Yogyakarta, Indonesia
Email Address: agusnur_salim@yahoo.com

Introduction: Encephalitis is considered as neurological emergency because it can result in


death or severe disability. HSE is the most common form of encephalitis and it risks to serious
condition and can be fatal if untreated properly. Precised diagnosis and treatment is a key to
reduce its mortality and morbidity. EEG is one of ancillary examination to support diagnosis
and management of HSE, but its exact role is unclear.

Results: We describe two cases of HSE that admitted to our neurology ward. Each case has
two distinct EEG results. In case number 1, there was a 40-year-old male came with acute
seizure, behavioral changes, and intermittent fever. The history, physical exam, laboratory,
lumbar puncture, brain MRI with contrast led to diagnosis of HSE. He also underwent EEG
which result was periodic lateralized epileptiform discharge (PLED). He had acyclovir and
phenytoin medication. In day 14, he was discharged. In case number 2, there was a 47-year-
old female came with reduced consciousness, recurring seizure, and fever. The history,
physical exam, laboratory, lumbar puncture, brain MRI with contrast led to diagnosis of HSE.
She also underwent EEG which result was focal continuous polymorphic delta (CPD) slowing.
She had acyclovir and phenytoin medication. In day 16, she was discharged. Two weeks later,
EEG was conducted and the result was normal.

Conclusion: EEG is the most accessible and inexpensive diagnostic tool for evaluation of
CNS function. EEG might be useful in the acute stage of HSE. It is more sensitive than CT but
its specificity is low. It has benefit to determine the diagnosis of HSE, presence of seizure,
treatment, and prognosis. PLED and focal CPD slowing are characteristic finding of EEG in
HSE but they are not pathognomonic. It is needed to consider other clinical data in order to
achieve final decision making.

132
EG002. AN EXPLORATORY STUDY OF BRAIN WAVES AND
CORRESPONDING BRAIN REGIONS OF FATIGUE POST-CALL DOCTORS
USING QUANTITATIVE ELECTROENCEPHALOGRAM

Gregory Xavier*1; Anselm Su Ting2; Norsiah Fauzan3


1Faculty of Medical and Health Sciences, Universiti Malaysia Sarawak (UNIMAS),

Malaysia 2Department of Public Health and Community Medicine, Universiti Malaysia Sarawak
(UNIMAS), Malaysia, 3Department of Cognitive Science, Universiti Malaysia Sarawak
(UNIMAS), Malaysia
Email Address: gregshc@yahoo.co.uk

Introduction: It is common to find doctors working long and odd hours and many at times
without rest and sleep. Despite the evidence of adverse risk, patient safety is jeopardised
under the hands of fatigue doctors. Such working hours have yet to change in many places. It
is argued that with such training and subsequent experience, issues pertaining to patient
safety risk reduces. Fatigue too is argued as subjective, as those who can withstand the stress
still performs. Nevertheless, undeniably working under fatigue is not safe for both patient and
doctor. This study is a novel attempt to explore and objectify the state of fatigue using
quantitative EEG among post-call doctors.

Methods: Seven volunteer post-call doctors were recruited to go through an EEG recording at
rest and subsequently while carrying out a Stroop Test exerting their cognitive function to work.

Results: The doctors have worked up to 34 hours in a row and with only an average sleep of
1.5 hours. During the task, there is a statistically significant increase in theta (frontal and
occipital regions) and beta (occipital region) band power. Frontal lobe shows an increase in
alpha band power but reduced in other regions. The Stroop Test conducted indicated that the
speed of answering the congruent trials was faster than the incongruent trials significantly with
a slightly more correct result.

Conclusion: Frontal increase of theta and alpha band power implies cognitive fatigue. This
objectively presents that fatigue doctors are under more strain while carrying out a task and
the implicated regions of brain stimulated by the task correspond accordingly.

133
EG003. ELECTROENCEPHALOGRAPHY (EEG) ABNORMALITIES PATTERN
IN BRAIN TUMOR AND STROKE

Indra Sari Kusuma Harahap*1; Ahmad Asmedi1; Sri Sutarni1


1Department of Neurology, Faculty of Medicine, Public Health and Nursing (FKKMK),

Universitas Gadjah Mada, Yogyakarta, Indonesia.


Email Address: iin_999@yahoo.com

Introduction: Electroencephalography (EEG) is commonly used to identify epileptiform


discharge confirming the diagnosis of epilepsy. However, EEG is rarely used in brain tumor
and stroke patients. The EEG abnormalities in brain tumor and stroke patients are not well
determined. Aim: To identify the EEG abnormalities pattern in brain tumor and stroke patients.

Methods: Thirty-seven brain tumor patients and 28 stroke patients, during their hospitalization
in general neurology ward and stroke unit, underwent the EEG examination at Electromedical
Clinic in Sardjito Hospital, Yogyakarta, Indonesia.

Results: EEG abnormal result was found in 32 (86.5%) brain tumor patients and 20 (71.4%)
stroke patients. The most common type was the abnormalities of both epileptiform discharge
and background. It was found in 19 (51,4%) brain tumor and 9 (32,1%) stroke patients. The
second common type was the abnormality of background only; 5 (13,5%) brain tumor and 6
(21,4%) stroke patients. More than a half of brain tumor (73%) and stroke (53,6%) patients
showed the existence of epileptiform discharge; dominated by sharp wave. The existence of
epileptiform discharge may reveal the possibility of functional brain lesion; meanwhile the
existence of background abnormality may reveal the possibility of structural brain lesion.

Conclusion: EEG in brain tumor and stroke patients showed the same abnormalities pattern,
which is the abnormalities of epileptiform discharge and background. It may suggest that there
are both structural and functional brain lesions in brain tumor and stroke cases.

134
EG004. THE NEED FOR ELECTROENCEPHALOGRAPHY SMARTPHONE-
BASED TELEMEDICINE TO ASSIST NEUROLOGISTS WORKING IN
SECONDARY HOSPITALS IN INDONESIA

Kennytha Yoesdyanto*1; David Susanto1; Karema Winifred1; Arthur H. P. Mawuntu1; Herlyani


Khosama1
1Sam Ratulangi University, Prof R. D. Kandou Hospital, Manado, Indonesia.

Email Address: kennytha.yoesdyanto@gmail.com

Introduction: Electroencephalography (EEG) is a very important method in establishing


epilepsy diagnosis but interpreting the result could be challenging for neurologists working in
secondary hospitals. Smartphone-based telemedicine (SBTM) has been widely used but the
application of this technology for EEG in Indonesia is not available yet. The 4G technology has
covered approximately 82% of the country’s territory so we think there might be an opportunity
to apply the SBTM to assist EEG in Indonesia. The study objective is to identify the need for
EEG SBTM among neurologists in secondary hospitals in Indonesia.

Methods: We made a web-based survey using a 14-items questionnaire made in Google


Forms application. The target respondents were non-epilepsy/clinical neurophysiology
consultant neurologists, working in non-type A hospitals, and operating an EEG machine. The
questionnaire was designed to study the number of EEG cases, network connection, and the
need for EEG SBTM.

Results: There were 210 neurologists nationwide responded to the survey, representing about
15% of all neurologists in Indonesia, working in 32 of 34 provinces. Only 109 respondents
were eligible. Most of them aged 41-50 years old and worked in North Sumatra, West Java,
and Bali provinces (27.5%, 13.7%, and 9.2% subsequently). About 64.2% of them have 1-10
EEG cases monthly. Although >85% of the respondents had previous EEG training, 74% still
encounter difficulty in interpretation. Only 1.8% do not have a smooth network connection. We
found that 23.8% and 73% of them agreed and strongly agreed that they need an EEG SBTM.
Respondents mostly prioritized the affordable submission fee, high data security, and bilingual
options for the application.

Conclusions: Most neurologists in secondary hospitals in Indonesia have problems in EEG


interpretation and agreed that they need an EEG SBTM. This provides an opportunity to create
a smartphone-based application to assist them.

135
EG007. EEG POWER ASYMMETRY AS PREDICTOR OF RECOVERY IN
ACUTE ISCHEMIC STROKE PATIENTS

Ahmad Asmedi*1; Dyanne Paramita Arindra Putri1; Wahyu Wihartono2; Abdul Gofir1
1Neurology, Gadjah Mada University, RSUP Dr. Sardjito, Indonesia, 2Neurology, RSPAU dr. S.

Hardjolukito, Indonesia.
Email Address: ahmad.asmedi@ugm.ac.id

Introduction: After a stroke, optimal recovery of movement depends on the potential for
neuroplasticity which allows surviving brain regions to reorganize into effective capable
networks of supporting arm and hand function. There is evidence that reduced lateralized
activation reflects the increased activity in the contralesional hemisphere, which reduces the
extent of interhemispheric balance as demonstrated in many stroke studies. QEEG provides
an objective measure of EEG asymmetry that correlates with clinical status and brain
asymmetry seen on MRI. The objective of this study is to know whether EEG power
asymmetry in the acute phase of stroke can predict stroke recovery after 90 days using the
NIHSS score.

Methods: All participants in this study underwent qEEG examination when they were in the
acute phase of stroke. There are 4 power asymmetry (delta, theta, alpha, and beta) and ROC
analysis are performed to determine the cut off of each band. On the 90th day after stroke
onset, NIHSS examination was conducted. The statistical analysis was done by comparing the
means of NIHSS score at 90 days in a group with and without asymmetry power of each band
in qEEG.

Results: Fifty-one subjects have accomplished this study. There are 16 participants with
asymmetry and 35 participants without asymmetry result in alpha power. A significant result
between alpha band power asymmetry 4+4,27 and symmetry 1,7+2,6 with 90th day of NIHSS
(p=0,025) but there is no significant results in delta (p=0,066), theta (p=0,973), and beta
asymmetry (p=0,323).

Conclusion: Brain asymmetry quantitative EEG can describe the value of NIHSS day 90.

136
EG008. ELECTROENCEPHALOGRAPHIC CHANGES ON PASSIVE
LISTENING TO 3 INDIAN CLASSICAL MUSIC SCALES

KIRTHANA KUNIKULLAYA U*1; Arun Sasidharan2; Vijayadas1; Anjani Bhushan Kumar3;


Mamta Sanjeeva Vernekar1; Radhika Kunnavil4; Jaisri Goturu5; V S Prakash6; N S Murthy7
1Physiology, M S Ramaiah Medical College, India, Brain research lab, 2Axxonet,

India, 3Neurology, Ramaiah Memorial Hospitals, India, 4Community Medicine, Ramaiah


Medical College, India, 5Physiology, International Medical School, India, 6Cardiology, Ramaiah
memorial hospitals, India, 7Division of research and patents, Ramaiah Medical College, India.
Email Address: kirthana.rguhs@gmail.com

Introduction: Music, though used predominantly as an entertainer, studies have shown that
music has a significant effect on the body and the mind. But, most of the scientific evidences
are now being created for western music (Mozart, Beethoven etc.). Unfortunately, evidence
regarding the health benefits of Indian music is extremely meagre. In this study the effect of
Indian music on health with special reference to electrophysiological parameters was
evaluated. Sama Veda enlists the various scales (ragas) and their health benefits. Objective:
To evaluate the effect of 3 select Indian classical ragas on stress markers and brain via
electroencephalogram (EEG).

Methods: In this triple blind study, 140 subjects aged 18-30 years were randomly divided into
3 groups (A, B, C, D; 35 in each group). The first 3 groups, received music intervention (3
ragas), while the group D served as control. Stress was measured before and after
intervention using State Trait Anxiety Inventory (STAI). EEG (19 channel, Galileo NT) was
recorded before, during and after music (each condition 10 min). Analysis of data was done
using SPSS and BESS Software (Axxonet System Technologies Pvt. Ltd.) and MATLAB
software (Mathworks).

Results: The 3 groups were comparable. Stress reduced with all 3 ragas. Between conditions
effects in theta, alpha, beta and gamma bands were observed which got restricted to only
alpha band at right frontal region after collapsing the conditions. First 2 minutes data revealed
predominant effects on raga A on theta, alpha, beta, gamma power; raga B on alpha, beta
gamma power and raga C showed a significant difference all 5 wave power of EEG.

Conclusion: Music reduced stress. It is the scale presented as a whole, as it unfolds, that
affected the EEG. The significant hemispheric differences with each raga, particularly, frontal
asymmetry is in line with the model of hemispheric specialization concerning perceived
positive or negative emotions proposed by Heilman.

137
Epilepsy

EP001. STATUS EPILEPTICUS AND INTRACTABLE SEIZURE IN HERPES


SIMPLEX ENCEPHALITIS PATIENT: CASE SERIES

Atitya Fithri Khairani*1; Sekar Satiti1; Rangga Adi Nugraha1; Ari Astuti1
1Neurology Department, Faculty of Medicine, Public Health and Nursing Gadjah Mada

University, Indonesia
Email Address: atityafk@gmail.com

Introduction: Herpes Simplex Encephalitis (HSE) is a rare disorder caused by infection of the
CNS by HSV that could have a potentially fatal outcome particularly with delayed treatment.
The early presentation is relatively nonspecific consisting of fever, headaches, seizures and
unconsciousness. Seizures have been associated with death in HSE. This case report aims to
publish case series of status epilepticus and intractable seizure in HSE patient at Sardjito
Hospital Yogyakarta.

Results: CASE 1: A 26year-old male was admitted to the ER Sardjito Hospital in 2018
presented with GTCS and behavior changes. GCS of this patient was E4V4M6 and there were
no signs of meningeal irritation, no weakness found in extremity but increase of physiological
reflex indicating UMN sign. After being transferred to the ward, patient developed to status
epilepticus, then patient was referred to ICU; midazolam & propofol were administered to
reduce seizures. Laboratory, CSF analysis and MRI were consistent with HSE. After
aggressive treatments with anti-edema, antiviral and multiple antiepileptic drug (AED), there
was a reduction in seizure but sometimes patient still developed GTCS. He was discharged
after 25 days treatments with full consciousness, reduction of seizure frequency but the focal
aware seizures were still remained. CASE 2: A 29-year-old female was admitted to the ER
Sardjito in 2019 presented with focal to bilateral tonic-clonic seizure and status epilepticus. The
complaints begin with acute progressive headache, fever, visual hallucinations and weakness
of the left limbs. GCS of this patient was E2VTM2 (on midazolam) and there were no signs of
meningeal irritation. Laboratory and CSF analysis were consistent with HSE. CT scan showed
cerebral edema. After treatments with anti-edema, antiviral and AED, the patient regained full
consciousness and there was a reduction of seizure frequency but focal aware seizures were
still remained.

Conclusion: Seizures in HSE are reported to be intractable seizures and more likely to
develop status epilepticus. The seizures are typically refractory to AED, often requiring
combination and aggressive treatments.

138
EP002. STATUS EPILEPTICUS DURING PREGNANCY: A CASE REPORT

Atitya Fithri Khairani*1; Desin Pambudi Sejahtera1; Elfi Rahmi1


1Neurology Department, Faculty of Medicine, Public Health and Nursing Gadjah Mada

University, Indonesia
Email Address: atityafk@gmail.com

Introduction: Status epilepticus is a rare but potentially life-threatening complication that


women with epilepsy may experience during pregnancy. Poor compliance may contribute to
the occurrence of status epilepticus, resulting in the need for substantial increased in
anticonvulsant dosing to suppress seizures. This case report aims to report case status
epilepticus in patients during pregnancy at Dr. Sardjito Hospital.

Results: CASE: A 30-years-old G5P2A2, 25 weeks gestation was admitted to the Emergency
Room Sardjito Hospital Indonesia in December 2018, presented with multiple seizure episodes
of 3 days duration. She was known epilepsy patient diagnosed about 3 years ago and was on
phenytoin but was not on regular medications after she knew that she got pregnant. Physical
examination revealed Glasgow Coma Scale (GCS) of E2VTM2 (on midazolam). Patient had
normal neurological examination. After aggressive treatments with midazolam and antiepileptic
drug, the patient regained full consciousness.

Conclusion: Pregnancy sometimes worsens the frequency of seizures in known epileptic


women. One of which is that the physiological changes in pregnancy can lead to
subtherapeutic serum level of the drugs and discontinuing medications during pregnancy can
precipitate status epilepticus.

139
EP004. SEIZURE AS EARLY SIGN PILOCYTIC ASTROCYTOMA: CASE
REPORT

Atitya Fithri Khairani*1; Rusdy Ghazali Malueka1; Desin Pambudi Sejahtera1; Wilda Khairani
Dalimunthe1
1Neurology Departement, Faculty of Medicine, Public Health and Nursing Gadjah Mada

University, Indonesia
Email Address: atityafk@gmail.com

Introduction: Pilocytic astrocytoma is a rare type of brain tumor that occurs mostly in children.
In most cases, the tumor is usually benign, slow growing tumor that usually does not spread to
surrounding brain tissue. Symptoms of a pilocytic astrocytoma will vary depending upon the
size and location of the tumor. This case report aims to publish pilocytic astrocytoma case with
least clinical sign at Dr. Sardjito Hospital Yogyakarta, Indonesia.

Results: A 20-year-old female was admitted to the outpatient clinic Sardjito Hospital in
September 2019 presented with focal to bilateral tonic clonic seizure. It was mentioned that the
frequency of the seizure was 2 times/ week and the patient was also experiencing non-
progressive paroxysmal headache. Head CTscan showed ischemic Stroke. She has no history
of hypertension, diabetic Melitus, smoking and stroke. There were no abnormalities found on
neurological and cognitive examination. One week later, MRI was performed and showed a
mass in the right frontal parietal lobe with a size of 3.21 x 3.40 x 3.14 cm, consistent with
pilocytic astrocytoma finding (WHO grade 1). EEG showed diffuse abnormal epileptiform
discharges indicating generalized seizures. Surgery treatment option is denied by the patient.
Later on, she was given levetiracetam 2x500mg as antiepileptic drug and paracetamol as pain
reliever. This treatment regimen resulting reduction of seizure frequency up to once per month.

Conclusion: Pilocytic astrocytoma can be presented with only seizure and nonspecific
headache with normal neurological and cognitive examination.

140
EP005. RECURRENT FOCAL SEIZURE IN SUBDURAL HEMORRHAGE
PATIENT AFTER ANTICOAGULANT ORAL THERAPY: CASE REPORT

Atitya Fithri Khairani*1; Paryono1; Distya Hening1; Siti Zulaikhah1


Neurology Department, Faculty of Medicine, Public Health and Nursing Gadjah Mada
University, Indonesia
Email Address: atityafk@gmail.com

Introduction: Subdural Hemorrhage (SDH) which occurred during warfarin treatment is a


bleeding complication related to anticoagulation intensity with a high level of mortality.
Subdural hemorrhage can be associated with different neurological signs and symptoms
including seizures. There is an increased risk of seizures in the first week after SDH, and this
risk may persist for some time after surgical decompression. This case report aims to publish
recurrent seizure in SDH patient after oral anticoagulant therapy at dr. Sardjito Hospital
Yogyakarta, Indonesia.

Results: A 25-year-old male patient was admitted to the Emergency Room Sardjito Hospital in
October 2019, presented with recurrent focal seizure accompanied by impaired awareness,
cephalgia with sign of increased intracranial pressure, and right hemiparesis. The patient has
history of open-heart surgery with valve replacement and atrial fibrillation with normal
ventricular response. He was on warfarin medication since September 2019. Physical
examination revealed Glasgow Coma Scale (GCS) of compos mentis state and there were
sign of meningeal irritation, right hemiparesis and increased physiological reflex on right
extremity was observed, indicating upper motor neuron sign. Head CT scan revealed subdural
hemorrhage on left fronto-temporo-parieto-occipitalis region and on the falx cerebri. After being
transferred to stroke unit, the patient developed more frequent seizure, intense cephalgia and
became somnolent. The seizure resolved after treatment with anti-edema, antiepileptic drug
and craniotomy decompression. After 14 days of admission, cardiologist started low dose
heparin to prevent valve occlusion. He was discharged from hospital after 25 days of
admission with full consciousness, seizure-free and absence of neurological deficits.

Conclusion: Treating the underlying cause and aggressive treatment is important after initial
treatment with antiepileptic drug on subdural hemorrhage patient with recurrent seizures.

141
EP006. RESEARCH ON EPILEPSY TREATMENT IN MALAYSIA: A SCOPING
REVIEW

Firzanah Adabiah Zulkernain*1; Hana Maizuliana Solehan1


1Faculty of Medicine and Health Science, Universiti Sains Islam Malaysia, Malaysia

Email Address: firzanahadabiah@yahoo.com

Introduction: The treatment of epilepsy patients is focused on three main goals: controlling
seizures, avoiding or minimizing the treatment side effects and maintaining or restoring the
quality of life. In Southeast Asia, there are about 1% of the population with epilepsy and only
10-20% of the patients receive appropriate treatment. The objective of this scoping review is to
identify the research pattern of epilepsy treatment in Malaysia and its gap.

Methods: Arksey and O’Malley’s scoping review methodology framework was used to guide
and conduct this scoping review. Databases of PubMed, EBSCOhost, and Google Scholars
were systematically searched using the keywords of “epilepsy”, “seizure”, “treatment”,
“therapy”, “management”, “antiepileptic drugs” (AEDs) and “surgery”. Studies of epilepsy
patients among the Malaysian population with no age preference were selected.

Results: A total of 9405 articles were retrieved. Thirty-four articles were included in the final
review; from these, 26 articles were published between 2010 to 2019. Ten out of 34 papers
reported on AEDs, six articles focused on genetic evaluation for better treatment, three articles
evaluated AEDs adherence, two articles reported the side effects and monitoring of AEDs and
one article studied on complementary medicine. Four articles analyzed the surgical aspects of
epilepsy. Six articles explored education and psychological interventions as part of epilepsy
treatment. One study reported on diet therapy and one article on neuromodulation.

Conclusion: There have been emerging studies on epilepsy treatment in Malaysia for the last
decade. However, more studies need to be conducted such as the treatment gap among rural
patients; other alternative therapies in poorly controlled epilepsy and the use of advanced
methods for educational intervention in epilepsy care. It is essential to explore more themes in
the research of epilepsy treatment in Malaysia for the development of better management of
epilepsy patients.

142
EP008. DYKE-DAVIDOFF-MASSON SYNDROME: A CASE REPORT IN A
FILIPINO MALE ADOLESCENT

Lalaine Villaflor-Oida*1; Alejandro Bimbo Diaz1; Maria Antonia Aurora Valencia1


1Department of Neurology and Psychiatry, University of Santo Tomas, Philippines.

Email Address: lalainebvillaflormd@gmail.com

Introduction: A 17-year-old male who presented with recurrent left focal seizure with
secondary generalization. He was born of a nonconsanguineous marriage, home-delivered
vaginally, full term with no perinatal complications. He had normal growth and developmental
milestones until at 7-month-old when he had febrile status epilepticus. Since then he was left
with residual left hemiplegia, dysarthria, cognitive delay and recurrent seizure occurring twice
daily. He was able to go to school with minimal supervision from his family for hygiene and
safety. Refractory seizures, hemiparesis, facial asymmetry, and intellectual disabilities along
with brain imaging evidence of cerebral hemiatrophy with compensatory calvarial thickening
and subsequent hyperpneumatization is consistent with Dyke-Davidoff-Masson Syndrome
(DDMS). A rare clinico-neuroradiologic condition occurring in fetal or early childhood period as
a consequence of chronic brain insult. Diagnosis is established clinically with characteristic
brain imaging findings. Multidisciplinary intervention is essential, primarily to optimize seizure
control as well as provide quality of life.

143
EP010. EXTINGUISHING FIRES USING TOCILIZUMAB: MALAYSIA’S FIRST

Chee Yong Chuan*1; Lim Chong Hong2; Ong Beng Hooi3


1Department of Medicine, School of Medical Sciences, Health Campus, Universiti Sains

Malaysia, Kubang Kerian, Kelantan, Malaysia, 2Department of Medicine, Hospital Sultanah


Bahiyah, Malaysia, 3Neurology Unit, Kedah Medical Centre, Malaysia
Email Address: cheeyongchuan@gmail.com

Introduction: Febrile infection related epilepsy syndrome (FIRES) is a devastating epileptic


encephalopathy with antecedent febrile infection preceding the onset of refractory status
epilepticus (RSE), without the evidence of structural, toxic or metabolic cause. FIRES often
manifest initially with a febrile infection preceding the onset of the acute phase with prolonged
duration of RSE, usually lasting days to months, followed by the chronic phase with refractory
epilepsy and neurological impairment without a silent period between the two phases. The
pathophysiology of FIRES is largely unknown but has been hypothesized to be due to
excessive cytokine release perpetuating the underlying seizure. This is supported by the fact
that children with FIRES exhibit high levels of cytokines, including interleukin-6, in the serum,
even more so in the CSF. Conventional immunotherapies such as steroid, IVIG, plasma
exchange, rituximab have previously been considered for the treatment of FIRES but has been
disappointing so far. Following a small retrospective observational case series showing
dramatic cessation of RSE in six out of seven adults with new onset RSE, the therapeutic
potential with Tocilizumab certainly needs to be further investigated. We report a successful
therapeutic encounter using Tocilizumab, a humanized monoclonal antibody against
interleukin-6 receptor to treat RSE in a 14-year-old girl with FIRES following dismal outcome
using conventional immunosuppressive strategies.

Results: The administration of IV Tocilizumab at a single dose of 4mg/kg has successfully


terminated RSE within 72 hours. Exhaustive workup to exclude metabolic, structural,
paraneoplastic, autoimmune and infectious causes has been unrevealing. The electro-clinical
features, brain imaging and outcome for this patient are illustrated.

Conclusion: The overproduction of cytokines and the poor performance of immunotherapies


implicate a putative role of inflammation in FIRES. Our case showed that complete termination
of RSE was achieved after Tocilizumab administration suggesting it may be a life-saving drug
for this catastrophic condition.

144
EP011. SAFETY OF COMPULSORY GENERIC SWITCHING FROM BRAND-
NAME TO GENERIC LEVETIRACETAM IN OUTPATIENT EPILEPSY CLINIC
OF TERTIARY HOSPITAL, KEDAH

Chew Beng Hoong*1; Khor Lian Fen1; Rahimi B Mohd Ariff1; Hoe Chui Ying1; Choy Wang
Lynn1
1Pharmacy, Hospital Sultanah Bahiyah, Malaysia

Email Address: phicology@gmail.com

Introduction: The rate of generic switching of levetiracetam is the highest in Hospital Sultanah
Bahiyah (HSB), Alor Setar. Yet, limited studies explored the efficacy and safety of generic
substitution of Levetiracetam.

Methods: This study includes only patients taking inventor brand Tablet Levetiracetam at least
90 days prior overnight switch and generic brand Tablet Levetiracetam at least 90 days after
switch. Subjects were switched abruptly (after April 2018) from branded levetiracetam to
generic levetiracetam while concomitant medications remained unchanged. All our patients
used only one type of generic Levetiracetam (Torleva 500 mg manufactured by TORRENT
PHARMACEUTICALS, India) KKM-39/2017-S (U) 3/4/2017- 2/4/2020. Tolerability was
assessed by adverse events experienced by patients while Efficacy of treatment was assessed
by the emergence or absence of fit in patients post switching to generic Levetiracetam, in
which the retrospective data were traced based on clinic visit notes from electronic medical
record (e-HIS).

Results: The switching increases fit severity (emergence of fit) is statistically significant
(p=0.031, < 0.05). The percentage of fit was noted to increase from 61.9% to 72.2% post
switching of generic Levetiracetam, whereas percentage of patients who are able to achieve fit
free decreases from 38.1% to 27.8%. However, the side effects experienced by patient pre-
and post-switching is not significant with a percentage of 3.1% (p=0.508).

Conclusion: There is difference in efficacy between branded and generic levetiracetam while
no difference in terms of tolerability and compliance of patient after switching.

145
EP012. SEGREGATION OF GABRA1 AND ERBB4 MUTATIONS IN A FAMILY
WITH GENETIC GENERALIZED EPILEPSY

Chung-Kin Chan*1; Kheng-Seang Lim2; Siew-Kee Low3; Chong-Tin Tan2; Ching-Ching Ng1
1Genetics and Molecular Biology, Institute of Biological Sciences, Faculty of Science,

University of Malaya, Malaysia, 2Division of Neurology, Department of Medicine, Faculty of


Medicine, University of Malaya, Malaysia, 3Cancer Precision Medicine Center, Japanese
Foundation for Cancer Research, Japan
Email Address: c_c_kin@siswa.um.edu.my

Introduction: Genetic generalized epilepsy (GGE) accounts for 15-20% of the epilepsy cases.
It is termed “genetic” as it has no known or suspected aetiology, other than possible hereditary
predisposition. The objective of this study is to identify causative variants of GGE in a Chinese
family with variable epilepsy phenotypes.

Methods: Whole exome sequencing was first conducted on the proband. Candidate variants
were validated, and the segregation study with other family members was performed using
Sanger DNA sequencing.

Results: The proband was a 30-year-old female with mixed syndromes of juvenile myoclonic
epilepsy and temporal lobe epilepsy. Whole exome sequencing identified two mutations in the
proband: c.448G>A in GABRA1 and c.1972A>T in ERBB4. Segregation analysis showed that
the GABRA1 mutation was inherited from the father whereas the ERBB4 mutation was
inherited from the mother. However, no history of epilepsy was observed in both the parents.
The two mutations were also detected in her affected sister diagnosed with GGE without
myoclonic jerks. Meanwhile, these mutations were absent in the unaffected brother.
Interestingly, only the GABRA1 mutation was found in the daughter of the proband, who was
diagnosed as focal epilepsy.

Conclusion: Based on the genetic data and clinical evidence, we hypothesize that the GGE in
this family may be largely caused by the inheritance of both susceptible GABRA1 and ERBB4
mutations. GABRA1 and ERBB4 have been shown to be interacting with each other in mouse
model. Further studies are needed to support these findings.

146
EP013. SUPERIOR MIGRATION PROPENSITY OF DENTAL PULP STEM
CELLS IN IN VITRO AND IN VIVO MODELS OF EXCITOTOXIC
NEURODEGENERATION

Sivapriya Senthilkumar*1; Chaitra Venugopal1; Shobha K1; Bindu M Kutty2; Anandh


Dhanushkodi1
1Regenerative Medicine, Manipal Institute of Regenerative Medicine, India, 2Neurophysiology,

National Institute of Mental health and Neurosciences, India


Email Address: ohmnamahsivaya04@gmail.com

Introduction: Mesenchymal stem cell therapy has gained colossal attention as a therapeutic
alternative in treating neurodegenerative diseases (ND). The challenges in cell-based therapy
comprises of defining a suitable cell type expressing homing factors, the precise
injection/administration timing based on blood brain barrier (BBB) dynamics and the route of
administration that would aid in functional recovery. Even though, bone marrow mesenchymal
stem cells (BM-MSC) were extensively investigated for their role in treating spectrum of
diseases, the limitations like painful bone marrow extraction, modest yield and poor CNS
engraftment following systemic injections prompt to search for an alternate source of MSCs.
We hypothesize that neural crest derived dental pulp stem cells (DPSC) would be a better
candidate for treating ND due to its ease isolation, higher yield and for their inherent propensity
towards neural lineage. We investigated the migratory potential of DPSC vs BM-MSC in in-
vitro and in vivo models of kainic acid (KA) induced neurodegeneration.

Methods: Trans-well, matrigel, and chorioallantoic membrane (CAM) migration assays were
carried out in an in vitro model of neurodegeneration. We assessed the CNS engraftment
potential of DPSC/BM-MSC following intravenous administration in animal model of KA
mediated hippocampal neurodegeneration and also studied the role of BBB damage after
excitotoxicity to facilitate efficient homing of DPSC/BM-MSC. Functional recovery was
assessed by a battery of behavioural tests after stem cell injection.

Results: Following KA treatment to neurons in vitro, the migration potential of DPSC towards
the degenerating site was significantly higher than BM-MSC. To validate the in vitro
observations, KA administration resulted in BBB damage and intravenous administration of
DPSC in synchronization with KA mediated BBB damage resulted in prominent CNS
engraftment and behavioral recovery compared to BM-MSC.

Conclusion: Neural crest originated DPSC might possess better CNS engraftment following
systemic injection.

147
EP015. RELATIONSHIP BETWEEN POLYMORPHISM SCN1A AND HISTORY
OF STATUS EPILEPTICUS IN EPILEPTIC PATIENT: PILOT STUDY

Atitya Fithri Khairani*1; Sri Sutarni1; Rusdy Ghazali Malueka1; Eti Nurwening Sholikhah2;
Audiza Luthffia3; Iqbal Amri Fauzal4; Sekar Satiti1; Cempaka Thursina Srie Setyaningrum1
1Neurology Department, Faculty of Medicine, Public Health and Nursing Gadjah Mada

University, Indonesia, 2Pharmacology Department, Faculty of Medicine, Public Health and


Nursing Gadjah Mada University, Indonesia, 3Internship Doctor, Cibinong Regional Hospital,
Indonesia, 4Internship Doctor, PDHI Hospital Yogyakarta, Indonesia
Email Address: atityafk@gmail.com

Introduction: From a genetic point of view, epilepsy is a polygenic multifactorial syndrome.


The SCN1A genes belong to a family of genes that provide instructions for making sodium
channels. Sodium channels are integral membrane proteins which play a central role in
neuronal membrane excitability and action potential generation. Alpha subunit of voltage gated
sodium channels encoded by SCN1A gene is pivotal for neuronal signalling. Polymorphism in
genes related to neurotransmission are believed to increase the susceptibility to epilepsy, but
in status epilepticus (SE), this is still unknown. The purpose of this study was to investigate
relationship between polymorphism SCN1A rs 2298771 and history of status epilepticus in
epileptic patient.

Methods: This was a cross sectional study located at Sardjito Hospital, Yogyakarta. Epileptic
patients were recruited in this study. Patients were divided into 2 groups namely status
epilepticus group and non-status epilepticus group. Genetic polymorphisms in the candidate
genes were detected in 35 epileptic patients by Polymerase Chain Reaction (PCR) and
Restriction Fragment Length Polymorphism (RFLP).

Results: The mean age of the subjects on this study was 29.34 years for SE group and 31.4
years for non-SE group. From 14 SE patients, 4 subjects (28,6%) had genotype AA, 7 subjects
(50%) had AG genotype, and 3 subjects (21,49%) had GG genotype. From 21 non-SE
patients, 11 subjects (52,3%) had genotype AA, 3 subjects (14,3%) had AG genotype, and 7
subjects (33.4%) had GG genotype. There were no statistically significant differences in the
genotype distributions between case and controls (p = 0.163).

Conclusion: No significant association between SCN1A rs 2298771 gene polymorphisms and


history of status epilepticus.

148
EP016. CORTICAL INVOLVEMENT OF POST STROKE EPILEPSY IN
CHILDREN: A SYSTEMATIC REVIEW

Grace Elizabeth Romorani Sigumonrong*1


1Faculty of Medicine, North Sumatera University, Indonesia

Email Address: graceromorani@gmail.com

Introduction: Epilepsy as an incident after stroke may cause disability in children. This very
detrimental effect makes particular marker for knowing the tendency to develop epilepsy after
stroke at later time very crucial. The aim of this study is to explain cortical involvement in post
stroke epilepsy incident in children.

Methods: The researcher searched in the Medline and Cochrane Library electronic databases
(2000-2020) to identify observational studies of post stroke epilepsy in children. The inclusion
criteria for the selected studies were accessible in full-text, English language, <19 year-old
children with post stroke epilepsy, additional assessments and adequate follow-up. The
researcher checked the validity of the studies with Critical Appraisal Skills Programme (CSAP)
checklist on cohort studies.

Results: A total of 7 retrospective cohort studies which involved 603 children were included.
The incidences of epilepsy after stroke were varied. Early onset strokes have higher tendency
to cause post stroke epilepsy. The features of cortical involvement were found radiologically
(CT scan and MRI) in 6 studies, and one study using Electroencephalography (EEG). Cortical
involvement has been reported in post stroke epilepsy. It was reported to have the highest
percentage in early seizure after stroke.

Conclusion: Cortical involvement is an important marker of determining the prognosis of


stroke in children, regarding the incidence of subsequent epilepsy.

149
EP017. BIOLOGICAL POTENTIAL AND NEUROPROTECTIVE ACTIVITY OF
PECTOLINARIN IN THE MANAGEMENT OF NEURODEGENERATIVE
DISORDERS: PHYTOTHERAPEUTIC IMPORTANCE THROUGH DATA
ANALYSIS OF VARIOUS SCIENTIFIC WORKS

Dinesh Kumar Patel*1; Kanika Patel1


1Faculty of Health Sciences, SIHAS, SHUATS Prayagraj, U.P., India

Email Address: dkpatel.rs.phe@itbhu.ac.in

Introduction: Flavonoidal class chemical have numerous medicinal importance and


pharmacological activities. Acetylcholinesterase (AChE) and Butyrylcholinesterase (BuChE)
enzymes play an important role in various forms of neurodegenerative disorders. Pectolinarin
is an important flavonoid found to be present in the Cirsium japonicum.

Methods: In order to understand the beneficial health aspect of flavonoids in


neurodegenerative disorders, present work summarized the health beneficial properties of
Pectolinarin through literature data analysis of various scientific research works.
Neuroprotective effects of Pectolinarin in the management of neurodegenerative disorders
‘Alzheimer's disease’ have been presented here in this study. Different biological experiments
have been conducted in the scientific field and data were collected from different literature
database to explore the neuroprotective potential of Pectolinarin in the medicine. Role of
antioxidant in the Alzheimer's disease have been also investigated and analyzed through
literature data analysis of various research work and presented in this investigation.
Importance of AChE and BuChE in the neurodegenerative disorders have been also
investigated through literature data analysis of different research work.

Results: Literature data analysis of various scientific research works revealed the beneficial
effects of pectolinarin in the medicine for the treatment of neurodegenerative disorders.
Scientific data analysis of various literatures revealed the biological importance of pectolinarin
against oxidative stress and cell death induced by H2O2. Literature data analysis of research
work revealed the antidepressant activity of pectolinarin in various animal models. Molecular
study data analysis revealed the biological importance of AChE and BuChE in the treatment of
neurodegenerative disorders and highlighted the key role of pectolinarin in the medicine for the
development of newer medicine against various forms of neurodegenerative disorders.

Conclusion: Literature data analysis of different research works revealed the biological
potential of pectolinarin in neurodegenerative disorders which could be beneficial for the
treatment of Alzheimer's disease in the future.

150
EP018. A SYSTEMATIC REVIEW ON THE ROLE OF TUMOR NECROSIS
FACTOR-ALPHA IN POST-TRAUMATIC EPILEPSY

Alina Arulsamy*1; Mohd. Farooq Shaikh1


1Jeffrey Cheah School of Medicine and Health Sciences, Monash University Malaysia,

Malaysia
Email Address: alina.arulsamy@monash.edu

Introduction: Post-traumatic epilepsy (PTE) is one of the detrimental outcomes of traumatic


brain injury (TBI), resulting in unprovoked recurrent seizures which impacts heavily on the
quantity of life. However, the pathological relationship between PTE and TBI remains unclear,
and commonly prescribed anti-epileptic drugs (AED) are ineffective against PTE. Fortunately,
emerging research implicates neuroinflammation particularly the tumor necrosis factor-alpha
(TNF-α) as the key mediator for PTE development. Thus, this systematic review aims to
examine the currently available literature regarding the role of TNF-α in PTE pathology and
consecutively, evaluate TNF-α as a possible target for PTE treatment.

Methods: A comprehensive literature search was conducted on four databases including


PubMed, CINAHL, Embase, and Scopus. Articles were screened according to the inclusion
and exclusion criteria as per the PRISMA guidelines. Articles with relevance in investigating
TNF-α expression in PTE were considered in this review. Qualitative analysis on the selected
animal studies were performed using SYRCLE tool.

Results: Critical evaluation of four articles; one in-vitro study, two in-vivo studies and one
computational study, that met the inclusion criteria suggests a proportional relationship
between TNF-α expression and seizure susceptibility post injury, and that neutralization or
suppression of TNF-α release results in reduced susceptibility to seizures after TBI.

Conclusion: In conclusion, this review elucidates the importance of TNF-α expression in


epileptogenesis post-injury and urges future research to focus more on clinical studies
involving TNF-α which may provide a clearer insight to PTE prevention and therefore
improving the lives of PTE patients.

151
EP019. SUDDEN UNEXPECTED DEATH IN EPILEPSY AMONG ASIAN: A
SCOPING REVIEW

Hana Maizuliana Solehan*1; Ahmad Faris Mohd Yusof1; Abdul Aziz Marwan1; Mohd
Dzulkhairi Mohd Rani1; Nurul Azmawati Mohamed1
1Medical Department, Universiti Sains Islam Malaysia, Malaysia

Email Address: hmsdcc678@gmail.com

Introduction: Around 23 million people in Asia suffer from epilepsy and the prevalence among
Asian countries varies from 1.5 to 14 per 1000 populations. Sudden unexpected death in
epilepsy (SUDEP) is defined as deaths in epilepsy patients that are not caused by injury,
drowning, or other known causes. Four percent of epilepsy patients die due to SUDEP. The
mechanism is poorly understood and commonly underreported. The objective of this scoping
review is to identify existing literature in SUDEP among Asian populations.

Methods: We conduct this scoping review by using Arksey and O'Malley's methodology
framework. Search engines such as PubMed, Ovid and EBSCOhost were used to screen the
citations. The following keywords: 'death' OR 'mortality' AND 'epilepsy' OR 'seizure' OR
'convulsion' AND ‘Asia’ or ‘Asian’ OR all Asian countries. The inclusion criteria include articles
published in English language, cohort of Asian population and available full-text articles.
Animal studies, review articles and confirmed cause of death due to other medical conditions
were excluded.

Results: Initial search retrieved 6909 citations. Only 25 articles fulfilled the inclusion and
exclusion criteria. Majority of the studies are from China (10). The rest are from Turkey (4),
Japan (3), Taiwan (2), India (2), Malaysia (1), Indonesia (1), South Korea (1) and Iran (1). The
articles were classified post-hoc. The areas of topics are the study of SUDEP’s risk factors
(17), prevalence (6) and psychoeducation (2). There are ten cohort studies, seven case
reports or case series, six case-controlled studies and only four autopsy reports.

Conclusion: SUDEP knowledge is scarce in Asia. Perhaps more research is needed to


identify its true prevalence. Exploring understanding of SUDEP in potential niches such as
psychosocial and education is likely to improve awareness among healthcare practitioners and
patients alike.

152
EP020. MORTALITY IN ADULT EPILEPSY PATIENT IN MALAYSIA:
HOSPITAL-BASED STUDY

Si Bao Khor*1; Kheng-Seang Lim1; Si-Lei Fong1; Chong-Tin Tan1; May Yi Koh1; Jun-Hui Ho1
1Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya,

Kuala Lumpur, Malaysia


Email Address: sibaokhor@gmail.com

Introduction: The reported mortality rate of epilepsy in Asia ranged from 2.5-5.1, but there
was no published data in Malaysia. This study aimed to find out the mortality rate of patient
with epilepsy in University Malaya Medical Centre (UMMC), a tertiary hospital in Malaysia.

Methods: There were 2389 patients with epilepsy recruited retrospectively from 2009-2018 in
this study. Fatal cases were identified from National Registry Department Malaysia. Age-
specific and gender-specific Standardized Mortality Ratio (SMR) were calculated.

Results: There were 175 deaths (7.33%). The all-cause overall SMR is 1.6 (CI 95% 1.4-1.8).
The SMR in the younger age groups (15- 19, 20-29, 30-39 and 40-49) were higher than the
older age groups. Overall SMR for male patients (1.8, CI 95% 1.5-2.2) was higher than female
(1.3, CI 95% 1.0-1.6). However, in specific age groups (15-19, 20-29 and 30-39), the SMR in
female was higher.

Conclusion: The mortality rate of epilepsy in Malaysia is higher than the general population in
Malaysia but lower than other Asian countries. Specifically, the rate is higher among the males
and younger age group.

153
EP021. IMPACT OF COVID-19 ON PEOPLE WITH EPILEPSY:
UNDERSTANDING THEIR NEEDS

May Yi Koh*1; Kheng-Seang Lim1; Si-Lei Fong1; Si Bao Khor1; Chong-Tin Tan1
Department of Medicine, University Malaya, Malaysia
Email Address: kohmayyi@gmail.com

Introduction: The Coronavirus disease 2019 (COVID-19) pandemic with the implementation
of the movement control order (MCO) resulted in unprecedented loss of access to healthcare
in Malaysia. This study aimed to identify the impact of COVID-19 on people with epilepsy
(PWE) in terms of their clinical, logistics and psychological aspects.

Methods: This is a cross-sectional anonymized web-based study on PWE in University


Malaya Medical Centre (UMMC) and Malaysia Epilepsy Society. An online questionnaire was
used to assess the direct and indirect impact of COVID-19 to the epilepsy patients, including
Hospital Anxiety Depression Scale (HADS) and Quality of Life in Epilepsy Inventory (QOLIE-
31).

Results: 461 patients were included in this study, with a mean age of 39.21±15.88 years,
majority female (50.1%), with focal epilepsy (54.0%), and experienced seizures at least once
yearly (62.5%). There were 11.1% reported increased seizure frequency during COVID period.
The associated factors of increased seizure frequency during COVID period included focal
seizures, baseline seizures frequency >1 per month, unemployed status, lower education, and
precipitating factors such as inadequate sleep and stress. There were 30.2% worried of
seizure worsening due to difficulty in re-scheduling clinic appointments, 28.9% were afraid of
going to the emergency unit, 11.5% had self-adjusted medication dosages to avoid running out
of supply. A significant number experienced anxiety (27.6%) and depression (18.8%), which
were correlated with poorer quality of life (p=0.01).

Conclusion: This study highlights the clinical and psychological impacts of the COVID-19 on
PWE in Malaysia.

154
EP022. CLINICAL DIAGNOSIS, EEG EVALUATION AND TREATMENT FOR
NMDAR AUTOIMMUNE ENCEPHALITIS

Neimy Novitasari*1; Subianto H2; Susilo I.R2; Fahmi A2


1Neurology, Haji Hospital, National Hospital, Indonesia, 2Neurosurgery, Airlangga University,

Indonesia
Email Address: neimynovitasari@gmail.com

Introduction: NMDAR autoimmune encephalitis is a very rare disease. Autoimmune


encephalitis (AE) is an increasingly recognized etiology of acute neuropsychiatric decline in
adults and children. The clinical manifestations of AE are broad and include seizures,
movement disorders, behavior and mood changes, psychosis, memory/ cognitive impairment,
autonomic dysfunction, and altered level of consciousness. We described one case of NMDAR
autoimmune encephalitis. Diagnosis was made base on clinical sign, serial
electroencephalogram (EEG) patterns and NMDAR CSF evaluation. The clinical course of the
disease, specific treatment and outcome were follow up until 3 months.

Results: An 11-year-old female presented with focal seizure, psychiatric features, behavioral
changes followed by autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction
and gradual altered consciousness. Serial EEG examination between August until November
2020 showed progressive changes. Initial EEG was normal (August 2020). Second EEG
showed continuous slow activity 5 - 6 Hz generally and excessive beta activity (September 21,
2020). Last EEG (September 26, 2020) before treatment showed excessive beta activity range
14–20 Hz, Extreme Delta Brush (EDB) and Generalized Rhythmic Delta Activity (GRDA) 2 - 3
Hz. Based on clinical manifestation and EEG examination we start immunotherapy treatment
with IVIG and Methylprednisolone IV to avoid fatal complication of the disease. Evaluation of
anti-NMDAR from cerebrospinal fluid tapping was done simultaneously. Patient showed
significant clinical improvement and Modified Ranking Scale improved after immunotherapy.

Conclusion: Early identification of NMDAR autoimmune encephalitis manifestation is very


important. Combination of symptom consist of psychiatric manifestations (behavioral changes),
clinical symptom (autonomic dysfunction, orofacialbrachial dyskinesias, speech reduction and
altered consciousness), should be followed by serial EEG evaluation. Immediate
immunotherapy treatment can be started based on clinical diagnosis to avoid fatal
complication.

155
EP023. ICTAL VIDEO ELECTROENCEPHALOGRAM OF AN AMNESIC
ATTACK ON WAKING IN A TRANSIENT EPILEPTIC AMNESIA PATIENT:
EVIDENCE FOR POSTICTAL RATHER THAN ICTAL AMNESIA

Taira Uehara*1; Naoki Akamatsu1; Chikara Yamashita1; Koki Suezumi1; Akihiko Taira1;
Hiroyuki Murai1
1Neurology, International University of Health and Welfare Narita Hospital, Japan

Email Address: tuehara@iuhw.ac.jp

Introduction: There have been few reports on the ictal video electroencephalogram (VEEG) of
amnesic attacks in patients with transient epileptic amnesia (TEA). TEA attacks often occur on
waking, but the reason remains unclear.

Results: A 65-year-old man had a three-month history of recurrent episodes of antero- and
retrograde amnesia. The episodes lasted for 10-20 minutes and occurred 2-3 times per month.
Brief episodes of behavioral arrest were also witnessed. TEA was suspected, but routine EEG
was equivocal. Therefore, we performed a long-term VEEG monitoring. On the second night of
the monitoring, a focal onset seizure with subtle motor manifestation occurred during N2 sleep.
Following sudden onset tachycardia, EEG seizure patterns arose from the right temporal
regions, spread to the left, and terminated in about 90 seconds. About 20 seconds after the
seizure cessation, he awoke while EEG was almost normal. On awakening, he showed
transient amnesia lasting around 30 minutes, which was confirmed by bystanders. Both
antero- and retrograde amnesia were observed. The diagnosis of TEA was made. Lacosamide
50 mg twice daily resolved seizures.

Conclusion: We provide the first evidence that TEA attacks on waking reflect postictal
hippocampal dysfunction due to unrecognized seizures during sleep. This finding is in line with
that the duration of TEA episodes is longer than typical temporal lobe seizures.

156
EP024. PREVALENCE AND CLINICAL CHARACTERISTICS OF PEDIATRIC
HAEMORRHAGIC STROKE COMPLICATED WITH SEIZURE IN SARDJITO
HOSPITAL, YOGYAKARTA, INDONESIA

Vega Pratiwi Putri*1; Desin Pambudi Sejahtera1; Paryono1


1Neurology, Universitas Gadjah Mada, Indonesia

Email Address: vegapratiwi90@gmail.com

Introduction: Intracerebral haemorrhage (ICH) is a condition of brain parenchymal


haemorrhage with or without intraventricular bleeding. Although rare, ICH remains the most
common cause of stroke in pediatric population. Previous study showed that prognosis of ICH
in pediatric population is better than elderly, however neurologic sequelae should be
considered, including seizure that may progress to epilepsy. Objective of this study is to
determine the incidence of seizure in pediatric patients with intracranial haemorrhage.

Methods: Retrospective cross-sectional study of children with ICH in Sardjito Hospital during 5
years period (January 2014 – December 2019).

Results: There was 94 total ICH patient complicated with seizure, 36 (38.29%) of them are
children (aged <18 years old). Approximately half of them suffered from status epilepticus,
accounted for 15 children. Mean age of children with ICH complicated by stroke was 60.58
months, while those complicated with status epilepticus were older than non-status epilepticus,
84 months and 47.8 months respectively. Mortality rate of children population (66.67%) in this
study was higher than adults (48%), which mainly as a consequence of status epilepticus
which has mortality rate of 50%. Children with status epilepticus also had prolonged length of
stay, around 19.92 days. Eleven children had hematologic disorder with thrombocytopenia,
such as ALL (6), AML (1), hemophilia (1), thalassemia (1), anemia apalastic (1), and ITP (1),
while congenital malformation was only in two children. Almost half of sample had multiple
ICH, and can be a combination of parenchymal, SAH, EDH, and SDH. Only two children
complicated with hypertension.

Conclusion: Mortality rate among pediatric ICH with seizure was high, especially when
complicated with status epilepticus. Multifocal hemorrhagic lesion was prominent in this
studied population, therefore further study is needed to evaluate this associations with seizure
symptoms.

157
EP026. CLINICAL PROFILE OF CHILDREN WITH WEST SYNDROME: A
RETROSPECTIVE CHART REVIEW

Virender Gehlawat*1; Vandana Arya1; Jaya Shankar Kaushik1; Narain Das Vaswani1
1Pediatrics, Pt. BD Sharma pgims, Rohtak, India

Email Address: gehlawatv@gmail.com

Introduction: This study was intended to document the clinical profile and treatment outcome
of West syndrome in children attending a tertiary care center in Northern India.

Methods: Data were collected by a retrospective chart review of children diagnosed with West
syndrome between January 2017 to January 2018. Information was recorded pertaining to the
age at onset and presentation, etiology, and associated co-morbidities; results of
electroencephalography (EEG) and neuroimaging; treatment given; and final outcome. The
following drugs were used for treatment: ACTH (n=7), prednisolone (n=17), vigabatrin (n=25),
sodium valproate (n=28), clonazepam (n=30), and levetiracetam (n=13) and modified Atkins
diet (n=7). The response was categorized as spasm cessation, partial improvement (>50%
improvement), or no improvement.

Results: Records of 30 children (21 boys) were analyzed. The mean (SD) age at onset was 4
(3, 6.5) months. The median lag time to treatment was 5 (2,14) months. Eight (26%) were
premature, 2 (7%) were SGA, birth asphyxia in 56%, neonatal encephalopathy in 62%. EEG
findings were compatible with hypsarrhythmia in 13 (43.3%) children and 9 (30%) had modified
hypsarrythmia. MRI finding was periventricular leucomalacia (54.1%), cystic encephalomalacia
(13.8%), normal MRI (20.7%) and 1 had arrested hydrocephalus. There was no improvement
with valproate (93%), clonazepam (89%) and levetiracetam (78%). Cessation of spasm was
achieved with vigabatrin (28 %), prednisolone (38.2%) and ACTH (42.8%). Hypsarrhythmia
resolved with presence of background and other epileptiform abnormalities in 34 children.

Conclusion: The present research highlights limited role of sodium valporate, levetiracetam
and clonazepam in initial management of children with West syndrome. However, further
studies with larger sample size are required before concluding its limited role.

158
EP027. ELDERLY STROKE PRESENTING AS EPILEPSIA PARTIALIS
CONTINUA

Dr Rajarshi Chakraborty*1
1Neurology, King George Medical University, India

Email Address: satyalung@gmail.com

Introduction: Stroke is one of the most common neurological event in elderly age group.
Stroke usually leads to neurological deficits like weakness, paresthesia, speech defect, altered
sensorium, etc. Seizures per se are a less common clinical manifestation of stroke, especially
ischaemic stroke. Epilepsia partialis continua is an uncommon variety of epilepsy and is far
less common in strokes.

Results: This 70-year-old man, known hypertensive for last 20 years, presented with sudden
onset abnormal involuntary movement of left upper limb. His vitals showed a blood pressure of
200/130 mmHg and pulse rate of 75/min, regular. Neurological evaluation revealed epilepsia
partialis continua involving left upper limb without any other neurological deficit. He was
thoroughly investigated and his brain MRI scan revealed infarct. He was treated with aspirin,
atorvastatin, oxcarbazepine, lacosamide, clobazam and levetiracetam. He improved drastically
on this medication and was discharged. He is doing well on follow-up.

Conclusion: New onset epilepsia partialis continua in elderly should be considered for
vascular origin. Diseases of vascular aetiology can have favourable prognosis than congenital
or degenerative lesions especially in epilepsies.

159
EP028. LOW-DOSE PERAMPANEL IMPROVES REFRACTORY CORTICAL
MYOCLONUS BY THE DISPERSED AND SUPPRESSED PAROXYSMAL
DEPOLARIZATION SHIFTS IN THE SENSORIMOTOR CORTEX

Kazuki Oi1, Shuichiro Neshige1, 2, Takefumi Hitomi1, 3, Katsuya Kobayashi1, Maya Tojima1,
Masao Matsuhashi4, Akihiro Shimotake4, Daiki Fujii1, 5, Riki Matsumoto1, 6, Shuhei Kasama7,
Masutaro Kanda8, Yoshiaki Wada9, Hirofumi Maruyama2, Ryosuke Takahashi1, Akio Ikeda4
1Department of Neurology, Kyoto University Graduate School of Medicine, 2Department of

Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical


and Health Sciences, 3Department of Laboratory Medicine, Kyoto University Graduate School
of Medicine, 4Department of Epilepsy, Movement Disorders and
Physiology, Kyoto University Graduate School of Medicine, 5Department of Neurology,
Kurashiki Central Hospital, 6Department of Neurology, Kobe University Graduate School of
Medicine, 7Department of Neurology, Hyogo College of Medicine, 8Department of Neurology,
Takeda General Hospital, 9Department of Rehabilitation, Nissan Tamagawa Hospital
Email Address: kazuki77@kuhp.kyoto-u.ac.jp

Introduction: Although low-dose perampanel (PER) is reportedly effective on cortical


myoclonus in a limited number of patients, its underlying mechanisms remain unclear. We
aimed to extract and elaborate the clinical effects of PER on refractory cortical myoclonus for
dose, etiology and also somatosensory-evoked potential (SEP) findings.

Method: We examined 18 epilepsy patients with seizure and cortical myoclonus (10
males; mean age, 48.4 ± 16.2 years; Unverricht-Lundborg disease = 7, benign adult familial
myoclonus epilepsy = 6, dentatorubral-pallidoluysian atrophy = 2, Gaucher disease = 1, and
Lance-Adams syndrome = 2). Based on data accumulated before and after PER treatment,
correlations among clinical scores (in myoclonus and activities of daily life [ADL]), short
latency, cortical components of SEP, and PER blood concentration were analyzed.

Results: PER (mean dose: 3.2 ± 2.1 mg/day) significantly improved seizure frequency,
myoclonus and ADL scores. It also significantly decreased the amplitude of and prolonged
latency of mostly giant SEP components. The overall changes in SEP amplitude did not
correlate with clinical scores among all patients. In contrast, the degree of P25 prolongation
(23.8 ± 1.6 to 24.7 ± 1.7 ms) significantly correlated with improved ADL score (p = 0.019) and
blood PER concentration (p = 0.011). The degree of N33 prolongation (32.1 ± 4.0 to 33.7 ± 3.4
ms) was positively correlated with ADL score and blood PER concentration (p = 0.025 and p =
0.025, respectively).

160
Conclusion: Low-dose PER markedly improved myoclonus and ADL in patients with
refractory cortical myoclonus. SEP, especially P25 latency, is a potentially good biomarker to
assess the objective effects of PER on intractable cortical myoclonus.
(https://doi.org/10.1016/j.clinph.2019.07.006)

161
Transcranial Stimulation (TCS)

TS001. MOTOR EVOKE POTENTIAL ASSESSMENT IN POST STROKE


REHABILITATION WITH ADD ON ACUPUNCTURE OR MALAY MASSAGE
THERAPY USING TRANS-MAGNETIC STIMULATION (TMS) ON CORTICAL
REGION OF BRAIN

Chew Beng Hoong*1 ; Ong Beng Hooi22 ; Tan Hon Yin3 ; Fadzila bt Ismail4 ; Datuk Dr.
Muhammad Radzi Abu Hassan5
1Pharmacy, Hospital Sultanah Bahiyah, Malaysia, 2Neurology, Kedah Medical Centre,

Malaysia, 3Rehabilitation, Hospital Sultanah Bahiyah, Malaysia, 4Physiotherapy, Hospital


Sultanah Bahiyah, Malaysia, 5Clinical Research Centre, Hospital Sultanah Bahiyah, Malaysia
Email Address: phicology@gmail.com

Introduction: We did the first TMS-Motor Evoke Potential (MEP) in assessing this group of
patients in Malaysia. This is the initial step looking into new method of assessment in clinical
recovery of stroke patient.

Results: 7 subjects completed the assessment where 2 of them (Group 1) managed by


rehabilitation programme only and the others (Group 2) with add on acupuncture or Malay
massage therapy to post-stroke rehabilitation programme. Besides MEP, each subject was
assessed with other 4 outcomes: NIHSS, Modified Barthel Index (MBI), Berg Balance Score
(BBS), and Quality of Life-EQ5D score to define clinical recovery of stroke. We have 1 patient
in Group 2 showed the emergence of MEP after interventional therapy. Neurological outcome
of Group 1 having NIHSS score ranging from 10-20 before discharged and improved to
ranging 5-6 after treatment. Group 2 having NIHSS score of ranging 3-11 before discharged
and improved to range 0-7. Functional outcome of Group 1 having MBI score of ranging 51-56
improved to MBI ranging 87-91 after treatment. Group 2 showed MBI ranged between 32-52
and improved to range of 91-95 after interventional therapy. We also have BBS score ranging
1-18 before therapy to 29-50 after therapy. In term of quality of life, both groups showed
improvement of EQ5D scores of ranging 0.024-0.539 before discharge to scores ranging
0.751-1.000 after treatment.

Conclusion: MEP predicted clinical recovery potentially favorable to intervention group where
we should explore further for the statistical significance of this assessment.

162
TS002. NON-SPECIFIC FACILITATORY AFTER-EFFECTS OF
TRANSCRANIAL RANDOM NOISE STIMULATION ON BILATERAL
ANTERIOR TEMPORAL CORTEX

Chou Ching Lin*1 ; Chih-Hsu Huang1


1Neurology, National Cheng Kung University, Taiwan

Email Address: cxl45@mail.ncku.edu.tw

Introduction: Previous studies demonstrated that transcranial random noise stimulation


(tRNS), as a noninvasive electrical stimulation technique, significantly increases task
performances or cortical excitability as it is directly applied to modulate those task-related brain
areas. Here, we aimed to investigate whether the facilitatory effect induced by the tRNS on
specific task performances could stem from the stimulations of non-task-related brain areas.

Methods: Three healthy subject groups were involved in this study, one received a 15-min
tRNS at an intensity of 1 mA over bilateral anterior temporal cortex, one at 2 mA, and the third
one was the sham-control group. We used EEG under both the resting and active (watching a
video) states, as well as resting-state functional MRI to measure changes on neuronal activity
and functional connectivity after tRNS stimulation. Behavioral effects were tested by using
cortical silent period (CSP) for examining cortical excitability and Corsi block tapping (CBT)
tests for assessing visuo-spatial working memory.

Results: We found that, as compared to the groups of 1 mA and the sham-control, 2-mA tRNS
significantly lengthened the CSP and increased CBT performance. It also tended to increase
global brain rate (mean frequencies of EEG oscillations), indicating that the vigilance of
subjects might be lifted or sustained by tRNS. We also found a significant transient increase in
the strength of resting-state functional connections globally between many different brain
areas, especially for connections between temporal and parietal cortices, after tRNS.

Conclusion: Based on our findings, it is concluded that tRNS over non-task-related brain
areas may enhance certain task performances or influence cortical excitability through the
increase in vigilance caused by the global enhancement on relevant functional connectivity.

163
TS003. TREATMENT OF STUPOROUS CATATONIA WITH REPETITIVE
TRANSCRANIAL MAGNETIC STIMULATION (RTMS) THERAPY IN A
FILIPINO ADULT PATIENT: A CASE REPORT

Ferron F. Ocampo1
1Institute for Neurosciences, St Luke’s Medical Center, Philippines

Email Address: ferron.ocampo@gmail.com

Introduction: Catatonia is a syndrome of altered motor behavior and can occur in patients
with underlying psychiatric and general medical disorders. Benzodiazepines are considered as
the first line treatment for this condition while electroconvulsive therapy is performed in
refractory cases that did not respond to pharmacologic therapy. Repetitive transcranial
magnetic stimulation (rTMS) is a non-invasive technique for brain stimulation and is being used
as an alternative treatment for psychiatric conditions.

Results: Our patient is a 30-year-old female admitted to our hospital due to a two-day history
of mutism, increased sleeping time, restlessness, and behavioral changes. Laboratory tests,
cranial magnetic resonance imaging with contrast and electroencephalogram were normal. A
lumbar puncture was performed and cerebrospinal fluid analysis was negative for infectious
agents. The patient was referred to psychiatry service and was assessed as a case of brief
psychotic disorder presenting as stuporous catatonia. She was administered with intravenous
diazepam, oral olanzapine, and vortioxetine. Despite pharmacologic therapy, the patient did
not show significant improvement of symptoms. Therapy with rTMS was started on the tenth
day of admission. After completion of 10 sessions, the patient was able to ambulate without
assistance, with marked improvement of speech and motor strength. On follow up, there was
complete disappearance of catatonic symptoms.

Conclusion: Our case highlights repetitive transcranial magnetic stimulation as a potential


alternative treatment option for patients with stuporous catatonia who did not respond to
pharmacologic intervention or when electroconvulsive therapy is not available.

164
TS004. THE RELATIONSHIP BETWEEN SCALP TO CORTEX DISTANCE
AND MOTOR THRESHOLD USING TRANSCRANIAL MAGNETIC
STIMULATION (TMS)

Yetty Hambarsari*1 ;
1Neurology Department, Sebelas Maret University, Indonesia.

Email Address: dr.yetty.hambarsari@gmail.com

Introduction: This study aims to determine if the scalp to cortex or coil to cortex distance has
a correlation and can be considered as one of the variables in determining the Motor
Threshold level in TMS.

Methods: This study used international analytical-quantitative design, with 17 men and 15
women participants. The Motor Threshold was determined using the stimulus and considered
successful when the value of 50% obtained from the standard value of the experiment. Scalp
to cortex or coil to cortex distance was determined using the vector (R) of 3-dimensional
pattern that corresponds to the X-axis, Y-axis and Z-axis.

Results: The mean average of the right Motor Threshold is 48.6%, while the left Motor
Threshold is 45.56%. The mean average of the right scalp to cortex distance was 75.91 mm,
and the left scalp to the cortex was 76.10 mm. This study used Spearman's Rho analysis to
determine the correlation of scalp to the cortex to the Motor Threshold. The p-value of the right
Motor Threshold to the right scalp-cortex distance was p = 0.410. Meanwhile, the p-value of
the left Motor Threshold to the left scalp-cortex was p = 0.128. In both results, the p value of >
0.05 was obtained.

Conclusion: There is no correlation of scalp to cortex distance with the Motor Threshold.

165
TS005. POTENTIAL OF TRANSCRANIAL DIRECT CURRENT STIMULATION
IN MODULATING SPINAL REFLEX EXCITABILITY IN PERSONS WITH
CHRONIC STROKE

Fariba Yadolahi*1; Masoud Mehrpour2


1Rehabilitation, Shahid beheshti University of Medical sciences, Iran, 2Neurology, Iran

University of Medical Sciences, Iran


Email Address: fariba.yadolahi@sbmu.ac.ir

Introduction: Stroke is the main cause of disability in adults living in developing countries.
Spasticity is a primary impairment of stroke with an effect on the activities of daily life. Neural
changes of the paretic limb often occur following spasticity. The aim of this study was to
investigate the effect of Transcranial Direct Current Stimulation on neural properties of the
spastic muscle in patients with chronic stroke.

Methods: In this study double-blinded trial 22 participants with >6 months after stroke were
randomized into two groups: active anodal or sham tDCS. The subjects in the experimental
group participated in active tDCS and balance training program of 5 sessions. Evaluation
performed at baseline and at the end of the intervention. Hmax/Mmax ratio and H-reflex
latency were assumed. Clinical assessment was conducted using the Modified Ashworth Scale
(MAS), Timed Up and Go (TUG), as well as the Timed 10-Meter Walk Test (10MWT).
Repeated measure analysis of variance was used in the analysis. Friedman test in SPSS
version 22.0 was used to analysis the response of ordinal variable in different stages of
evaluation.

Results: The active tDCS group showed significant differences in the Hmax/Mmax (P<0.005),
10MWT (P<0.01) Scale from sham subjects. Also, the latency of the H response from the
stimulation was increased for the experimental patients compared to sham. Time by group
interaction in the 10MWT, Hmax/Mmax ratio and H-reflex latency was significant.
Nevertheless, there were no significant differences in the TUG (P=0.30) scores. The results
indicated that the H-reflex latency, Hmax/Mmax ratio were altered after a-tDCS intervention
which is correlated with improvement in walking outcome.

Conclusion: The findings of the study illustrate that tDCS treatment with balance training can
improve reflexes, and walking capacity in chronic stroke and can be considered as a promising
therapeutic tool in rehabilitation setting.

166
Nerve Conduction Studies (NCS) and Electromyography
(EMG)
NE002. THE DIFFERENCES OF VITAMIN D SUPPLEMENTATION IN NERVE
CONDUCTION STUDIES BASED ON DIABETIC DURATION AND GLYCEMIC
CONTROL IN DIABETIC NEUROPATHY

Aida Fitri*1; Hasan Sjahrir1; Adang Bachtiar2; Muhammad Ichwan3


1Neurology, Universitas Sumatera Utara, Indonesia, 2Public Health, University of Indonesia,

Indonesia, 3Pharmacology & Therapeutic, Universitas Sumatera Utara, Indonesia.


Email Address: aida.fithrie@gmail.com

Introduction: Diabetic neuropathy (DN) is the most common form of neuropathy in developed
countries. Diabetic duration and glycemic control are risk factors for DN. Evidence that vitamin
D can improve glycemic control is still uncertain. In some literatures, there are conflicting
reports on the effect of vitamin D on nerve conduction studies (NCSs). Aim: To assess the
differences of vitamin D supplementation in NCSs based on diabetic duration and glycemic
control in type 2 diabetes mellitus (T2DM) Indonesian patients.

Methods: Twenty-five subjects with T2DM in Haji Adam Malik General Hospital Medan were
studied. They were treated for 10 weeks with vitamin D (D3) supplementation of 50,000
IU/week. They were evaluated by HbA1c level and routine NCSs in the upper and lower limbs,
before and 10 weeks after vitamin D supplementation. The differences of vitamin D
supplementation in NCSs were based on diabetic duration and glycemic control. This was
analyzed with dependent t-test.

Results: There were significant differences of vitamin D supplementation in distal latency (DL)
of all nerves, and amplitude (A) of motor median, ulnar, peroneal, and tibial nerves and
sensory sural nerve based on diabetic duration. There were significant differences in A of
motor and sensory median and ulnar nerves and motor peroneal and tibial nerves, and nerve
conduction velocity (NCV) of all nerves.

Conclusion: There were differences in vitamin D supplementation in NCSs based on diabetic


duration and glycemic control in T2DM Indonesian patients.

167
NE003. FREQUENCY OF CARPAL TUNNEL SYNDROME IN PATIENTS
HAVING DIABETES MELLITUS WITH NEUROPATHY IN A TERTIARY CARE
HOSPITAL

Md. Rashedul Islam*1 ; Dr. Tanbin Rahman2 ; Dr. Rafi Nazrul Islam3 ; Dr. Rumana Habib1 ;
Dr. Aminur Rahman1 ; Prof. Dr. Nirmalendu Bikash Bhowmik1 ; Prof. Dr. Md. Amirul Haque1
1Neurology, Birdem General Hospital, Bangladesh, 2Internal Medicine, Greenlife Medical

College, Bangladesh, 3Nephrology, Birdem General Hospital, Bangladesh.


Email Address: rashed2k2001@yahoo.com

Introduction: Carpal tunnel syndrome (CTS) is one of the most frequent entrapment
neuropathies of the upper limb. Carpal tunnel syndrome (CTS) and diabetic polyneuropathy
(DPN) are common conditions in patients with diabetes and therefore frequently occur
concomitantly. Diagnosis of CTS in patients with DPN is important, as therapeutic interventions
directed toward relief of CTS may be effective irrespective of diffuse neuropathy.

Methods: This study was a hospital based descriptive cross sectional study done in BIRDEM
general hospital between July, 2015 and June, 2016, for a period of twelve months. The initial
clinical diagnosis of DPN was made and was confirmed by doing nerve conduction study. After
having informed written consents, a standard preformed questionnaire was filled up for each
case.

Results: A total of 354 cases were finally analyzed with 153 (43.2%) patients being
symptomatic and among those 54 (58.7%) had electrophysiology proven CTS. It was observed
that 26.0% of patients established as having DPN also had CTS. The frequency of CTS among
those with symptoms was significantly higher than in asymptomatic participants. Majority
(38.4%) of patients belonged to the age group of 51-60 years. The mean age was found to be
55.99 ± 9.25 years with a range from 28 to 80 years. Among all cases of electrophysiology
proven CTS (92 patients), females (53.3%) numbered greater in comparison to males (46.7%).
Study subjects with CTS had significantly higher BMI, higher fasting blood sugar and higher
HbA1c in comparison to patients without CTS. The mean compound motor action potential
was not significantly different between patients with and without CTS.

Conclusion: Given the high prevalence of CTS in subjects with DPN, it is recommended that
therapeutic decisions should be made carefully after nerve conduction study and proper
diagnosis.

168
NE004. NERVE CONDUCTION STUDIES IN PATIENTS WITH NEUROPATHY
BY ELECTRICAL INJURIES

Nona Suci Rahayu*1; Syahrul, Dessy R Emril1, Endang Mutiawati Rahayuningsih1


1Department Neurology, Faculty of Medicine Syiah Kuala University,Dr. Zainoel Abidin Public

Hospital, Banda Aceh, Indonesia.


Email Address: nonasucirahayu@yahoo.com

Introduction: Electrical injury can cause impairment in peripheral nerves. Butler and Gant
reported that only a small portion of population with electrical injury showed the peripheral
nerve damage. Ulnar and median nerves are two peripheral nerves that are commonly
damaged in upper extremities. Electrophysiology test is an objective modality to examine the
condition of peripheral nerves.

Methods: We reported an assessment of nerve conduction study in a case of a 35-year-old


woman with pain radiating at her right shoulder to fingertips after an electrical injury. She also
felt a weakness in her right shoulder and arm. The motor strength on her right elbow was 4
and on her proximal humerus was 4. Sensory and autonomic functions were normal.

Results: The Nerve Conduction Study showed a distal radial motor latency of 2.8 ms on left,
and 2.0 ms on the right side. The amplitude was 6.4 mV on the left and 4.6 mV on right side.
The latency of the median nerve in the right was 8.2 ms and 7.9 ms on left side with the
amplitude of 4.2 mV on the left and 9.7 mV on right side. The latency of the ulnar nerve was
5.1 ms on the right side, 5.0 ms on the left. The NCV of the ulnar nerve was 16.1 m/s on the
right side and 82.1 on the left. The amplitude was 4.0 mV on the left and 7.6 mV on the right
side.

Conclusion: An electrical injury can cause some damage in peripheral nerves. In this report,
the distal latency in median and ulnar nerves on the right hand increased with normal
amplitude. This type of abnormality indicates a demyelination affecting the majority of the
nerve fibers more or less equally as compression neuropathy

169
NE005. CORRELATION BETWEEN NERVE CONDUCTION STUDY AND
VIBRATION PERCEPTION THRESHOLD IN DENTAL RESIDENTS

Rekha Limbu*1; Dilip Thakur1; Nirmala Limbu1; Prakash Parajuli2; Navin Agrawal3; Shivalal
Sharma4; Robin Maskey5
1Basic and Clinical Physiology, B.P. Koirala Institute of Health Science, Nepal
2Prosthodontics, B.P. Koirala Institute of Health Science, Nepal, 3Conservative & Endodontics,

B.P. Koirala Institute of Health Science, Nepal, 4Periodontology, B.P. Koirala Institute of Health
Science, Nepal, 5Internal Medicine, B.P. Koirala Institute of Health Science, Nepal.
Email Address: limrekh86@gmail.com

Introduction: Dentists are exposed to handheld vibrating tools in their daily clinical practice.
Repetitive exposure to vibration has been shown to cause peripheral nerve dysfunction. Nerve
conduction study (NCS) provides objective assessment of peripheral nerve function. Vibration
perception threshold (VPT) measures the sensorineural abnormalities in the distal fingers
associated with vibration. Most studies have been performed in symptomatic subjects.
Therefore, we aimed to study correlation between vibration perception threshold and NCS in
apparently healthy asymptomatic dental residents.

Methods: This cross-sectional study enrolled 22 apparently healthy asymptomatic dental


residents exposed to vibrating hand-held tools at least for 10 months (exposure group) and 22
age-matched medical residents who were not exposed to vibration (non-exposure group).
They were selected by purposive sampling method. VPT was measured on the palmar surface
of second and fifth fingertips of both hands. NCS of the bilateral median and ulnar nerves were
recorded by belly-tendon montage. Pearson’s correlation was applied to find out the relation
between NCV and VPT. A p-value of < 0.05 was considered significant.

Results: Positive correlations were found between right median nerve proximal latency and
VPT on left hand; minimum F-wave latency of left median nerve and VPT on left hand and
minimum F-wave latency of left ulnar nerve and VPT on left hand.

Conclusion: Significant correlations were observed between VPT and electrophysiological


parameters indicating that both methods are valuable for evaluating peripheral nerve
dysfunction.

170
NE006. ATYPICAL PRESENTATION OF GUILLAIN-BARRE SYNDROME
(GBS) IN ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY
(AIDP) WITH CONDUCTION BLOCK AND ITS ASSOCIATION WITH ANTI-
SULFATIDE ANTIBODY: A CASE REPORT

JK Tan*1; Dhayalen K1; Hazfadzila MU1; R Remli1; HJ Tan1


1Neurology Unit, UKM Medical Centre, Malaysia

Email Address: juenkiem@gmail.com

Introduction: AIDP represents a subtype of GBS, whereby demyelination impairs


transmission of impulse via altered paranodal and internodal membranes. Conduction blocks
and velocity reduction occurs as a result in which our case illustrates.

Results: A 63-year-old Chinese retired salesman with dyslipidemia presented with sudden
limb weakness and slurring of speech. He experienced fever, lethargy and flu-like symptoms
two days prior. On arrival, temperature was 38.8 degrees Celsius while other vital signs were
within normal limits. He was oriented with left lower motor seventh nerve lesion and dysarthria.
Other cranial nerve functions were intact. Both upper limbs had power of 4/5 except for a left
wrist drop while lower limbs had power of 3/5 proximally and 4/5 distally except for a right foot
drop. Reflexes were present and sensation was intact. Computed tomography of the brain with
perfusion study was normal. He was treated as lacunar stroke with NIHSS 8/42 and initiated
on antiplatelet. Nerve conduction study (NCS) done in view of patchy clinical distribution shows
evidence of generalized motor demyelinating polyneuropathy with multifocal conduction block
suggestive of AIDP variant of GBS. A lumbar puncture performed revealed cytoalbuminologic
dissociation with cerebrospinal fluid (CSF) protein of 726 mg/L and absence of cells. Meningitis
panels were negative and his antiganglioside antibodies demonstrated borderline positivity of
anti-sulfatide IgG. Intravenous immunoglobulin (0.4 g/kg) was administered over 5 days. He
was discharged and regained full power of both upper and lower limbs. Ensuing NCS at three
weeks revealed minimal improvement with return of F-waves recording.

Conclusion: GBS presents in myriad of ways underlining the importance of


electrophysiological studies. Our patient presented with debilitating symptoms but regained full
function after intravenous immunoglobulin. Sensory impairment of varying degree has been
observed in raised titres of anti-sulfatide antibodies. Further studies to define phenotype of
GBS with anti-sulfatide antibodies are recommended.

171
NE008. ASSOCIATION BETWEEN EXCITABILITY OF THE SPINAL MOTOR
NEURON FUNCTION AND AN EVALUATION INDEX FOR IMAGE RECALL
ABILITY

Hiroyuki Matsubara*1 ; Masataka Kurobe1 ; Toshiaki Suzuki2


1Department of Rehabilitation, Tanabe Central Hospital, Japan, 2Graduate School of Health

Sciences, Graduate School of Kansai University of Health Sciences, Japan.


Email Address: matsubara00123@gmail.com

Introduction: We examined the association between the image recall ability and excitability of
the spinal motor neuron function during motor imagery of the Box and Block Test (BBT).

Methods:14 right-handed healthy subjects participated in this study. The image recall ability
was evaluated by the Vividness of Movement Imagery Questionnaire (VMIQ) and Mental
Chronometry (MC) using the BBT. We investigated the spinal motor neuron function during
motor imagery of the BBT. The motor imagery task was performed with the right index finger
and thumb at the fastest possible speed in the BBT. Subjects were then asked to perform the
BBT at the same speed in motor imagery. We evaluated the correspondence between the
number of blocks moved with motor imagery and the actual physical movement. The
excitability of the spinal motor neuron function was examined using the F-wave. We measured
the F-wave of the right thenar muscles after stimulating the right median nerve at the wrist. The
F-wave was recorded at rest and during motor imagery of BBT. This study examined the
correlation between the VMIQ and MC. In the statistical analysis, the Wilcoxon signed-rank
test was used to compare the F/M amplitude ratio at rest and during motor imagery of the BBT.
Significance was set below the 5% level.

Results: The VMIQ and MC results were similar for the image recall ability. The F/M amplitude
ratio significantly increased during motor imagery of the BBT compared to that at rest in the
subjects with a low image recall ability. The BBT showed no significant difference in the F/M
amplitude ratio compared to that at rest in subjects with a high image recall ability.

Conclusion: The VMIQ and MC have a correlation and may be useful for evaluating image
recall ability.

172
NE009. EXCITABILITY OF THE ANTERIOR HORN CELLS WITH PERIODIC
MOVEMENTS AFTER DIFFERENT NUMBER OF MOVEMENTS

Masataka Kurobe*1; Hiroyuki Matsubara1; Toshiaki Suzuki2


1Department of Rehabilitation, Tanabe Central Hospital, Japan, 2Graduate School of Health

Sciences, Graduate School of Kansai University of Health Sciences, Japan.


Email Address: kurobe_m@yahoo.co.jp

Introduction: Inhibition and facilitation of the muscle tone are important to improve the motor
functions in stroke patients. Herein, we report the excitability of the anterior horn cells
depressed after periodic thumb movements in our previous study. Periodic movements may be
effective in inhibiting the muscle tone. However, the effect of the number of periodic
movements on the excitability of the anterior horn cells with periodic movements is unclear.
We investigated the excitability of the anterior horn cells with periodic movements after
different number of movements.

Methods: Twelve right-handed healthy subjects participated in this study. F-waves were
recorded from the right abductor pollicis brevis with electrical stimulation to the right median
nerve before, immediately after, and 2 and 4 min after a movement task. The movement task
was periodic right thumb abductions at 2 Hz, and the number of movements was 10 or 20. For
the statistical analysis, the Friedman’s test was used to compare the F/M amplitude ratio
before, immediately after, and 2 and 4 min after the movement task. The lower rank test was
compared using the Wilcoxon signed-rank test with Bonferroni correction. Significance was set
below the 5% level.

Results: The F/M amplitude ratio immediately after 10 periodic movements was lower than
that before the task. However, the F/M amplitude ratio immediately after 20 periodic
movements was the same as that before the task.

Conclusion: The excitability of the anterior horn cells is inhibited after 10 periodic movements
at 2 Hz.

173
NE010. SPECTRUM OF REFERRALS TO THE ENMG LABORATORY OF
BNSRC, THE APEX NEURO CENTER IN BRUNEI

Muhammad Sulaiman Bin Haji Sufardi*1 ; Dayang Nurauni Hamizah Binti Awang Mohd Ali1 ;
Dayangku Siti Noorazemah Binti Pengiran Ali1 ; Siti Nuraimi binti Saman1 ; Jonly Deocareza
Sabaricos1 ; Mathew Alexander1
1Neurology OPD, BNSRC, Brunei Darussalam

Email Address: sulaimansufardi@gmail.com

Introduction: Neuro-Electrophysiological (NEP) laboratories play an important role in precise


characterisation, helping to guide treatment strategies and prognosticating neurological
disorder. Brunei Neuroscience, Stroke Rehabilitation Centre (BNSRC) is the tertiary referral
neurology centre for Brunei, a country in the South East Asian region with a population of
350,000. The NEP lab does about 700-800 ENMG studies a year and tests were conducted
using a Dantec Keypoint. The aim of this study is to examine the spectrum of referrals to the
NEP lab of BNSRC from November 2018 to October 2019.

Methods: Retrospective descriptive study. The referrals during the study period were retrieved
from a computerised database and the records were reviewed.

Results: There were a total of 688 referrals; 260 males, 428 females with a mean age of 49.1
years. The commonest referral was Mononeuropathies, predominantly Carpal Tunnel
Syndrome at 53% (362) with Polyneuropathy at 12% (77) being the next common. Other
mononeuropathies include Bell’s Palsy, Ulnar Neuropathy, Peroneal Neuropathy and Radial
Neuropathy made up 8% (55). Guillain-Barre Syndrome and Critical Illness Neuromyopathy
made up 1.5% (10) and Myasthenia Gravis accounted for 4.6% (32) of referrals while
Inflammatory Myopathies and Muscular Dystrophies accounted for 1.9% (13). Other referrals
included conditions such as ALS, Spastic Paraparesis and Stiff Person Syndrome.

Conclusion: ENMG Tests play a crucial role as they measure functional anatomy. This study
captures the entire spectrum of disorders where the NEP laboratory helps guide the referral
doctor. There is a need for protocol based studies, to learn newer techniques and to assist the
Neurologist in EMG. The role of the NEP technologist is vital in getting technically robust
recordings, even in less conducive surroundings like ICU and vital in developing a ‘State of the
Art’ lab.

174
NE012. COMPARISON OF ORTHODROMIC AND ANTIDROMIC MEDIAN AND
ULNAR SENSORY NERVE CONDUCTION STUDIES IN NEUROPATHY

Theresia Christin*1 ; Marissa Sylvia Regina1 ; Erial Bahar1


1Neurology Department, Sriwijaya University, Indonesia

Email Address: dyan_destry@yahoo.com

Introduction: Median and ulnar nerves are the most frequent nerves involved in the upper
limb neuropathy. Sensory nerve conduction studies (NCS) are often practiced to identify the
abnormalities in neuropathy. NCS methods consist of orthodromic and antidromic. The
antidromic method produces a greater amplitude, faster conduction, yet with the potential of
motor nerves stimulation. On the other hand, the orthodromic method shows smaller
amplitude, more difficult to detect neuropathy, but do not stimulate motor nerves. Aim: This
study compares the orthodromic and antidromic sensory (NCS) examination of median and
ulnar nerve using signals in different directions.

Methods: A cross sectional study at RSUP dr. Mohammad Hoesin Palembang in the period of
July15, 2019 until September15, 2019 was conducted. There were 64 subjects with median
and ulnar nerve neuropathies included in the study after selection through inclusion and
exclusion criteria. Each sample had an orthodromic and antidromic sensory (NCS)
examination. Data analysis used was the kappa comparative hypothesis test.

Results: The results of the statistical analysis showed slight agreement with a value of k =
0.01-0.20 between the two orthodromic and antidromic methods in the latency and amplitude
of ulnar nerve and latency of median nerve. The kappa result in amplitude of median nerve
showed fair agreement with a value of kappa 0.21-0.40, and substantial agreement in velocity
with the value of kappa 0.61-0.80. Meanwhile, in ulnar nerve, the velocity showed moderate
agreement with the value of kappa 0.41-0.60.

Conclusion: Both sensory (NCS) orthodromic and antidromic methods have a good
agreement in velocity, thus both methods can be used and are able to provide interpretations
of similar abnormalities in neuropathy examination.

175
NE013. DISTAL MYASTHENIA GRAVIS - A MISSED CLINICAL DIAGNOSIS

Khushnuma Anil Mansukhani*1 ; Alika Sharma1 ; Lajita Balakrishnan1 ; Priyanka Chavan1


1Clinical Neurophysiology, Bombay Hospital, India

Email Address: kilom@hotmail.com

Introduction: What is well known: Myasthenia gravis presents with ocular, bulbar and
proximal limb weakness. What is less known: Myasthenia gravis can rarely present with
predominant distal weakness of hand muscles. Myasthenia gravis (MG) is an immune
mediated, post-synaptic neuromuscular transmission disorder, presenting with predominant
ocular, bulbar and/or proximal fluctuating weakness and fatiguability. Distal and asymmetric
onset of symptoms in MG is rare and could lead to delay in diagnosis especially when
fatiguability or fluctuation is not clearly reported as a prominent initial symptom - as seen in our
patients.

Results: We report 3 cases of distal MG who presented with predominant distal asymmetric
hand weakness. Their clinically diagnoses for referral to the electrodiagnostic (EDx) laboratory
were chronic inflammatory demyelinating polyneuropathy, C8T1 motor radiculopathy and
multifocal motor neuropathy. It was in the EDx laboratory that the diagnosis of MG was
suspected in these patients. Since the routine EDx tests were normal in all 3 patients - a
repetitive nerve stimulation test (RNST) was done - which was positive in the affected muscles
and hence a diagnosis of distal MG made (This is the protocol followed in our laboratory).
These patients were then found to have high serum levels of antibodies to acetylcholine
receptors - confirming the diagnosis of MG. RNST is not routinely done in all patients sent for
electrodiagnostic testing and none of these patients had a reference for the same – however
using a protocol based method of EDx testing, leading to the diagnosis of a treatable condition.

Conclusion: Electrodiagnosticians need to be aware of this presentation of MG and follow


protocols or else the diagnosis could be missed.

176
NE014. ELECTROMYOGRAPHY IN THE DIAGNOSIS OF STIFF-PERSON
SYNDROME

Sumanth Shivaram*1 ; Madhu Nagappa1 ; Doniparthi V Seshagiri1 ; Anita Mahadevan2 ;


Sanjib Sinha1
1Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India,
2Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India

Email Address: sumanth1983s@gmail.com

Introduction: Stiff-person syndrome (SPS) is a rare autoimmune or paraneoplastic disorder


with a wide clinical spectrum but core features of trunk and limb stiffness and muscle spasms.
It is commonly associated with glutamic acid decarboxylase (GAD 65) antibody and responds
to treatment with benzodiazepines and immunotherapy. We present the details of a patient
with SPS and highlight the diagnostic utility of electromyography (EMG).

Results: A 58-years-old man developed progressive asymmetric stiffness of both lower limbs
and walking difficulty of ten months duration. Superimposed on that, he had painful spasms of
lower limb which were triggered by sound or touch. On examination, he was found to have
asymmetric hypertonia in both lower limbs and gait difficulty requiring the assistance of two-
persons to walk. Serum GAD-65 antibody was strongly positive. He underwent
electrophysiological evaluation. Nerve conduction studies were normal. Lower limb surface
EMG with recording from quadriceps, hamstrings, tibialis anterior, and lateral head of
gastrocnemius on the right side and quadriceps and hamstrings on the left side was
performed. It showed continuous motor unit activity in agonist and antagonist muscle groups.
After the administration of diazepam, the motor unit activity reduced significantly while
stimulation with a loud sound led to an increased in activity. Based on these findings, he was
diagnosed with SPS and treated with immunotherapy and symptomatic medications. He had
significant improvement and was able to walk independently at four months of follow up.

Conclusion: SPS is a rare but treatable neurological illness that can be confused with many
other disorders like parkinsonism, dystonia, musculoskeletal, psychogenic, etc. EMG can
provide a useful clue to the diagnosis by demonstrating characteristic continuous motor unit
activity in agonist and antagonist muscles which triggered by specific stimuli and improved with
diazepam.

177
NE015. ACUTE EFFECTS OF RESPIRATORY MUSCLE STRETCHING ON
VENTILATORY PATTERNS AND RESPIRATORY MUSCLE ACTIVITY

Hiroyasu Iwatsuki*1 ; Toshiya Urushihata1 ; Toshihiro Haba1


1Physical Therapy, Aomori University of Health and Welfare, Japan

Email Address: h_iwatsuki@auhw.ac.jp

Introduction: Respiratory muscle stretching is useful in reducing chest wall rigidity,


consequently increasing chest wall expansion and improving ventilatory patterns. There are
few reports that have examined the effects of these techniques in relation to both minute
ventilation and inspiratory muscle activities. The purpose of this study was to evaluate the
acute effects of respiratory muscle stretching on ventilatory patterns and respiratory muscle
activities in healthy persons.

Methods: Ten healthy young men participated in the experiment. Subjects were placed in the
Fowler’s position. The stretching of both bilateral neck and pectoralis major muscles by a
physical therapist was performed throughout the expiratory phase, allowing inspiratory
muscles to reach their maximum length during the relaxation time. Two sets of 10 consecutive
stretches were performed for each muscle, with a 30 s interval between the series. The
ventilatory pattern and inspiratory muscle activities (both sternocleidomastoid and external
intercostal muscles) were evaluated through spirometry and electromyography before and
immediately after each intervention. Both measurements were taken while the subject
breathed at a rate of 30 times per minute. Muscle discharges were quantified using the root
mean square to analyze the changes in the contraction levels. The study was performed
according to the guidelines set by the Ethics Committee of Aomori University of Health and
Welfare. All participants signed an informed consent form.

Results: Respiratory muscle stretching promoted a significant acute increase in maximum


voluntary ventilation, mean inspiratory flow, and inspiratory muscle activities during tachypnea
compared with the control group.

Conclusion: Acute effects of respiratory muscle stretching suggested increased respiratory


muscle activities and/or improved expansion of ventilatory functions.

178
NE016. AN OVERVIEW OF NERVE CONDUCTION STUDY IN CARPAL
TUNNEL SYNDROME PATIENTS IN SANGLAH GENERAL HOSPITAL
DURING 2018-2019

Ni Made Dwita Pratiwi*1 ; I Komang Arimbawa1 ; I Gusti Ngurah Purna Putra1


1Neurology Department, Department of Neurology, Faculty of Medicine, Udayana University,

Sanglah General Hospital, Indonesia


Email Address: dwtpratiwi@gmail.com

Introduction: Carpal tunnel syndrome (CTS) is the most common nerve entrapment.
Electrodiagnostic (EDX) studies are a valid and reliable method of confirming the diagnosis.
CTS will likely continue to be a common problem encountered by the electrodiagnostician. The
purpose of this study was to compare the diagnostic accuracy of various nerve conduction
tests and to determine the properties of the most accurate test.

Methods: This was a descriptive cross sectional study conducted at the neurologic policlinic at
Sanglah Hospital from January 2018 to Desember 2019. Sixty six patient with clinical symptom
of CTS were included within the study period.

Results: From 66 patients, women were 72.7% with the largest age group (42.4%) in the
elderly, range 46-55 years. Elongation of the SNAP median nerve latency was in 74.2% of
patients. Lengthening of the CMAP median nerve latency was in 66.7% of patients. Median–
Ulnar Sensory Latency Difference to the Ring Finger ≥0.4 in 78.8% of patients.

Conclusion: Addition of a single test of median and ulnar sensory latency difference to the
ring finger in routine protocol will improve the sensitivity for the diagnosis of CTS in all patients.

179
NE017. MID-PALMAR STIMULATION AND INCHING METHOD OF THE
MEDIAN NERVE FOR DISTINGUISHING THE CARPAL TUNNEL SYNDROME
(CTS) FROM THE DIABETIC POLYNEUROPATHY (DPN)

Rie Kanki*1 ; Eiko Murase2 ; Yoshiaki Segawa1 ; Takashi Tokunaga1 ; Hisao Tachibana1 ; Jun
Kimura3
1Neurology, Nishinomiya Kyouritsu Neurosurgical Hospital, Japan, 2Neurology, National

Hospital Organization Nara Medical Center, Japan, 3Neurology, University of Iowa, United
States
Email Address: riek@kuhp.kyoto-u.ac.jp

Introduction: The electrodiagnosis of CTS continues to evolve with renewed interest centering
on the clinical use of mid-palmar stimulation and inching study. We reanalyzed our data
(Neurology 1979;29: 581 [abstract]) on distal conduction abnormalities in DPN and CTS to
discuss pros and cons of currently available nerve conduction techniques.

Methods: We stimulated the median nerve at the wrist and mid palm to record muscle
responses from the abductor pollicis brevis and sensory potentials from the index finger in 40
nerves from 22 DPN patients, 38 nerves from 24 CTS patients and 50 nerves from 25 normal
controls (NL). Of these DPN patients, 24 median nerves were examined by a sensory inching
study with 1 cm incremental stimulation.

Results: The wrist-to-palm segment showed a significant (P< 0.001) increase in motor and
sensory conduction time (mean ± SD ms) in DPN (2.25±0.67 and 1.80±0.38) and CTS
(2.21±1.34 and 1.59±0.50) compared with NL (1.15±0.21 and 1.12±0.21). In contrast, the
terminal segment from the palm had significantly (P<0.001) prolonged latencies in DPN
(2.58±0.58 and 1.70±0.42) compared to either CTS (2.01±0.36 and 1.35±0.27) or NL
(1.86±0.36 and 1.34±0.31). Inching studies, known to identify an abnormal latency shift
localized to 1 cm segment in majority of CTS hands, revealed such focal slowing in only 3 of
24 (12%) DPN hands. Unlike DPN, typically resulting in a diffuse, distal slowing of both motor
and sensory conduction, CTS characteristically shows a sharply localized lesion near the distal
edge of the transverse carpal ligament. Other techniques useful in identifying a subtle
conduction abnormality may include single fiber recording, which helps document a delay
affecting only a limited number of motor axons.

Conclusion: Mid-palmar stimulation and inching method can distinguish diffuse slowing in
DPN from a localized change in CTS along the distal segment of the median nerve.

180
NE018. THE IMPORTANT TO PAY ATTENTION THE WAVEFORM PATTERN
OF THE F WAVE IN A PATIENT WITH CEREBROVASCULAR DISEASES
WHO SHOWED MARKEDLY INCREASED MUSCLE TONE OF THE THENAR
MUSCLES

Toshiaki Suzuki*1 ; Yuki Fukumoto1 ; Marina Todo2 ; Makiko Tani1 ; Sohei Yoshida1
1Graduate School of Health Sciences, Graduate School of Kansai University of Health

Sciences, Japan, 2Faculty of Health Sciences, Kansai University of Health Sciences, Japan.
Email Address: suzuki@kansai.ac.jp

Introduction: We investigated F wave in a patient with cerebrovascular diseases in which


there was no voluntary movement of the hand, especially the thumb, due to markedly
increased tone of the thenar muscles. We experienced a case in which characteristic change
in the appearance pattern of F wave and the effect of rehabilitation.

Methods: Case was a patient with right hemiplegia due to left cerebral hemorrhage (putamen)
who showed markedly increased muscle tone of thumb flexors (MAS 4) and no voluntary
movements. F-waves were recorded from the affected thenar muscles with median nerve
stimulation (0.5 Hz, 30 times) in supine position. Exercise therapy, which included stretching of
the affected thenar muscles were also carried out, and subsequent changes in the F-wave
waveform were examined. The exercise therapy was performed twice a week for 20 minutes
each session.

Results: An F-wave with an average latency of 28.6 ms appeared after the appearance of the
M-wave. However, various forms of F-wave-like waveforms were observed during the short
latency (19.9 ms). No discharge of the short latency waveform was observed without electrical
stimulation. After 3 months of exercise therapy, the short latency waveform disappeared,
muscle tone was slightly improved (MAS 3), and voluntary movements of the paralyzed thumb
was also appeared a little.

Conclusion: The short latency waveform was not identical, and therefore cannot be
considered to be axonal reflexes. As this waveform was not observed without electrical
stimulation, the possibility that the excitability of the anterior horn cells of the spinal cord may
have been increased. In addition, since exercise therapy improve muscle tone and did not
show the appearance of the short latency, it is necessary to pay attention to the waveform
pattern of F waves to evaluate spasticity in which markedly increased muscle tone in patients
with cerebrovascular diseases.

181
NE019. THE PROFILE OF NERVE CONDUCTION STUDY EXAMINATION IN
CHEMOTHERAPY INDUCED PERIPHERAL NEUROPATHY AT DR.
SARDJITO HOSPITAL

Nuardi Yusuf*1 ; Yudiyanta1 ; Desin Pambudi Sejahtera1 ; Indra Sari Kusuma Harahap1
1Neurology, Faculty of Medicine Gadjah Mada University, Indonesia

Email Address: nuardi_yusuf@yahoo.com

Introduction: Chemotherapy induced peripheral neuropathy (CIPN) is a common adverse


effect of antineoplastic agents and can have a profound impact on quality of life.
Chemotherapeutic agents can damage nervous system structures and cause a variety of
neuropathies such as sensory and/or motor, demyelinating and axonal.

Methods: This cross-sectional observational descriptive study was carried out at Dr. Sardjito
Hospital Yogyakarta from April 1, 2020 to September 30, 2020, with purposive sampling
method. The subjects were cancer patients who had chemotherapy agents. Diabetic
neuropathy and other causes of neuropathy were excluded. NCS examination included CMAP
of median and tibial nerve, and SNAP of median nerve.

Results: Fifty six subjects included in this study were 80.4% breast cancer, 5.3% colorectal
cancer, 5.3% leukemia, 3.6% non-hodgkin lymphoma, 3.6% lung cancer, and 1.8% cervical
cancer. The majority types of nerve damage were mixed type (both axonal and demyelinating
type). The majority nerves affected in this study were 50% median sensory nerve, 46.43%
tibial motor nerve, and 37.5% median motor nerve.

Conclusion: In CIPN, the abnormalities in SNAP are more frequent than CMAP. The majority
type is mixed type, both axonal and demyelinating.

182
NE020. NERVE CONDUCTION STUDIES IN COVID-19: AN INDIAN
HOSPITAL SUGGESTION

Ashish Arvind*1
1Physiology, AIIMS Gorakhpur, India

Email Address: drashisharvind@gmail.com

Introduction: COVID-19 is no longer a scientific acronym to anyone. The whole world is


aware of this pandemic which has affected every human being on the globe. Health facilities
were at the centre stage of this global pandemic. The global shutdown had forced all the
hospitals and diagnostic centres to limit their activity for COVID treatment only. When the
entire world was forced to adopt virtual modes of communications, non COVID diagnostics
could not be accomplished by virtual platform.

Methods: With the initiation of unlock guidelines, the need for nerve conduction studies came
back to normal. To fulfil the demand of nerve conduction testing for diagnosis and treatment of
neuromuscular diseases, there must be adherence to covid guidelines and precautions. In
Indian hospital scenario, during the time of economic instability due to COVID, it is
recommended to adopt newer strategies to ensure the safety of patients and the technicians
performing the test.

Results: These provisions are cost effective and easily justifiable. Adherence to these new
strategies is not a big problem but yes everyone needs to learn newer way of healthcare
delivery to remain safe and keep safe.

Conclusion: The cost of new set up or provisions is quite less than the weekly cost of PPE for
lab staff and it does not need much expertise. These provisions and modifications are in
accordance with American Association of Neuromuscular & Electrodiagnostic Medicine
guidelines. Compliance from lab staff and patients will not be a problem as it does not have the
complicated steps.

183
NE021. SPINAL CORD INJURY VERSUS BRACHIAL PLEXUS INJURY: THE
IMPORTANCE OF NERVE CONDUCTION STUDIES (NCS) IN MAJOR
TRAUMA PATIENTS

Lin Cheng*1; Prasad Venkat2


1Trauma rehabilitation medicine, Salford Royal NHS Foundation Trust, United Kingdom,
2Rehabilitation medicine, Salford Royal NHS Foundation Trust, United Kingdom

Email Address: Lf.cheng@doctors.org.uk

Introduction: High impact injuries to the neck, shoulder and upper arm can lead to both
cervical cord or brachial plexus injuries, which at times prove to be a diagnostic dilemma
should imaging be inconclusive. We present a case of a trauma patient, with indeterminate
scans. A 20 year-old motorcyclist, with no significant past medical history, presented after
colliding with a cyclist and shoulder pain was complained on scene. Trauma series imaging
(CT head, spine, thorax, abdomen and pelvis) noted intact clavicles, scapulae, sternum and
proximal humerus with no fracture identified. He had a small pneumothorax and lung
contusion. Clinically, the patient had significant pain across the whole right upper limb, power
0/5 on muscle power assessment (MRC) scale on shoulder abduction, elbow flexion and
extension, and wrist flexion and extension. The presentation was in keeping with a brachial
plexus injury.

Results: Cervical spine and brachial plexus MRI noted not only cervical cord oedema and
haemorrhage, but also noted possible preganglionic type of root avulsion injury. There was
also no gross discontinuity of the brachial plexus and was inconclusive. The right
hemidiaphragm was also elevated in chest x-ray which was suggestive of phrenic nerve injury.
Nerve conduction studies performed confirmed severe right pan-plexopathy, with all sensory
and motor response on the right upper limb attenuated or absent. The neuropathic pain was
managed with gabapentin, amitriptyline and opiates which controlled the pain. He was referred
to the plastic surgeons for considerations of nerve reconstruction surgery.

Conclusion: Although brachial plexus MRI is deemed to be the gold standard for assessing
brachial plexus injuries, its role maybe limited in cases where images are inconclusive and
proves a diagnostic dilemma to distinguish between spinal cord and brachial plexus injury.
Clinical examination together with nerve conduction studies plays a salient role in diagnosing
for appropriate management.

184
NE022. ULNAR NEUROPATHY IN THE HAND: A CLINICAL &
ELECTRODIAGNOSTIC STUDY IN 20 PATIENTS

Alika Sharma*1 ; Priyanka Chavan1 ; Lajita Balakrishnan2 ; Khushnuma A Mansukhani1


1Department of Clinical Neurophysiology (EMG & EP), Bombay Hospital & MRC, Mumbai,

India, 2Department of Clinical Neurophysiology (EMG & EP), Apollo Hospital, Navi Mumbai,
India.
Email Address: alika.sharma@gmail.com

Introduction: Ulnar neuropathy at the wrist is less frequent than at the elbow and could be
difficult to localize clinically. Electrodiagnosis can help to localize the site of the lesion along
the nerve and differentiate it from other conditions like motor neuron disease. We conducted a
retrospective study in patients with ulnar neuropathy in the hand between 2013 and 2020. The
objective was to study the electrodiagnostic features and correlate them with clinical
impression and probable aetiology. Twenty patients were diagnosed with ulnar neuropathy in
the hand- due to various causes over an 8 -year period. Two patients had bilateral symptoms.
The clinical reference for electrodiagnosis varied from multifocal motor neuropathy, anterior
horn cell lesion to “ulnar neuropathy studies to identify site of involvement”. Seven patients had
history of trauma; eleven of acute or repetitive pressure and two had a ganglion in the Guyon’s
canal.

Results: Using the classification devised by Wu et al. there were two patients with type I
(sensory -motor at wrist) one with type II ( sensory branch ) seven with type III (proximal deep
motor branch) and nine with type IV ( distal deep motor branch) involvement. One patient with
trauma had multifocal involvement of the ulnar nerve branches in the palm. History of pressure
was not always forth coming and direct questions needed to be asked. Patients with ganglion
cysts had history of insidious onset and chronic denervation on needle electromyography,
while those with history of acute pressure related weakness presented with sudden onset
symptoms and electrodiagnostic evidence of focal slowing in the motor fibres with or without
acute denervation depending on the severity and duration of the pressure. Traumatic
neuropathies showed electromyographic evidence of acute axonal loss and reinnervation
when partial.

Conclusion: Electrodiagnosis helped to localize and predict the probable cause of the ulnar
neuropathy in the hand.

185
Autonomic Dysfunction

AD001. GASTROPARESIS AS AUTONOMIC MANIFESTATION OF


MYASTHENIA GRAVIS: A RARE CASE REPORT

Putri Auliya*
Neurology, DR Cipto Mangunkusumo Hospital, Universitas Indonesia, Indonesia
Email Address: putriauliya@me.com

Introduction: Myasthenia gravis (MG) has a major clinical symptom as fluctuating voluntary
muscle weakness that are innervated from the brain stem (ocular, masticatory, facial and
lingual muscles) but autonomic symptom is considered very rare. There are several symptoms
that can be found in autonomic dysfunction of myasthenia including gastrointestinal,
orthostatic, sudomotor, vasomotor and pupillomotor disorders. These symptoms can occur
whether in myasthenic crisis or without. Moreover, patient with autonomic symptom can fall to
myasthenic crisis more easily. The presence of these symptoms indicates inadequate
treatment and resulting a poor prognosis.

Results: A 51-year-old woman have been diagnosed with MG in the last 9 years with history of
three times myasthenic crises, was complaining fluctuating abdominal bloating and had
difficulty in swallowing. In the physical examination, the abdomen was about the size of third
trimester of pregnancy, but only for about 10-15 minutes than shrunk back to normal size.
Laboratory, abdominal CT, endoscopic, and tumor markers were performed but none of the
results can explain the symptoms. Esophageal manometry study showed weak peristaltic but
with normal pattern. Meanwhile the symptoms became worse. The patient's symptoms showed
improvement after administration of plasmapheresis and adequate myasthenia therapy.

Conclusion: Although rare, autonomic dysfunctions of MG must be noted by every clinician.


Controlled voluntary muscles cannot be used as a parameter of adequate therapy if there are
autonomic symptoms. Therefore, autonomic disorder should be evaluated.

186
AD003. AUTONOMIC DYSFUNCTION IN CASPR2 ANTIBODY ASSOCIATED
NEUROLOGICAL DISEASE

Sumanth Shivaram*1 ; Madhu Nagappa1 ; Doniparthi V Seshagiri1 ; Anita Mahadevan2 ;


Yashwanth Gangadhar2 ; T. N. Satyaprabha3 ; Vijay Kumavat4 ; Rose D. Bharath5 ; Sanjib
Sinha1 ; Arun B. Taly1
1Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India,
2Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore,

India, 3Neurophysiology, National Institute of Mental Health and Neurosciences, Bangalore,


India, 4Transfusion Medicine and Hematology, National Institute of Mental Health and
Neurosciences, Bangalore, INDIA, 5Neuroimaging and Interventional Radiology, National
Institute of Mental Health and Neurosciences, Bangalore, India.
Email Address: sumanth1983s@gmail.com

Introduction: Contactin-associated protein-like 2 (CASPR2) antibody-associated neurological


disease is a rare autoimmune illness with central and peripheral manifestations. The core
clinical features are peripheral nerve hyperexcitability, autonomic dysfunction, insomnia, and
fluctuating encephalopathy. We aim to describe the autonomic manifestations in a cohort of
patients with CASPR2 antibody-associated neurological disease.

Methods: This study was a retrospective chart review that included eight patients with
CASPR2 antibody-associated neurological illness. Their medical records were reviewed and
details regarding clinical manifestations of autonomic dysfunction and cardiac autonomic
function testing (AFT) were collected. The response of autonomic manifestations to
immunotherapy was also noted on follow up.

Results: Clinically, autonomic manifestations were present in 7/8 patients. Most commonly,
cardiovascular manifestations (palpitations, postural dizziness, tachycardia, fluctuating blood
pressure, and postural hypotension) were noted in six patients while others included
gastrointestinal (diarrhea or constipation), genitourinary (urinary frequency, urinary retention),
and sudomotor (hyperhidrosis) manifestations in three patients each. One patient developed a
hypertensive emergency with posterior reversible encephalopathy syndrome. AFT was carried
out in five patients and it showed severe cardiac autonomic dysfunction in three and definite
autonomic dysfunction in two patients. All patients had a reduction in total power with
sympathetic dominance. On follow-up after treatment with immunotherapy (mean duration
19.71±17.61 months; range, 2-52 months), all patients had improvement in autonomic
symptoms and one patient had persistent tachycardia but no symptoms.

187
Conclusion: Autonomic dysfunction is a core feature of CASPR2 antibody-associated
neurological disease and is usually multi-organ in nature with varied manifestations. Cardiac
AFT shows sympathetic dominance. While the clinical response to treatment is good, cardiac
autonomic dysfunction in the acute period can lead to dangerous fluctuations in blood pressure
and result in complications.

188
Sleep
SP001. SLEEP DISORDERS AMONG FILIPINO MOTHERS: A CROSS-
SECTIONAL STUDY IN LAGUNA, PHILIPPINES

Cherry Ann Durante*1


1College of Nursing, University of the Perpetual Help Systems Laguna, Philippines

Email Address: cggarcia@up.edu.ph

Introduction: Sleep disorders can affect an individual’s general health and quality of life.
There remains a knowledge gap on the prevalence of insomnia and its causes in developing
countries. The aim of this study is to describe sleeping disorders among mothers and its
association with social media addiction, stress, and work-life balance.

Methods: The researchers conducted a cross-sectional analytical study among 400 mothers in
the province of Laguna. The Alberta Sleep Disorder Questionnaire was used to gather data on
sleep quality, while the DASS-21 Questionnaire was used to test for depression, anxiety and
stress levels.

Results: Data showed that in the sample population the prevalence of insomnia was 44%
(n=176), circadian rhythm disturbance, 17% (n=68); restless leg syndrome, 13.5% (n=54); and
sleep apnea 53.8% (n=215). Most mothers were experiencing mild to moderate anxiety (68%),
mild to severe depression (45.5%), and mild to severe stress (42%). It was also found out that
social media addicted mothers are twice more likely to suffer from insomnia (OR=2.58, CI
1.58-4.22). Furthermore, mothers who were stressed (OR=2.76, CI 1.79-4.25), anxious
(OR=2.43, CI 1.52-3.92), and depressed (OR=1.84, CI 1.21-2.81) were significantly associated
with insomnia and parasomnia. Mothers who reported to have work insecurity are twice more
likely to suffer from sleeping disorders (OR=2.22, CI 1.35-3.67).

Conclusion: The study concludes that social media addiction, work imbalance, stress, anxiety
and depression are associated with the increase prevalence of sleep disorders among Filipino
mothers. Therefore, there is a need for public health education about the connection of internet
use and sleeping disorders.

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SP002. PREVALENCE OF DAYTIME SLEEPINESS AMONG
EMERGENCY MEDICAL SERVICE AMBULANCE DRIVER IN KUALA
LUMPUR HOSPITAL.

Rosdi Zainol*1 ; Shuib Rambat2 ; Norazlinda Saad3


1Neurology Department, Hospital Kuala Lumpur, Malaysia, 2MJIIT, UTM, Malaysia, 3College of

Arts & Sciences, UUM, Malaysia


Email Address: rosdi.neuro@gmail.com

Introduction: The Emergency Medical Service (EMS) ambulance driver's prevalence of


daytime sleepiness is the most important field of study because it concerns the health and
well-being of a rescue team member, a survivor or patient and the public. Objective of the
study was to measure the self-reported level of daytime sleepiness among EMS ambulance
drivers at Kuala Lumpur Hospital, Malaysia.

Methods: Cross-section and descriptive analysis using a self-filled questionnaire for the target
population sample of 31 EMS ambulance drivers was conducted in Asia's largest public
hospital, HKL, Malaysia, using the Epworth Sleepiness Scale (ESS).

Results: Measures for the prevalence of daytime sleepiness among 31 respondents. The ESS
shows that 20 (65%) respondents have excessive daytime sleepiness while 11 (35%) were
free of daytime sleepiness. In comparison, the frequency of a near-miss crash was relatively
high (74%) and also significant (52%) slept while driving.

Conclusion: Ambulance driver at HKL working as an EMS is at risk symptoms of daytime


sleepiness. This result highlights the importance of sleepiness screening, providing adequate
instruction, support and providing the necessary facilities to prevent adverse health effects
among EMS ambulance drivers from developing.

190
SP003. IDIOPATHIC CENTRAL SLEEP APNEA WITH PERIODICITY
PRESENTING AS CHEYNE-STOKES BREATHING

Sajeesh Parameswaran*1 ; Anil Kumar T V1 ; A Marthanda Pillai1


1Neuroscience, Ananthapuri Hospitals and Research Institute, India

Email Address: psajeesh@gmail.com

Introduction: Cheyne-Stokes breathing (CSB) is described by a cyclic variation in breathing


with periods of central apneas or hypopneas alternating with periods of hyperpneas in a
gradual waxing and waning manner. We describe this rare case of idiopathic central sleep
apnea coming in a periodic fashion presenting as CSB pattern without specific causes.

Results: A 72-year-old male (body mass index: 21.9 kg/m2) with the complaints of excessive
daytime sleepiness and fatigue for about 2 years. On detailed sleep history patient denied
snoring, cataplexy, hypnogogic hallucination, sleep paralysis and sleep attack. No neurological
deficit was seen in clinical evaluation. ENT evaluation was normal. Cardiac evaluation showed
artrial fibrillation with no cardiac failure. An overnight Polysomnography (PSG) test showed
total sleep time of 443 minutes, sleep latency of 19.0 minutes, and sleep efficiency of 70.0%.
Sleep architecture for stage 1 sleep was 31.7%, stage 2 sleep was 52.4%, slow wave sleep
was 12.9%, REM sleep was 3.1%. Total arousal index was 21.8/hr. Mean SaO2 was 96.4%,
the lowest SaO2 was 82.8%, and SaO2 below 90% was 0.1%. Snoring was not observed and
periodic limb movement index was 8.8/hr. PSG also showed cyclic fluctuation in breathing with
periods of central apneas; alternating with periods of hyperpneas in a gradual waxing and
waning fashion; but cardiac evaluation and magnetic resonance imaging of the brain could not
find any cause of this sleep-disordered breathing.

Conclusion: The pathogenesis and etiology of CSA is still vague. We think that further studies
and follow up will be required to determine whether idiopathic central sleep apnea presenting
as CSB pattern is the initial indication of certain diseases. So we concluded that this case was
an idiopathic central sleep apnea with periodicity; presenting as CSB pattern.

191
SP004. THAI SCOPA AND MPDSS; TWO SCALES FOR ASSESSMENT OF
NIGHT SLEEP DISORDER COMPARE BY PSQI IN PARKINSON'S DISEASE
AT FACULTY OF MEDICINE VAJIRA HOSPITAL

Sirinapa Saneemanomai*1; Suwat Srisuwannanukorn1


1Division of Neurology, Department of Internal Medicine, Navamindradhiraj University,

Thailand
Email Address: sirinapa.s@nmu.ac.th

Introduction: The sleep disorders in Parkinson patients affect the patient’s quality of life.
Therefore, we are interested in studying the evaluation of sleep disorders in Parkinson
patients. The Thai translated versions are MPDSS test, which was adapted from PDSS, and
SCOPA-Sleep scale. We are interested in comparing both tests to see which test is more
appropriate to assess night sleep quality of Parkinson patients, by using the PSQI sleep test as
a standard of sleeping assessment.

Methods: Cross-sectional study. Parkinson patients at Faculty of Medicine Vajira Hospital


were enrolled. The study was conducted from 1 March 2019 - 31 December 2019. Volunteers
completed Thai SCOPA-NS and MPDSS sleep tests, compared with the PSQI test, in order to
obtain the sensitivity and specificity of each test.

Results: 200 volunteers met the criteria. The prevalence of SCOPA-NS test and MPDSS test
is 62.5% (95%CI: 55.4 - 69.2) and 52.5 (95%CI: 45.3 - 59.6) respectively. SCOPA-NS test has
a sensitivity of 83.1% (95%CI: 75.3-89.2), a specificity of 71.1% (95%CI: 59.5- 80.9), a positive
predictive value (PPV) of 82.4 % (95%CI: 74.6-88.6), and a negative predictive value (NPV) of
72% (95%CI: 60.4-81.8). Whereas MPDSS test has a sensitivity of 71.8% (95%CI: 63.0-79.5),
a specificity of 78.9% (95%CI: 68.1-87.5), a positive predictive value (PPV) of 84.8% (95%CI:
76.4-91.0), and a negative predictive value (NPV) of 63.2% (95%CI: 52.6-72.8).

Conclusion: Thai SCOPA-NS test has more sensitivity than MPDSS test but has less
specificity because MPDSS test has more numbers of questions which allow more accurate
screening. However, patients significantly prefer doing Thai SCOPA-NS test than MPDSS.
Both tests can be used to evaluate Parkinson patients with similar accuracy.

192
Movement Disorders
MD001. EVALUATION OF GAIT IN SPINOCEREBELLAR ATAXIA TYPE 12
(SCA-12)

Achal Kumar Srivastava*1 ; Manpreet Kaur Narang1 ; Rajeev Aggarwal1 ; Roopa Rajan1 ;
Mohd Faruq2 ; MV Padma Srivastava1 ; K Prasad1
1Neurology, All India Institute of Medical Sciences New Delhi, India, 2Functional Genomics,

CSIR Institute of Genomics and Integrative Biology, India


Email Address: achalsrivastava@hotmail.com

Introduction: SCA12 an autosomal dominant ataxia, is characterized by tremor in hands at


onset followed by gait ataxia and other cortical features during the course of illness. The aim of
the study was to identify and quantify spatiotemporal parameters of gait in a group of SCA12
patients and to compare it with healthy subjects.

Methods: Genetically confirmed SCA12 patients (n=25) with mean age of 55.7 ± 10.2 years
and age matched healthy individuals (n=25) with 55.2 ±10.1 years were studied. A walkway
system (by Teckscan, USA) was used to evaluate spatiotemporal parameters of gait in both
groups. All the participants were instructed to walk bare foot within comfortable normal speed
on sensor plates of walkway system for 30 seconds. The two sampled t test was used with the
level of significance p value < 0.05.

Results: All spatial and temporal parameter mean values were significantly different from the
control group, except for single support time and swing time. Most significant findings in spatial
and temporal parameters concerned stride length (106.8±7.4cm in control group vs 79.0±19.4
cm in patients, p=0.000), step width (7.8±1.7 cm in control group vs 13.8±2.8 cm in patients,
p=0.000), double support time (0.35± 0.6 sec in control group vs 0.61± 0.30 sec in patients,
p=0.005) and gait velocity (83.5±14.5 cm/sec in control group vs 48.86±21.6 cm/sec in
patients, p=0.000). Ten patients with normal gait had mean duration of illness 4.4±1.2 year and
mean CAG repeat number 55.7±5.4. Mean duration of illness and mean CAG repeat number
of 15 patients having abnormal gait was 6.8±5.4 years and 53.8±7.3.

Conclusion: Gait ataxia in SCA12 patients was a late feature and developed after around 5
years of illness. Gait showed significant reduction in stride length, step length, gait velocity with
mark increase in step width and double support time.

193
MD003. AUDIT OF CARE HOME RESIDENTS WITH PARKINSONISM IN
SALFORD

Cheng Khuang Lim*1; DR Theodora Jones*1; Carol Miller*1


1Ageing and Complex Medicine, NHS, United Kingdom

Email Address: limchengkhuang@hotmail.com

Introduction: Parkinsonism is common in care home. The prevalence and demographics of


care home patients with parkinsonism remains poorly investigated. Previous studies suggested
that around 5-7% care home patients have parkinsonism (Susan L. Mitchell MD 1996; Robert
J.Buchanana 2002). The 3 year mortality was 50% in care home with parkinsonism
(Fernandez HH 2002). Despite that, the diagnosis and management of care home patients
with parkinsonism were suboptimum (JP. 1991; Weerkamp NJ 2012; Makoutonina M 2010).

Methods: We obtained a list of care home residents with idiopathic Parkinson’s disease,
vascular parkinsonism and parkinsonism plus syndrome from community geriatricians in
February 2018. Following this, we collected data about patient demographic data, hospital
admissions in the last 24 months and advanced care plan using EPR. The data was processed
using Microsoft Excel.

Results: 56 patients (27 male) with the mean age of 79.89 (SD 8.14) were included in this
study. 36 of them also have the diagnosis of dementia.

Parkinsonism (Number of patients)


Idiopathic parkinson’s disease 31
Vascular parkinsonism 8
Parkinsonism-plus syndrome 11
Drug induced parkinsonism 6

Staging of IPD
Stage (Number of patients)
Stage 1: 0
Stage 2: 4
Stage 3: 17
Stage 4: 8

Number of medications
(Number of patients)
0: 13
1: 32

194
2: 8
3: 3
Regular physician who reviewed patients
Community geriatrician 31
Neurologist 11
Neurolopsychiatrist 5
None 9
34 of them had at least 1 admission in the last 24 months.
Reasons for admission (Number of patients)
Infection 11
Fall 6
Reduced conscious level 5
4 patients passed away since January 2018. None of them had up-to-date advanced care plan
and one of them died in ED after CPR.
(Number of patients)
Advanced care plan 19
Resuscitation 25
Preferred place of care/death 18

MDT input Number of patients


Physiotherapy 14
Occupational therapy 11
Speech and language therapy 19
Dietician 22

Conclusion: The management of care home residents with parkinsonism is suboptimum. We


should aim to improve their care in community and hopefully this can reduce unnecessary
hospital stay.

195
MD004. PEAK DOSE BALLISTIC MOVEMENTS TOWARDS THE
IMPLANTABLE PULSE GENERATOR LIFE END IN DEEP BRAIN
STIMULATION

Denzel Chong Jen-Rei*1; Hoe Wai Leng*2; Lee Hock Keong*2; Lim Thien Thien*2
1Neurology, RCSI and UCD Malaysia Campus, Malaysia, 2Neurology, Island Hospital Penang,

Malaysia
Email Address: jen_rei_95@hotmail.com

Introduction: The objective of this case report is to highlight the unusual presentation of peak
dose ballistic movements towards the end of the implantable pulse generator (IPG) life span in
a patient with bilateral sub-thalamic nucleus (STN) deep brain stimulation (DBS).

Results: We report a case of sudden severe peak dose ballistic movements and increased
“off” periods in a PD patient with bilateral STN DBS towards the end life of the IPG despite a
normal battery range according to the Medtronic Battery Status Indicator. The Unified
Parkinson Disease Rating Scale III (UPDRS III) escalated by 12 points (from 63 to 75 points)
with severe uncontrollable peak dose dyskinesia despite no medication adjustment.
Stimulation settings were cut down to as low as 1.0 V on the left STN, and 1.5 V on the right
STN at a frequency of 130 Hz and pulse width of 60 μs, yet the ballistic movements were still
noticeable. The patient remained in “on” period with no dyskinesia throughout the day after
replacement of the IPG with similar stimulation settings prior to the replacement of the IPG,
with 2.1 V on the left STN, and 1.6 V on the right STN at a frequency of 130 Hz and pulse
width of 60 μs. The patient’s UPDRS-III score was at 62 points after the replacement of IPG.

Conclusion: We observed that the battery status indicator did not accurately predict the
battery life of the IPG. Peak dose ballistic movements towards the end life of the IPG is an
unusual presentation and could be an indicator of end life of the IPG in DBS.

196
MD005. NEUROPROTECTIVE EFFECTS OF GOSSYPETIN IN ALZHEIMER'S
DISEASE: THERAPEUTIC APPROACHES TO EVALUATE THE
ACETYLCHOLINESTERASE AND BUTYL CHOLINESTERASE INHIBITORY
POTENTIAL

Dinesh Kumar Patel*1; Kanika Patel1


1Department of Pharmaceutical Science, Shalom Institute of Health and Allied Sciences,

Faculty of Health Sciences, Prayagraj, India


Email Address: dkp.itbhu@gmail.com

Introduction: Phytochemical present in medicinal plants have been used for the treatment of
numerous human health complication. Phytochemical can be categorized into different
chemical classes including flavonoidal compounds which are well known in the medicine for
their anti-cancer, anti-oxidant, anti-aging and anti-inflammatory activity. Gossypetin is pure
phytochemical found to be present in the numerous medicinal plants including Hibiscus
species.

Methods: Biological importances of gossypetin in the treatment of Alzheimer’s disease were


studied in the present investigation to know their effectiveness against acetylcholinesterase
and butyl cholinesterase (AChE and BuChE) through scientific database analysis of different
research work of the scientific fields. Present investigation revealed the biological importance
and therapeutic benefit of gossypetin in medicine and other allied health sectors. In order to
know the medicinal importance of gossypetin in brain related disorder, here in the present
investigation therapeutic benefit of gossypetin on cholinesterase enzymes such as AChE and
BuChE were investigated through scientific data analysis of different In-silico research work of
the literature. Experimental works have been performed to know the benefit of gossypetin in
the medicine.

Results: Scientific data analysis of different research work of the literature signified the
biological importance of gossypetin in the medicine due to their significant antioxidant
potential. Scientific research work data analysis revealed their effectiveness against various
form of cancerous disorders such as breast cancer and hepatocellular cancer. Molecular
docking study revealed the biological importance of AChE and BuChE enzymes in the brain
related disorders. Molecular docking study also signified the probable mode of interaction and
binding modes of inhibition of AchE and BuChE enzymes. Research work of different scientific
field also revealed the biological importance of hydrophobic interaction in the stability of
complex.

Conclusions: Present investigation signified the therapeutic benefit of gossypetin for the
treatment of Alzheimer's disease through interaction of cholinesterase enzymes.

197
MD006. SEROTONIN SYNDROME - CASE SERIES

Jayasree*1; Praveen Kumar Yada*1; Mohandas Surat*1; Subash Kaul*1; Sita Jayalakhsmi*1;
Varalakshmi*1
1Neurology, KIMS, India Neurology, KIMS, India

Email Address: babitasingh1987@rediffmail.com

Introduction: Serotonin syndrome (SS) is a life threatening condition caused by serotonin


toxicity, and usually involves a combination of drugs that increase serotonergic transmission.

Methods: All patients admitted with a clinical diagnosis of serotonin syndrome in KIMS
hospital between april 2019 to September 2019 were studied. A total of 3 patients were
diagnosed with Serotonin syndrome over a period of 6 months.

Results: Case 1: 58-year-old male patient, diabetic and hypertensive, on Amantadine and
Rasagiline for complaints of tremors, slowness of gait and activities since 3 months.
Escitalopram and clonazepam with amitriptyline was advised by the treating doctor.
Olanzapine was given for psychosis. Patient’s tremors increased further. CPK was 839 U/L.
Considering possibility of Serotonin syndrome, patient was treated with cyproheptadine and
clonazepam. Patient improved symptomatically and was discharged. Case 2: 70-year-old male
patient presented with complaint of involuntary movements of both upper limbs of 5 days
duration. Patient was on multiple antipsychotic drugs for psychiatric disorder. On examination,
patient had rigidity of both upper limbs with rest and postural tremor both upper limbs with
restless behaviour, agitation, decreased sleep and hallucinations. On evaluation CPK was
2413 U/L. Considering possibility of serotonin syndrome, all offending medications were
stopped and patient was treated with cyproheptadine, clonazepam, and levodopa. Case 3: 69-
year-old male patient with psychiatric disorder on multiple antipsychotics presented with
complaint of tremors all over body, irrelevant talk, and auditory hallucinations since 2 days. He
had history of right thalamic infarct in 2015. Considering possibility of serotonin sundrome,
antipsychotics were stopped and treated with cyproheptadine, clonazepam and levodopa.

Conclusion: The incidence of serotonin syndrome is rising, reflecting the growing number of
serotonergic drugs available and the increased use of these agents in clinical practice.

198
MD007. HEMICHOREA-HEMIBALLISMUS WITH NON-KETOTIC
HYPERGLYCEMIA: A MOVEMENT DISORDER RELATED TO DIABETES
MELLITUS

Rita Mulyana*1; Syahrul*1


1Department of Neurology Faculty of Medicine Syiah Kuala University, Dr. Zainoel Abidin

Public Hospital, Banda Aceh, Indonesia


Email Address: ritamulyana67@gmail.com

Introduction: Hemichorea-hemiballismus (HCHB) is characterized by unilateral, brief,


unpredictable involuntary movements of one body side.

Methods: Three patients with HCHB complaints with hyperglycemia in the early symptoms of
acute ischemic stroke were collected.

Results: Three cases of sudden-onset hemichorea-hemiballismus movements of the upper


and lower extremities were reported. First: Male, 63, uncontrolled movements in his right arm
and leg worsened over time with a history of type 2 diabetes mellitus, uncontrolled for 10 years
with the same history of DM in his family. The serum glucose level was 450 mg/dL, with 10.8%
HbA1c, 3+ urine glucose and negative urine ketone. CT brain showed infarction of left basal
ganglia. Second: Male, 51, involuntary movements in his left arm and leg for 13 days before
admitted to the hospital. Serum glucose levels at the beginning of the disorder were 455 mg/dL
with negative urine ketone. Head MRI showed hyperacute infarction in the right basal ganglia
and right caudate nucleus. Third: Male, 44, with past history of uncontrolled DM, coming with
involuntary movements in his right arm and leg occurring suddenly 4 hours while driving a car.
The serum glucose level at the beginning of the disorder was 264 mg/dl and negative urine.
Head CT scan showed infarction of the left basal ganglia.

Conclusion: The combination of new striatal lesions and hyperglycemia is related to the
appearance of unilateral hyperkinetic movement disorders in patients. When hyperglycemia is
detected and managed, movement disorders disappear within a few days and may not require
symptomatic therapy with dopamine receptor antagonists.

199
MD008. DIFFERENTIAL EFFECTS OF THYROTROPIN RELEASING
HORMONE ON MOTOR PERFORMANCE AND MOTOR ADAPTATION IN
PATIENTS WITH SPINOCEREBELLAR DEGENERATION

Takahiro Shimizu*1; Ryosuke Tsutsumi*2; Naomi Tominaga*3; Yoshikazu Ugawa*4; Kazutoshi


Nishiyama*2; Ritsuko Hanajima*1
1Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori

University, Japan, 2Department of Neurology, Kitasato University School of Medicine, Japan,


3Department of General Medicine, Kitasato University School of Medicine, Japan, 4Department

of Neuro-regeneration, School of Medicine, Fukushima Medical University, Japan


Email Address: tashimizu-sin@umin.ac.jp

Introduction: The cerebellum is known to play an important role in motor adaptation, including
prism adaptation. Thyrotropin releasing hormone (TRH) has been used as a treatment for
cerebellar ataxia in Japan. However, effects of TRH on cerebellar adaptation function have not
been studied. Herein, we studied effects of TRH treatment on the prism adaptation task.

Methods: Eighteen patients with spinocerebellar degeneration (SCD), including MSA-C,


SCA3, SCA6, SCA31, participated in this study. The participants received intravenous injection
of 2 mg/day protirelin tartrate once a day for 14 days. In the prism adaptation task, the
participants reached to the target on the screen wearing wedge prisms. We compared the
Scale for Assessment and Rating of Ataxia (SARA), baseline errors and the after effect (AE) of
the prism adaptation task between before and after TRH therapy. We also performed
correlation analysis between improvement of baseline errors or AE in the adaptation task, and
1) improvement of the total SARA score, 2) improvement of upper limb ataxia score, and 3)
improvement of axial ataxia score.

Results: TRH therapy significantly improved SARA (p=0.005). Multiple regression analysis
revealed that improvement of SARA score was mainly due to improvement of “Stance”
category score. TRH decreased baseline errors of the prism adaptation task (p=0.021), while
unaffected AEs (p=0.25). Correlation analysis revealed no significant correlation between any
factors.

Conclusion: TRH differentially affected cerebellar ataxic symptoms including baseline


reaching performance in the prism adaptation task, whereas TRH did not affect the learning
process of prism adaptation. Different cerebellar functional aspects may underlie the learning
process of motor adaptation and simple motor performance (classical ataxia).

200
MD009. A NEUROPHYSIOLOGICAL CHARACTERISTIC OF HOLME’S
TREMOR WITH THALAMIC LESION

Pattamon Panyakaew*1; Warongporn Phuenpathom*2


1Medicine, Chulalongkorn Center of Excellence for Parkinson’s Disease & Related Disorders,

Chulalongkorn University, Thailand; 1Medicine, Chulalongkorn Center of Excellence for


Parkinson’s Disease & Related Disorders, Chulalongkorn University, Thailand
Email Address: wph@chulapd.org

Introduction: Holme’s tremor (HT) is defined as a slow frequency tremor with irregular
amplitude that mostly occurs at rest and worsens with voluntary movements. Dystonia can be
occurred with HT and almost all patients had thalamic lesions. The neurophysiological
characteristics of HT are limited. The objective of this case report is to highlight the
neurophysiological finding of HT with co-occurrence of dystonia from the thalamic lesion.

Results: We reported a 46-year-old female patient with 13-year-history of left cerebellar


infarction with bilateral vertebral artery dissection post left cerebellar hemispherectomy with full
recovery. Recently she presented with left hemiparesis, hemiataxia and left hemianesthesia,
diagnosed as the right thalamic hemorrhage. Seven months later, she developed irregular
jerky movement of the left hand as well as dystonic posture. The neurophysiological test was
done to distinguish among HT, myorhythmia and dystonic tremor. The one-axis accelerometer
(ACC) was put on the left thumb and surface EMG was placed on the abductor pollicis brevis
(APB), the flexor digitorum superficialis of the left index (FDS 2) and the left extensor indicis
proprius (EIP). The ACC and EMG data showed regular sinusoidal movement of left hand at
the peak frequency of 3 Hz with synchronous contractions of the antagonist pairs at rest. The
similar pattern with larger amplitude of movement was demonstrated during postural holding at
several positions. These findings were consistent with the definition of HT with concomitant
dystonia rather than myorhythmia or dystonic tremor. The functional imaging also revealed
hypometabolism of right thalamus, cerebral peduncle, medulla and left cerebellum. Botulinum
toxin injection was done with good benefit.

Conclusion: The neurophysiological test is capable to differentiate HT from mimics.


Sinusoidal rhythmic slow oscillation with co-contraction of the antagonist pairs at rest with
worsening at voluntary movements might indicate HT with concomitant dystonia.

201
MD010. EARLY-ONSET PARKINSON’S DISEASE IN MALAYSIAN MALAYS

Yen Theng Chin*1; Zariah Abdul Aziz1; Khairul Azmi Ibrahim1; Jia Lun Lim2; Azlina Ahmad-
Annuar2; Shen-Yang Lim3; Ai Huey Tan3
1Neurology Unit, Hospital Sultanah Nur Zahirah, Kuala Terengganu, Malaysia, 2Department of

Biomedical Sciences, Faculty of Medicine, University of Malaya, Malaysia, 3Division of


Neurology and the Mah Pooi Soo & Tan Chin Nam Centre for Parkinson's & Related
Disorders, Department of Medicine, Faculty of Medicine, University of Malaya, Malaysia
Email Address: alishachin@gmail.com

Introduction: Although PD most commonly affects older individuals, it is not rare for people to
be diagnosed in their 40 or 50s, in whom genetic factors presumably contribute a greater role
in aetiology. To date, literature regarding epidemiology and genetics of PD in Malays remains
very scarce.

Methods: We analysed data from the PD registry of a tertiary hospital in East Peninsular
Malaysia, collected over 2011-2019. Diagnosis of PD was assigned by neurologists using
clinical diagnostic criteria. Patients with revised diagnoses during follow-up were removed from
the registry. EOPD was defined using two age cut-offs: age of diagnosis 40 years and 50
years. Positive family history was defined as having at least one member in the immediate or
extended family diagnosed with PD or having motor features that could be consistent with PD.

Results: Two hundred and thirty-one Malay patients were included in this study. The
frequency of EOPD was 4.32% (10/231) using the 40-years cut-off and 16.9% (39/231) using
50-years. Of 136 patients in whom family history information was available, 25 (18.4%) had a
positive family history. Interestingly, none of those with PD diagnosis 40 years had a positive
family history, while (4/31) 12.9% of those diagnosed at 50 years had a positive family history.

Conclusion: This pilot study revealed a relatively high frequency of EOPD in Malays. Further
studies to elucidate the role of genetics as well as environmental factors among Malay PD
patients may potentially unravel new insights into disease causation.

202
MD011. PERSISTENT CEREBELLAR DYSFUNCTION POST TOLUENE
SNIFFING

Loo Yin Pin*1; Tan Yin Yin2; Wong Chee Keong3; Eow Gaik Bee4; Kenny Tan5
1Medical Department, Hospital Pulau Pinang, Malaysia, 2Medical, Hospital Langkawi,

Malaysia, 3Neurology, Hospital Umum Sarawak, Malaysia, 4Neurology, Hospital Pulau Pinang,
Malaysia, 5Neurology, Loh Guan Lye Hospital, Malaysia
Email Address: lyplcw@yahoo.com

Introduction: Toluene toxicity from glue, paint thinners and petrol (gasoline) sniffing is being
recognized globally for its neurotoxic effect. We describe 2 patients (siblings) with toluene
induced encephalopathy at our centre. Both of them presented with cerebellar signs and had
glue sniffing activities.

Methods: A 24 years old gentleman presented with progressive hand tremor, unsteadiness
with abnormal eye movement for 3 years duration. Patient was an active substance abuser
and glue sniffer. On examination, he had an ataxic gait. He was dysarthric and had rotatory
nystagmus in both eyes. He demonstrated past pointing, intention tremor with
dysdiadochokinesia. The Romberg test was normal. He had normal tone, power, reflexes and
sensory over all limbs. He had normal full blood count, renal function, liver function test, serum
B12, red cell folate, T4, TSH, urine microscopy, HIV- 1 and 2, ANA, C-reactive protein, ESR,
creatinine kinase and serum magnesium. MRI of the brain revealed symmetrical T2W/FLAIR
hyperintense signals in substantia nigra of midbrain and surrounding the third ventricles. No
enhancing brain parenchymal lesion. There was presence of cerebral and cerebellar atrophy.
His sibling is a 22 years old gentleman with underlying bronchial asthma, active heroin chaser
and glue sniffer presented with complex partial seizure with post-ictal drowsiness. He had
ataxic gait, staccato speech, right upper limb past pointing, dysdiadochokinesia and bilateral
eyes nystagmus. Romberg test is normal. Neurological examination revealed normal tone and
reflexes, and full power of all extremities. His CT brain, cerebrospinal fluid analysis and other
blood investigations were normal. He remained ataxic upon discharge but subsequently
defaulted follow up.

Conclusion: Chronic toluene exposures can affect central nervous system by causing
demyelination and gliosis in cerebral and cerebellar white matter. This case series highlight the
presence of persistent cerebellar signs post toluene abuse.

203
MD013. IMPORTANCE OF BIOACTIVE FLAVONOIDS “PECTOLINARIN” IN
INFLAMMATION AND PAIN: VALIDATION THROUGH IN-VIVO, IN-VITRO
AND MOLECULAR STUDY

Kanika Patel* 1; Dinesh Kumar Patel1


1Department of Pharmaceutical Science, Shalom Institute of Health and Allied Sciences,

Faculty of Health Sciences, Shalom Institute of Health and Allied Sciences, Faculty of Health
Sciences, India
Email Address: kanikapatel1989@gmail.com

Introduction: Inflammation is part of the non-specific immune response that occurs in reaction
to any type of bodily injury which further generates chronic inflammatory diseases such as
rheumatoid arthritis, peripheral nerve disorders, asthma, cardiovascular diseases, diabetes,
cancer etc. Flavonoids represent a huge family of polyphenolic compounds and more referred
as “nutraceuticals” because of their vide range of pharmacological activities in the mammalian
cell. Pectolinarin is one of the main phytoconstituents of Cirsium japonicum which is an
important herb of Korea, China and Japan.

Methods: Pharmacological data have been collected and analyzed for analgesic and anti-
inflammatory activity through various in-vivo and in-silico model. Moreover molecular study
was also performed through scientific data analysis to know their interaction with various
inflammatory markers to confirm their pharmacological activity. Present work also summarized
the scientific data analysis of various molecular studies of pectolinarin with inflammatory
receptor targets such as COX-2, TNFα, IL-1β and IL-6.

Results: Scientific data analysis showed that pectolinarin reduces tissue damage and
alleviates the oxidative and inflammatory events through direct influence on COX-2, TNFα, IL-
1β and IL-6. Molecular study against inflammatory receptor targets showed their effectiveness
in the inflammatory disorders. Further data analysis also suggested that pectolinarin
significantly inhibited cell viability in rheumatoid arthritis and have inhibitory activities against
carrageenan-induced mouse paw edema and arachidonic acid-induced mouse ear edema in
some other study. However pectolinarin also exert significant analgesic and anti-inflammatory
activities in mice and rats which also support the molecular study. Owing to the importance of
pectolinarin in the medicine, a better understanding of their biological activities indicates their
potentials as anti-inflammatory agents.

Conclusion: Present study data analysis indicated and proven the anti-inflammatory potential
of pectolinarin which can be exploited for commercial designing of novel anti-inflammatory
drug in the future to treat various inflammatory disorders.

204
MD014. LONG TERM REPEATED USE OF BOTULINUM TOXIN INJECTION
FOR HEMIFACIAL SPASMS IN SRI LANKA

Vajira Weerasinghe*1; Padmini Dahanayake1; Chandima Sapurugala1; Nipuna Weerasinghe2;


RGL Shiroma1; Tharaka Dassanayake1
1Department of Physiology, Faculty of Medicine, University of Peradeniya, Sri

Lanka,2Department of Anatomy, Faculty of Medicine, University of Peradeniya, Sri Lanka


Email Address: vajira54@gmail.com

Introduction: Hemifacial spasm is a common indication for botulinum toxin use. Aim of this
study was to find out the efficacy and safety of long-term use of botulinum toxin for hemifacial
spasms in Sri Lanka.

Methods: A group of 28 patients with hemifacial spasms who had been given botulinum toxin
for more than 7 years were included in the study. Botulinum toxin was given at multiple sites
including over the eyelid, at the ipsilateral orbicularis oculi, nasalis, orbicularis oris, mentalis
and contralateral orbicularis oris sites on a dilution of 2 ml/100 units. Patients were then
followed up every 3 months. Degree of improvement, side effects if any, the total number of
units used and the information regarding the last injection used were collected.

Results: There were 24 females with a female preponderance of 86%. Mean age (SD) at first
injection of the patients was 58.7 (9.1) years. Mean (SD) duration of the repeated use was
10.5 (2.2) years. Range of the duration of treatment was from 7 to 15 years. During this
duration, mean (SD) number of injections given was 25.2 (8). Average number of units given
for each patient during these multiple injections was 20.3 (2.6). Improvement was seen in
96.5% of the times and side effects were seen only in 2.7% of the time and included heaviness
of the eye, mild lower facial weakness and pain at the site of injection which did not last long.
Patient compliance was good even after repeated long term use. There were no systemic side
effects and drug resistance to botulinum toxin was not seen in these patients.

Conclusion: These findings show that the long-term use of botulinum toxin for more than 10
years seems to be safe and efficacious for hemifacial spasm.

205
MD015. VITAMIN D INTAKE ENHANCES VITAMIN D RECEPTOR
EXPRESSION IN THE STRIATUM AND RESCUES MEMORY AND MOTOR
DYSFUNCTION IN MOUSE MODEL HUNTINGTON’S DISEASE

Manjari SKV*1 ; PG Satwik1 ; Sanjana Srinivas2 ; Pragya Komal1


1Biological Sciences, BITS Pilani Hyderabad India, India, 2Biotechnology, PES University

Banglore, India
Email Address: pragya@hyderabad.bits-pilani.ac.in

Introduction: A coordinated neuronal network between cortex and the basal ganglia is
required for normal motor function, which gets severely impaired in Huntington’s disease (HD).
In particular, the selective loss of medium spiny neurons in the striatum is considered as a
prime brain region responsible for movement disability observed in HD. In this regard, we
explored whether high Vitamin D (VD) supplementation shows neuroprotection in 3-
nitropropionic acid induced mouse model of HD (3-NP; 75mg/kg) as described previously
(Fernagut et al., 2002).

Methods: Male C57BL/6J mice (3-4 months old) were divided following groups: Vehicle (group
I), 3-nitropropionic acid (HD induced group, intraperitoneal injection), only VD supplemented
(group III, 500IU/kg) and 3-NP + VD supplementation (group IV). Animals were then subjected
to various behavioral tests like locomotion, gait analysis, Morris water maze (MWM) and rota-
rod analysis. After 30 days, animals were decapitated and striatal tissues were isolated to
check the mRNA expression of nerve growth factor (NGF), brain derived neurotrophic factor
(BDNF) and Vitamin D receptor (VDR).

Results: Our behavior and mRNA data shows that VD intake significantly rescued striatal
functions like motor co-ordination, locomotion and memory known to be severely impaired in
HD. Our results also indicate that VD mediated downstream neuroprotective pathomolecular
pathway involves increase expression of Vitamin D receptor (VDR) and neurotrophic factors
like NGF and BDNF in the striatum.

Conclusion: Altogether, we show that Vitamin D can be a potential “Biodrug†to rescue


neurodegeneration as observed in Huntington's Disease.

206
MD017. SPINAL SEGMENTAL MYOCLONUS MASQUERADING AS A
PSHYCOGENIC MOVEMENT DISORDER

Ankitta Sharma*1; Laxmi Khanna1


1Neurophysiology, Sir Ganga Ram Hospital, India

Email Address: ankkita13@gmail.com

Introduction: Spinal generated movement disorders are uncommon. An elderly gentleman


presented with distressing jerks of both lower limbs which caused him much social
embarrassment. He had received psychiatric treatment for these abnormal muscular spasms
without relief. He had become depressed and withdrawn when he first presented to our
outpatient department. In view of his clinical presentation, long term video EEG was done with
simultaneous EMG recording from the left tibialis anterior muscle. During his hospital stay, this
gentleman experienced several involuntary rhythmic contractions of both legs. The movements
were characterized by a sudden brief flexion of the trunk and hips with the thighs and legs
bending forwards and ankles dorsiflexing. The patient was fully conscious, alert and awake
during the attack. These jerks occurred in the recumbent position, but he could lose his
balance if they occurred on standing. These attacks were not stimulus sensitive and could be
provoked by stress. Myoclonic jerks of both lower limbs were recorded with no EEG correlate.
During jerking, EMG recording showed persistent contractions of the left tibialis muscle
occurring at frequency of 4-8 bursts per second, with each burst comprising of 4-5 motor unit
discharges. The burst of tibialis anterior muscle activity was involuntary and was absent during
voluntary leg movements.

Results: A routine clinical examination followed by a long-term video EEG with simultaneous
EMG clinched the diagnosis of a spinal segmental myoclonus. He underwent spinal
decompression surgery with fixation followed by treatment with levetericetam and clonazepam
which relieved his symptoms.

Conclusion: Spinal generated movement disorders are uncommon and are often attributed to
a psychiatric disorder in the absence of a better explanation. We take this opportunity to
elaborate on the case details, investigations and the treatment of spinal myoclonus.

207
MD018. DIAGNOSTIC APPROACH FOR IDIOPATHIC SIMPLE WRITER’S
CRAMP: A RARE CASE OF NEUROFUNCTIONAL DISORDER

Fahimma*1; Neila Raisa2


1Neurology Department, Brawijaya University, Indonesia, 2Movement Disorder Division,

Neurology Department, Brawijaya University, Indonesia


Email Address: fahimma.albinsaid@gmail.com

Introduction: Writer’s cramp is a part of focal hand dystonia that occurs task specifically
during writing. It may happen in a person with the habit to write rapidly, continuously, and
combined with individual vulnerability. This condition is highly disabling, accompanied by pain,
and significantly decreases functional activity. Not only professionally disturbing, but this
condition also leads to emotional and social problems for the patient.

Results: We present here an unusual case of a 25-year-old right-handed woman with


complaint of difficulty in writing since one year ago. She complained of involuntary muscle
contraction in her upper right extremity, an extension of the finger, deviation of the wrist and
elbow, followed by pain that happened only when she was writing. The symptoms progressed,
and she was unable to write even a few words. Neurological examination revealed no
abnormality. A series of laboratory tests, neurophysiology examinations, and brain MRI was
performed. Diagnosis was suggestive of idiopathic simple writer’s cramp. Botulinum toxin was
injected to the affected muscle. We also performed neuromuscular electrical stimulation as a
neuro-rehabilitation therapy. The handwriting performance was examined and compared
before and after treatment. The response to treatment was good, and the patient could write
better with reduced tension in her right hand after treatment.

Conclusion: Diagnosis writer’s cramp requires careful observation of the movements and
motor control. Identifying patient properly can be of great first step to overcome this condition.
Several diagnostic processes should perform to make an accurate diagnosis and appropriate
treatment.

Keywords: Writer’s Cramp, Focal Dystonia, Diagnostic Approach

208
MD019. GABAPENTIN AS ADD-ON THERAPY TO TRIHEXYPHENIDYL IN
CHILDREN WITH DYSKINETIC CEREBRAL PALSY: A RANDOMIZED
CONTROLLED TRIAL

Sonu kumar*1;
1Pediatrics, Pt. B.D.Sharma PGIMS Rohtak, India

Email Address: 9416593940s@gmail.com

Introduction: Mangement of hypertonia in dyskinetic cerebral palsy is challenging. The


present study was designed to compare the efficacy and safety of gabapentin as add-on
therapy to trihexyphenidyl in the treatment of children with dyskinetic CP.

Methods: An open labelled, randomized controlled trial was conducted among 60 children
aged 3-9 years with dyskinetic CP (GMFCS 4-5). The participants were randomized into two
groups: Gabapentin group receiving gabapentin with trihexyphenidyl (n=30) and
Trihexyphenidyl group receiving trihexyphenidyl alone (n=30). Primary outcome measures
include total Dyskinesia Impairment Scale (DIS), Dystonia Severity Assessment Plan (DSAP)
and International Classification of Functioning Disability, and Health-Children and Youth
Version (ICF-CY) assessed at four weeks and twelve weeks. Secondary outcome measure
include frequency and nature of adverse drug reaction observed after initiation of drug therapy.

Results: Out of 60 children, five children in gabapentin group and two children in
trihexyphenidyl group were lost to follow up at twelve weeks. There was significant reduction in
severity of dystonia in both the groups at four weeks as well as at twelve weeks (DIS: P<0.001,
DSAP: P=0.007, ICF-CY: P<0.001) but when the data were compared between the groups,
there was no significant reduction in severity of dystonia at four weeks and at twelve weeks
(DIS: P=0.09, DSAP: P=0.49, ICF-CY: P=0.25). Constipation (11.5% in gabapentin group and
14.3% in trihexyphenidyl group) was the commonest side effect observed in both groups and
both gabapentin as well as trihexyphenidyl were well tolerated without any serious side effect.

Conclusion: Trihexyphenidyl alone is as effective as combination of gabapentin with


trihexyphenidyl in reducing the severity of dystonia at twelve weeks. Hence, there is no added
benefit of gabapentin in management of dystonia in children with dyskinetic CP.

209
MD020. ATAXIA WITH OCULOGYRIC CRISIS REPRESENTING EARLY
PARANEOPLASTIC MANIFESTATION

Dr Rajarshi Chakraborty*1;
1Neurology, King George Medical University, India

Email Address: satyalung@gmail.com

Introduction: Paraneoplastic syndrome is an important masquerade involving multisystem


disease esp nervous system. Anti-Yo antibody can lead to paraneoplastic cerebellar
degeneration. Oculogyric crisis is a neurological emergency. This 45 year old lady presented
with insidious onset clumsiness of movement of all limbs for 15 days, altered behaviour 10
days along with abnormal eye movement for 3 days. There was no significant past history or
drug history. Her vitals were stable, with normal cardiovascular, gastrointestinal, respiratory,
lymphoreticular and gynaecological examination. There were cerebellar signs in nervous
system examination along with oculogyric crisis. Her routine tests along with brain MRI scans
were normal and she was managed with intravenous steroids. She showed improvement in
her sensorium along with eye movements in the due course of time

Results: This patient was suspected for paraneoplastic syndrome, the evaluation revealed
Anti-Yo positive status. However, her gynaecological workup did not reveal any significant
lesion at present and she has been advised for regular screening.

Conclusion: Paraneoplastic syndrome can have a protean manifestation. There should be a


suspicion of paraneoplastic syndrome even without evidence or anticipation of a malignancy.

210
MD021. DRUG INDUCED EXTRAPYRAMIDAL SYNDROME IN A YOUNG
GIRL DIAGNOSED TO BE SLE

Dr Rajarshi Chakraborty*1;
1Neurology, King George Medical University, India

Email Address: satyalung@gmail.com

Introduction: Extrapyramidal syndrome is a unique neurological entity. Drugs are an important


aetiology capable of causing it at any age group, esp young to adults. SLE is a common
autoimmune disease affecting young females and gas a myriad if presentations.

Results: This 18 year old girl presented with sudden onset tremulousness of body for 10 days
along with difficulty to move her limbs for 6 days. She was on olanzapune and valproate for
LADT 3 months because of behavioural abnormalities in the form of abnormal cries/laughters,
agitation, aggresive behaviour, convulsions. Her vitals were normal, and systemic examination
showed generalised rigidity with tremors. She was evaluated for NMS but her CPK was mildly
elevated. She was treated for eps with fluids, stoppage of valproate and olanzapine, initiation
of levetiracetam, lorazepam and supportive care. Her workup revealed the diagnosis of SLE
with positive anti-ribosomal antibody. She was started on steroids and she is off antipsychotics
during her follow-up, doing well.

Conclusion: It is equally prudent to find out the cause for need of antipsychotics rather than
just its initiation. SLE is an important entity that should be evaluated for psychosis in young
females. Immunosuppression is the key for treatment in SLE.

211
Motor Neuron Disease
MN003. IMPROVEMENT OF PROGRESSIVE DISTAL BIMELIC HIRAYAMA
DISEASE AFTER SURGERY: A CASE REPORT

Suwat Srisuwannanukorn*1; Piti Sinsoongsud1; Yupadee Fusakul2


1Division of Neurology, Faculty of Medicine Vajira Hospital, Navamindradhiraj University,

Thailand, 2Department of Rehabillitation, Faculty of Medicine Vajira Hospital, Navamindradhiraj


University, Thailand
Email Address: piti@nmu.ac.th

Introduction: Hirayama disease, a benign muscular atrophy of the distal upper extremity, is a
rare cervical myelopathy usually characterized by slowly progressive weakness of one hand
(monomelic amyotrophy). The pathognomonic finding is a forward-shifted spinal cord during
neck flexion by magnetic resonance imaging (MRI) , as in this young Thai man with rapidly
progressive weakness and muscle atrophy in the bilateral distal upper extremities. In this
report, we describe a 16-year-old man who had finger drop then rapidly progressive weakness
and wasting of both hands without pain for 6 months, electrophysiological evidences of motor
neuronopathy. He was diagnosed as progressive distal bimelic type of Hirayama disease by a
neurologist before getting MRI. Radiological findings showed normal study in neutral position
but during neck flexion showed cervical compression and venous plexus engorgement dorsal
to thecal sac. After three months without improvement by neck collar he underwent surgery to
prevent flexion. We will provide detail of clinical findings. electrophysiological findings,
interesting radiological findings and detail of operation.

Results: There were improvements of muscle power of both hands and muscle wasting at 3
months postoperatively.

Conclusion: Although Hirayama disease is benign in nature and frequently self-limiting but
radidly progressive weakness in some cases may have benefit from surgery to prevent neck
flexion.

212
MN004. MULTIPLE NEUROPHYSIOLOGIC BIOMARKERS EVALUATED THE
PULMONARY FUNCTION OF ALS

Naohiko Iguchi*1 ; Tomoo Mano2 ; Naoki Iwasa1 ; Akira Kido3 ; Kazuma Sugie1
1Department of Neurology, Nara Medical University, Japan, 2Department of Nerurology,

Department of Rehabilitation Medicine, Nara Medical University Japan, 3Department of


Rehabilitation Medicine, Nara Medical University, Japan
Email Address: naohiko-iguchi@naramed-u.ac.jp

Introduction: Declining respiratory-muscle function is the primary cause of death in patients


with amyotrophic lateral sclerosis (ALS). Spirometry is the most frequently used to predict
hypoventilation and survival in ALS, but their cut off value cannot indicate disease prognosis.
Multiple neurophysiological evaluations, which performed using diaphragmatic compound
motor-action potential (DCMAP) by phrenic nerve conduction study, diaphragm thickness
(DDT) by ultrasonography and new measurement system of thoracic excursion are proposed
as new variable biomarkers.

Methods: A total of 14 ALS patients diagnosed by El Escorial criteria were enrolled (4 females
and 10 males, mean age 71.0 years, mean disease duration 29.2 months, mean body mass
index ( BMI ) 19.5 kg/m2). We prospectively evaluated the spirometry, arterial partial
pressure of oxgen and carbon dioxide (PaO2 and PaCO2), baseline-to-peak amplitude of
DCMAP, and the differences in DDT and thoracic excursion between timed maximal inspiration
and maximal expiration (UMIN:000042222). We then analyzed the relationships between
neurophysiological biomarkers, the respiratory-function test, and the other quantitative
evaluations.

Results: All patients completed the examinations with the exception of the respiratory-function
test of two patients. The test–retest analysis of neurophysiologic biomarkers showed a high
level of reliability. Decrease of DCMAP and DDT was detected in patients who reported no
subjective dyspnea. DCMAP and DDT were found to correlate strongly with both PaCO 2 and
forced vital capacity (FVC); in contrast, thoracic excursion was strongly correlated to PaCO2
but not to FVC. Except for one female patient with high BMI (> 25), correlation with thoracic
excursion and FVC was observed.

Conclusion: Multiple neurophysiologic evaluations are reliable and reflects respiratory


function. DCMAP and DDT are useful for the objective evaluations of respiratory function
before the awareness of subjective dyspnea in patients with ALS.

213
MN005. PHARMACOLOGICAL IMPORTANCE AND BIOLOGICAL
POTENTIAL OF APIGETRIN FOR THE TREATMENT OF
NEURODEGENERATIVE DISORDERS: INVESTIGATION THROUGH
NUMEROUS SCIENTIFIC RESEARCH DATA ANALYSIS IN THE MEDICINE.

Dinesh Kumar Patel*1; Kanika Patel1


1Faculty of Health Sciences, SIHAS, SHUATS Prayagraj, U.P., India

Email Address: dkpatel.rs.phe@itbhu.ac.in

Introduction: Medicinal plants have been used as remedies and pharmaceutical products
since very ancient time and demand of herbal based drugs have been increasing in the
modern age due to their beneficial pharmacological activities of plants and their derived
products such as extracts, fractions and pure isolated phytocompounds. Flavonoidal
compounds have been known as a better natural phytoconstituents over a century and are
found to be present in the foods and plant derived natural product. Apigetrin, also called as
apigenin-7-O-glucoside is a natural flavonoid glucoside found to be present in the nature for
their anticancer activity.

Methods: In order to know the effectiveness of the apigetrin in the medicine for the treatment
of neurodegenerative disorders, here in the present investigation numerous scientific research
work data have been collected and analyzed. Detailed pharmacological activities of apigetrin
have been searched through numerous scientific data analysis in the present investigation.
Biological importance of cytokines, interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α)
in the treatment of neurodegenerative disorders have been also investigated through scientific
data analysis of different research works.

Results: From the analysis of various scientific researches work of different field of science
revealed the biological potential of apigetrin in the medicine. Detailed pharmacological studied
have proven the biological importance of apigetrin in the medicine for the treatment of
neurodegenerative diseases of human being. In some scientific research work apigetrin was
found to be effective against neurodegenerative diseases as it significantly reduced the
secretion of cytokines, TNF-α and interleukin-6 in BV-2 mouse microglia. In the scientific study
apigetrin significantly enhanced expression of antioxidant enzymes and increased radical
scavenging activity. Apigetrin has been found to inhibit neuroinflammation through antioxidant
and neuroprotection properties.

Conclusion: and biological investigations have proven the effectiveness of apigetrin for the
treatment of neurodegenerative disorders in the modern medicine

214
MN006. WARNING SIGN TO DIAGNOSE AMYOTROPHIC LATERAL
SCLEROSIS (ALS) – RARE CASE

Adecya Amaryllis Risa Putri*1; Shahdevi Nandar Kurniawan2


1Neurology Resident, Faculty of Medicine Brawijaya University, Saiful Anwar General Hospital,

Malang, Indonesia, 2Staff, Division of Neuropshyology, Neurology Department, Faculty of


Medicine Brawijaya University, Saiful Anwar General Hospital, Malang, Indonesia
Email Address: adecyaamaryllis@gmail.com

Introduction: A progressive and devastating Amyotrophic Lateral Sclerosis (ALS) disorder


locations are upper and lower motor neurons at the spinal or bulbar level. ALS is a fascinating
case because it is rare, with an unclear aetiology and many problems in choosing the right
management strategy. In the data obtained at the General Hospital in Malang Indonesia, there
were 3 cases of ALS and 2 cases of death in the last 4 years.

Results: We report 2 serial cases of suspected ALS patients with tetraparesis UMN type and
parese N. XII LMN type (atrophy and fasciculation). The first case, accompanied with
complaints of dysphagia, dysphonia, and parese N. IX, X, with an Electromyography (EMG)
result severe axonal demyelinating motoric sensoric polyneuropathy. And for the second case,
accompanied with complaints of dysartria, parese N. VII D UMN type, with an
Electromyography (EMG) result severe motor neuron disease lower brain stem dd severe
motoric neuropathy. In first case it is not an ALS because there were a sensory
polyneuropathy and second case there were clinically definite diagnosis criteria of ALS by
showing the presence of UMN and LMN symptoms in three regions with a result of an
electrophysiology. In first case patient died after 1 year of diagnosis and in second cases the
patient was still alive with limited physical activity.

Conclusion: ALS is difficult to diagnose because it has similar symptoms to some other
neurological diseases. From this rare series of cases, a clinician should be able to assess a
symptom that leads to ALS diagnosis such as the presence of upper and lower motor neuron
symptoms strongly suggests the presence of the disease.

Keywords: Amyotrophic Lateral Sclerosis (ALS), Electromyography (EMG), Neuron Motoric


Disease, Rare Case

215
Neuromuscular junction disorders (NMJ)
NJ001. QUALITY OF LIFE COMPARISON BETWEEN MYASTHENIA GRAVIS
WITH PLASMA EXCHANGE AND MYASTHENIA GRAVIS WITHOUT PLASMA
EXCHANGE

Caesario Adi Sukresna*1; Yudiyanta Samekto Wibowo1; Ahmad Asmedi1


1Neurology, Universitas Gadjah Mada, Indonesia

Email Address: rio117uk@gmail.com

Introduction: Myasthenia gravis (MG) is an autoimmune disease affecting neuromuscular


junction of skeletal muscle. Plasma Exchange (PE) is one of the treatment of choice in severe
MG. Myasthenia gravis with or without PE can weaken patient motor function and furthermore
produce negative impact on quality of life. Aim: To investigate Quality of Life difference
between MG with PE and without PE.

Methods: Twenty one adults with MG in outpatient clinic Neurology Department of Dr. Sardjito
General Hospital, Yogyakarta, Indonesia from September to November 2019 were recruited
and divided into two groups according to PE treatment. Quality of Life was evaluated with the
Short-Form 36-item Health Survey (SF-36).

Results: There were statistically significant differences in physical functioning scale (47.73 +
11.04 vs 74.50 + 10.39; p = 0.0001) and in role physical scale (54.54 + 24.54 vs 82.50 +
16.87; p = 0.007) between MG with PE group and MG without PE group. There were no
statistically significant differences in bodily pain, general health, vitality, social functioning, role
emotional, and mental health scale.

Conclusion: This study demonstrated that quality of life in physical functioning and role
physical scale was significantly reduced in MG with PE treatment.

Keywords: Myasthenia Gravis, Quality of Life, SF-36, Plasma Exchange

216
NJ002. MYASTHENA GRAVIS, A GREAT ‘MIMICKER’ AS AN INITIAL
AUTOIMMUNE PRESENTATION OF DIFFUSED LARGE B CELL
LYMPHOMA: A CASE REPORT

Thung Su Fui*1; Raja Ahmad Reza Raja Lope Ahmad1; Rose Izura Abdul Hamid2
1Medical, Hospital Raja Perempuan Zainab II, Kelantan, Malaysia, 2Neurology, Medical,

Hospital Raja Perempuan Zainab II, Kelantan, Malaysia


Email Address: thung3107@gmail.com

Introduction: Myasthenia gravis (MG) belongs to a spectrum of autoimmune diseases


mediated by antibodies to acetylcholine receptors (AChRs) or muscle specific tyrosine kinase
(MuSK). About 10-15% of MG cases have concurrent thymoma. However, only few reported
cases of MG had been associated with lymphoid malignancies.

Results: We report a case of MG in association with non-germinal center Diffuse Large B cell
lymphoma (DLBCL) in a 40 years old gentleman. He initially presented with MG in crisis with
bulbar and respiratory involvement in 2015. He was given intravenous immunoglobulin, steroid
and pyridostigmine. He improved, however, defaulted his follow-up. Three years later, he
presented back with MG in crisis. AChR antibody was positive and CT thorax showed anterior
mediastinal mass with differential of thymoma or teratoma. Intravenous immunoglobulin was
administered, but he developed acute extensive myocardial infarction. Thus, he underwent
plasma exchange and tracheostomy due to prolonged ventilation. He was discharged well with
steroid, pyridostigmine and azathioprine. He presented back 6 months later due to upper
airway obstruction. Direct laryngoscope revealed enlarged fungating mass. Biopsy from right
tonsil showed non germinal center DLBCL. He, subsequently, developed overwhelming sepsis
with multiorgan failure and succumbed to death

Discussion: There are more than one pathogenetic mechanism that underlie the association
between MG and lymphoma. There are pathways and genes involved in uninhibited
lymphocytic proliferation which could result in both autoimmunity and lymphoma. Alternatively,
lymphoid malignancy occurring after longstanding MG might be that the lymphoid malignancy
is the result of chronic MG. MG also occur with higher frequency in lymphomas with
mediastinal involvement due to the close proximity of lymphoma to the thymocytes that
express proteins with antigenic similarities to AChR leading to autoimmune reaction to the
latter.

Conclusion: The association between MG and lymphoma although rare, can occur especially
in those with mediastinal involvement.

217
NJ003. SONOGRAPHIC PATTERN OF MEDIAN NERVE ENLARGEMENT IN
HANSEN’S NEUROPATHY.

Madhu Nagappa*1 ; Lokesh Bathala2 ; Guruprasad S Pujar2 ; Anuradha H Keshavan2 ; Richa


D Jain3 ; Abhijit Das4 ; Man Mohan Mehndiratta5 ; Leo. H Visser6 ; Himanshu Mohan Kumar7
1Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS),

Bangalore, India, 2Neurology, Aster CMI Hospital, India, 3Radiology, Aster CMI Hospital, India,
4Pathology, Janakpuri Super Speciality Hospital, India, 5Neurology, Janakpuri Super Speciality

Hospital, India, 6Neurology, ETZ, St. Elisabeth Hospital, Tilburg, Netherlands, Netherlands,
7Statistics, Karnataka Medical Research Foundation, India

Email Address: madhu_nagappa@yahoo.co.in

Introduction: Median nerve enlargement in leprosy using high-resolution ultrasound seems to


be more proximal than in carpal tunnel syndrome (CTS), which might be an important
discriminating sign, but has not been studied systematically. The aim of this study is to
compare the sites of median nerve enlargement in patients with leprosy with that of patients
with CTS.

Methods: Transverse sections of the median nerve were recorded from wrist until the forearm
(at distal wrist crease, and at 2-cm: M1, 4-cm: M2, 6-cm: M3, 8-cm: M4 and 10-cm: M5,
proximal to the distal wrist crease in the forearm) and at mid arm and forearm in patients with
leprosy, CTS and healthy subjects.

Results: Twenty-six patients each with leprosy and CTS were compared with healthy controls.
Patients with leprosy included 6 (23%), 7 (26.9%), 7 (26.9%) and 6 (23.1%) patients with
borderline-tuberculoid, borderline-borderline, borderline-lepromatous and lepromatous leprosy
respectively. Cross-sectional area (CSA) of median nerve was increased in all patients with
leprosy as compared to healthy controls at all points of measurement. CSA was higher among
patients with leprosy as compared to CTS at all points except at the wrist. In patients with
leprosy, the maximal enlargement was noted 2-cm (M1) proximal to the wrist crease with
gradual tapering of the CSA proximally (p<0.05). In contrast, in patients with CTS the median
nerve was maximally enlarged at the distal wrist crease (p<0.05).

Conclusion: Enlargement of median nerve 2-cm proximal to the distal wrist crease is a
characteristic feature that distinguishes leprosy from CTS. This important finding can be used
at point-of-care ultrasound to identify patients with leprosy.

218
NJ004. MEDICINAL IMPORTANCE AND VISAGNIN FOR THE TREATMENT
OF NEUROINFLAMMATORY DISORDERS: PHYSIOLOGICAL FUNCTIONS IN
THE OXIDATIVE STRESS THROUGH SCIENTIFIC DATA ANALYSIS

Dinesh K Patel*1
1Shalom Institute Of Health and Allied Sciences, Prayagraj, India

Email Address: dineshpatel23shiva@gmail.com

Introduction: Visnagin is the main active phytoconstituents of Ammi visnaga belongs to the
furanochromone class chemical which have physiological functions against oxidative stress
and neurodegenerative disorders.

Methods: In order to know the medicinal importance and of visnagin for the treatment of
neurodegenerative disorders, in the present investigation biological potential of visnagin
against oxidative stress and neurodegenerative disorders have been investigated through
scientific data analysis of various literature works. Importance of visnagin for the treatment of
neuroinflammatory disorders have been investigated in the present investigation through
literature data analysis of various scientific research works. However pharmacological activities
of visnagin for their effectiveness against oxidative stress induced neurodegenerative
disorders have been also investigated in the present investigations through literature data
analysis. Literature data analysis of the numerous scientific research works of visnagin have
been also carried out to know the biological importance of and health beneficial potential of
visnagin in the medicine.

Results: Scientific data analysis of various scientific research work of the literature revealed
the biological importance of visnagin in the medicine and other allied health sectors. Literature
data analysis in the present investigation revealed the biological importance of visnagin in the
neuroinflammation and oxidative stress. Literature data analysis revealed the biological
importance of visnagin on cell injury. Molecular study data analysis signified the therapeutic
importance of visnagin in the medicine for the treatment of oxidative stress induced
neurodegenerative disorders.

Conclusion: Literature data analysis of various research works signified the biological
potential of visnagin in the oxidative stress induced neurodegenerative disorders.

219
NJ005. CLINICAL PROFILE AND OUTCOME OF CHILDHOOD-ONSET
MYASTHENIA GRAVIS

Nibu Varghese*1 ; Sumanth Shivaram1 ; Madhu Nagappa1 ; Doniparthi V Seshagiri1 ; Sanjib


Sinha1 ; Arun B. Taly1
1Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India

Email Address: nibuvrghs@gmail.com

Introduction: Myasthenia gravis (MG) is an autoimmune disease with peak incidence in fourth
decade. Childhood-onset MG is uncommon. We aim to describe the clinical profile and
outcome of childhood-onset MG.

Methods: Retrospective chart review of 27 patients (M:F 13:14) with childhood-onset MG seen
in a single neurology unit between 2000 and 2019. Diagnosis was established by decremental
response in repetitive nerve stimulation, response to neostigmine, and/or antibody positivity.
Congenital Myasthenic syndromes were excluded.

Results: Demographic and clinical features included: mean onset-age of 12.81±4.63 years,
mean symptom-duration of 23.63±36.49 months, ptosis (100%), ophthalmoplegia (63%), facial
weakness (55.55%), chewing difficulty (40.74%), dysphagia (48.14%), dysarthria (14.81%),
neck weakness (25.92%), shoulder-girdle weakness (44.44%), pelvic-girdle weakness
(48.14%), and respiratory weakness (14.81%). Myasthenia Gravis Foundation of America
(MGFA) grade at first evaluation was I (n=11), IIa (n=7), IIb (n=2), IIIa (n=4), IIIb (n=2), and IVb
(n=1). Two patients developed myasthenic crisis during course of illness. Associated illnesses
included seizure disorder (n=2), diabetes (n=1), hypothyroidism (n=1), and psychiatric illnesses
(n=4). Acetylcholine receptor antibody was positive in 12 patients while MuSK was positive in
one patient. Chest computed tomography (CT) showed thymic hyperplasia in 12 patients and
was normal in 6 patients. Thymectomy was performed in 9 patients (hyperplasia in 7, normal
histology in 2). Time from MG onset to initiation of pyridostigmine was 13.80±27.83 months
and mean maximum dose of pyridostigmine was 172.22±127.50 mg/day.
Immunosuppressants administered in 22 patients included intravenous immunoglobulin (n=3),
plasma exchange (n=5), steroids (n=22), azathioprine (n=4), mycophenolate (n=2),
methotrexate (n=3), cyclophosphamide (n=2), and rituximab (n=1). Of 20 patients with follow-
up details, remission was achieved in 17, of which 12 could stop pyridostigmine.

Conclusion: In our cohort, majority had mild-moderate MG and myasthenic crises were
uncommon. Treatment response was good. Remission was achieved in majority and in many
of them, pyridostigmine was discontinued.

220
NJ006. RITUXIMAB IN MYASTHENIA GRAVIS: EXPERIENCE FROM A LOW
– AND MIDDLE – INCOME COUNTRY

Sumanth Shivaram*1 ; Nibu Varghese1 ; Madhu Nagappa1 ; Doniparthi V Seshagiri1 ; Sanjib


Sinha1 ; Arun B. Taly1
1Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India

Email Address: sumanth1983s@gmail.com

Introduction: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular


junction with 10% being refractory to conventional immunosuppressants. Rituximab is a
potential therapeutic option for these patients. We aim to describe the response to rituximab
and safety profile in MG.

Methods: Retrospective chart review of 12 patients (M:F=5:7, mean age: 41.08±10.96 years)
with MG treated with rituximab and followed up in a single neurology unit at the National
Institute of Mental Health and Neurosciences, Bangalore, between 2017 and 2020.

Results: Mean duration of MG was 105.16±71.8 months (range 6-260 months). Pre-rituximab
treatment included steroids, azathioprine, methotrexate, mycophenolate, cyclophosphamide,
and thymectomy. Pre-rituximab MGFA grading included: IIa (n=1), IIb (n=1), IIIa (n=2), IIIb
(n=5), IVb (n=1), and V (n=2). Reasons for initiation of rituximab included poor response to
steroids (n=10), comorbid illnesses limiting the use of other drugs (n=4), and drug-induced side
effects (n=2). The mean duration of follow-up was 20.33±13.64 months (range 3-37 months).
Two patients developed myasthenic crisis after the induction phase of rituximab and one
patient died. Another patient died after 25 months of rituximab therapy due to an unrelated
cause. The rest improved, pyridostigmine dose was reduced in four and was stopped in six
patients. Other immunosuppressants were continued in all but one patient. Complete stable
remission and pharmacological remission were achieved in one and four patients respectively
while five patients had minimal manifestations. No infusion-related adverse events were noted.
One patient developed transient asymptomatic neutropenia three weeks after rituximab
infusion.

Conclusion: Rituximab appears to be a safe and effective therapeutic option for patients with
refractory MG. however, a proportion does develop worsening similar to steroid-induced
exacerbation. Most of the patients in our cohort had a good response with infrequent side
effects.

221
Intraoperative Monitoring (IOM)
IM002. SOMATOSENSORY EVOKED POTENTIALS IN CERVICAL AND
CEREBELLOPONTINE ANGLE NEUROSURGICAL PROCEDURES:
EXPERIENCE FROM A TERTIARY CARE CENTER IN INDIA

Mahesh Gadhvi*1
1Physiology, All India Institution of Medical Sciences, Jodhpur, India

Email Address: magadhvi30@gmail.com

Introduction: The role of Intraoperative neuromonitoring (IONM) as a guiding technique in


neurosurgical procedures is well established. Somato-Sensory Evoked Potentials (SSEPs) are
an important modality in IONM. We report our experience with SSEPs in surgeries of Cervical
Spine, Cranio-Vertebral junctions and Cerebellopontine angle at our centre.

Methods: Sixty two patients underwent surgery in the cervical region and cerebellopontine
angle under IONM guidance at the Department of Neurosurgery from May 2018 to September
2018. Out of which SSEPs were not performed in 22 patients and SSEPs could not be
recorded in 20 patients due to technical reasons, therefore 20 patients were included in the
study. SSEPs were recorded during the surgical procedures to provide information about
neural integrity. Median nerves in both the limbs were stimulated at the level of the wrist using
sterile transdermal needle electrodes. SSEP recordings were obtained using corkscrew needle
electrodes placed at the scalp in Cp3, Cp4 and Fz, according to the 10-20 recording system.
Signals were acquired using Medtronics NimEclipse© and Natus Protektor© system. The
cortical recordings were taken from Cp3-Fz and Cp3-Cp4 montage for right sided SSEP and
Cp4-Fz and Cp4-Cp3 montage for left sided SSEP.

Results: The stimulation intensity was decided on a case-to-case basis. Range of stimulation
varied from 10 - 23 mA with frequency of 4.70 Hz and stimulation duration of 0.2 ms. Real time
averaging of 1000 waveforms was done. N20 and P22 latencies were checked pre-operatively
just after induction, intra-operatively and post-operatively just before closure. In all the 20
patients, intra-operative period was uneventful. All cases showed normal recovery in the post-
operative period.

Conclusion: SSEP monitoring is a very useful tool for intraoperative sensory tract monitoring
in the patients undergoing Cervical and Cerebellopontine region surgery. Change in the
latency or amplitude in the cortical SSEP recordings can predict injury to the vital structures
which can be reversed by surgeons if early warning is raised by Neurophysiologists.

222
IM003. THE UTILITY OF INTRAOPERATIVE NEUROPHYSIOLOGICAL
MONITORING IN SURGICAL CORRECTION FOR SPINAL ARTERIOVENOUS
SHUNTS: A HISTORICAL CONTROL STUDY

Sooyoung Kim*1 ; Jun-Soon Kim1 ; Ki-Jeong Kim2 ; Kyung Seok Park1


1Department of Neurology, Seoul National University Bundang Hospital, Seongnam, Korea,
2Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National

University College of Medicine, Seoul, Korea


Email Address: ksy9911@naver.com

Introduction: This study aimed to investigate the utility of intraoperative neurophysiological


monitoring (IONM) in surgical correction for spinal arteriovenous shunts (SAVSs).

Methods: We retrospectively reviewed 42 patients underwent surgical correction for SAVSs.


29 patients who underwent multimodal IONM (transcranial electrical motor-evoked potentials
[tcMEP], somatosensory-evoked potentials [SSEP], continuous electromyography, and
bulbocavernosus reflex [BCR]) from 2011 to 2020 were compared to 13 historical controls
(non-IONM groups) from 2003 to 2009. The rates of postoperative neurological deficits,
neurophysiological warnings, and their characteristics were analyzed. Postoperative
neurological deficits were evaluated at one month, six months, and one year after surgery,
respectively.

Results: Among 42 patients, postoperative neurological deficit rates were 13.8% and 46.2% in
the IONM and non-IONM groups, respectively (p=0.046). In total, 5 warnings were observed
during IONM (3 tcMEP; 1 tcMEP and SSEP; 1 BCR). Three cases were true-positive. The
amplitude of right abductor halluces MEP decreased during surgery in patient 1 who had no
weakness of the right leg previously. After surgery, weakness of right leg was newly observed
in patient 1. Patient 2 who had both leg weakness previously got worse both leg weakness
than before surgery. Her left abductor halluces MEP disappeared and never recovered during
surgery. Patient 3 had normal bladder function before surgery. His baseline BCR was
successfully recorded but the end of the surgery, the bilateral BCR was completely
disappeared. He had severe urinary retention after surgery. Sensitivity, specificity, positive
predictive value, and negative predictive value of IONM warnings for detecting postoperative
neurological complications were 75.0%, 92.0%, 60.0%, and 95.8% respectively.

Conclusion: The utility of IONM in surgical correction for SAVSs has not been evaluated due
to its rarity. This is the first study to provide results revealing that IONM helps to improve
postoperative neurological outcome through comparison with non-IONM group in surgical
correction for SAVSs.

223
Evoked Potential Studies (EP)
EV001. VISUAL EVOKED POTENTIAL FINDINGS IN NEUROMYELITIS
OPTICA SPECTRUM DISORDERS

Htet Htet Lin*1 ; Seinn Mya Mya Aye2 ; Win Min Thit1
1Neuromedical Department, University of Medicine (1) Yangon, Myanmar, 2Consultant

Neurologist, University of Medicine (1), Myanmar


Email Address: drhtethtetlin80@gmail.com

Introduction: Neuromyelitis optica is an inflammatory, demyelinating syndrome of central


nervous system that is characterised by severe attacks of optic neuritis and myelitis. It has a
high morbidity and mortality. Five years after initial presentation, more than 50% of patients
with NMO will be blind in one or both eyes or require ambulatory help.

Methods: A total of 68 patients with neuromyelitis optica spectrum disorders (NMOSD)


admitted to neuromedical ward of Yangon General Hospital were prospectively studied for
visual acuity, and visual evoked potential were performed.

Results: Visual acuity was ranged from normal to blindness. Visual impairment were classified
into mild, moderate, severe and blindness according to WHO classification. At admission,
among 68 patients, 4.4% of the patients had moderate vision impairment, 1.5% had severe
vision impairment, 50.0% had blindness, and 44.1% had no vision impairment. Among 136
eyes examined, 43 (31.6%) had no visually evoked response. Out of 93 eyes (68.4%) in which
VEP responses were detected, 61 (65.6%) had P100 wave latency within normal limits, while
remaining 32 (34.4%) had increased wave latency. Latency values among study population
were found to be ranging between 84.7 ms and 164.6 ms and the average latency of P100
was 111.8 ± 19.5 ms. Among 93 eyes in which visual response was evoked, it was found that
14 eyes (15.1%) had reduced P100 wave amplitude with normal latency.

Conclusion: In Neto et al. study (2013), reduced P100 wave amplitude with normal latency in
65% of the 20 eyes in which the visual response was evoked .This pattern was called the
‘NMO pattern’. So regarding these data, such an NMO pattern was not evident in present
study. Because most of patients have severe vision impairment and latency is prolonged in
most of the patients.

224
EV002. VISUAL EVOKED POTENTIALS IN NEUROMYELITIS OPTICA
SPECTRUM DISORDERS

Otto Hernandez Fustes*1 ; Claudia S. Kamoi Kay1 ; Paulo José Lorenzoni1 ; Renata Dal-Prá
Ducci1 ; Lineu Cesar Werneck1 ; Rosana Hermínia Scola1
1Neurology & Neurophysiology, Complexo Hospital de Clínicas da UFPR, Brazil

Email Address: otto.fustes@hc.ufpr.br

Introduction: Neuromyelitis optica spectrum disorders (NMOSD) are rare antibody-mediated


disorders of the central nervous system with a predilection for the spinal cord and optic nerves.
The clinical utility of evoked potential recordings (EPs) has already been established for
multiple sclerosis, in particular, the abnormal visual evoked potentials (VEP) a key criterion in
the McDonald diagnostic criteria for MS. On the other hand, there have been few reports on
EPs in patients with NMOSD.

Methods: The VEP were prospectively performed in 13 patients with NMOSD. All the patients
were recruited from the outpatient clinic of a demyelinating diseases center. The recorded was
done as recommended by the International Federation of Clinical Neurophysiology. All studies
were conducted in accordance with ethical principles after obtaining patient informed consent.

Results: We evaluated 12 women with mean age of 42 years old and one man of 25 years
old. In 6 the examined eye, a response was not obtained. In the remaining 20, we found a
significant increase in P100 latency without amplitude change. The relation to the VEP variable
(Normal vs Abnormal), there was a statistical difference in relation to P100 Oc latencies.

Conclusion: VEP is a well-known noninvasive tool for investigating the function of the visual
system. Decreased amplitude and prolonged latency of VEP recording is believed to reflect the
damage of axon and demyelination in the optic nerve. VEP showed a significant increase in
P100 latency. VEP is a non-invasive, painless, fast and low-cost exam which provides
neurophysiological data for diagnosis of NMOSD. Our results reinforce the concept of VEP
evaluation is currently the standard method used to confirm the presence of clinical and
subclinical Optic Neuritis.

225
EV003. ORIGIN OF GIANT SOMATOSENSORY EVOKED POTENTIALS
(SEPs) IN BENIGN ADULT FAMILIAL MYOCLONUS EPILEPSY (BAFME)
USING PRINCIPLE COMPONENT ANALYSIS

Maya Tojimo*1
1Neurology, Kyoto University Hospital, Japan

Email Address: maya0826@kuhp.kyoto-u.ac.jp

Introduction: Both benign adult familial myoclonus epilepsy (BAFME) and Unverricht-
Lundborg disease (ULD) belong to progressive myoclonus epilepsy with giant
somatosensory evoked potentials (SEPs), but their clinical courses are different from each
other. We aimed to delineate the generator source of giant SEP P25 and their high
frequency oscillations (HFOs) in patients with BAFME and ULD using principal component
analysis (PCA).

Methods: PCA was performed on giant SEPs and their HFOs obtained by multi-channel
scalp recordings of five patients with BAFME and four patients with ULD. To identify H FOs,
SEPs were digitally filtered using a 400–1000 Hz band-pass filter. From the peak latencies
of the first or second principal component (PC), we identified the corresponding peaks of
original P25 and HFOs near P25 (P25-HFOs). Then, from the polarity and distribution of first
and second PCs, the P25 and P25-HFOs were estimated as tangential dipole with phase
reversal between frontal and parieto-occipital electrodes or as radial dipole without phase
reversal.

Results: All five patients with BAFME had P25-HFOs, and both P25 and P25-HFOs were
estimated as radial dipole, whereas in all four patients with ULD, P25-HFOs was not present
and the estimated components of P25 varied among patients.

Conclusion: The generator sources of both P25 and P25-HFOs in giant SEPs in patients
with BAFME are radial dipole, which suggests crown origin, and P25-HFOs did not occur in
patients with ULD. The generator mechanism of giant SEP P25 and P25-HFOs of BAFME
may be different from that of ULD.

226
Peripheral Nerve Disorders
PN001. ELECTROMYOGRAPHY (EMG) OF ABDUCTOR POLLICIS BREVIS
(ABP) MUSCLES IN DIABETES MELLITUS TYPE 2

Dil Maya Limbu*1 ; Dr. B. H. Poudel1 ; Dr. Dilip Thakur1 ; Dr. Robin Maskey2
1Basic and Clinical Physiology, B.P. Koirala Institute of Health Sciences, Dharan -18,

Nepal, 2Department of medicine, B.P. Koirala Institute of health sciences, Dharan, Nepal
Email Address: agusnur_salim@yahoo.com

Introduction: The prevalence of chronic disease diabetes mellitus is rapidly rising all over the
globe. The number of diabetic mellitus patients with diabetic neuropathy is increasing as
estimated as 45% of patients develop diabetic polyneuropathy.

Methods: The study was done in 10 (M=4, F= 6, age 51.38±11.71 years) consenting diabetic
patients. sEMG of ABP muscles were recorded during rest and contraction state. Values
obtained were compared between male and female diabetic groups. An independent t-test was
applied to analyze the data.

Results: EMG amplitude of right and left ABP muscles at rest are (8.67±1.15, p < 0.002 vs
5.00±0.00, 10.00±0.00, p < 0.001) were decreased significantly in male than in female. Only
EMG amplitude of left ABP muscles at contraction (243.60±51.48 vs 124.00±117.78, p <
0.042) was decreased significantly in male than in female diabetic group. EMG frequency of
right and left ABP muscles (53.45±72.92, 76.81±11.42, p < 0.001) at contraction was
decreased significantly in male than female diabetic group.

Conclusion: The results suggest neuropathy can be detected early by the EMG test. Gender
affects EMG parameters in diabetic patients.

227
PN002. ISOLATED AXILLARY NERVE INJURY: A CASE REPORT

Pricilla Yani Gunawan*1 ; Novia Lauren Sieto1 ; Devita Widjaja1 ; Yusak Mangara Tua
Siahaan1
1Neurology, Universitas Pelita Harapan, Indonesia

Email Address: pricilla.gunawan@uph.edu

Introduction: The axillary nerve is a terminal branch of the posterior cord of the brachial
plexus and derives from the ventral rami of the fifth and sixth cranial nerves. Its injury is
infrequently diagnosed and a rare occurence. It generally appears secondary to the trauma
and characterized by severe shoulder pain at night with limitation of the shoulder movements
followed by shoulder atrophy in the following weeks. Case Illustration: A 22-year-old male was
admitted with decreased of consciousness, headache, neck stiffness, subconjunctival
hemorrhage, and decreased left arm strength along with range of movement, especially left
shoulder abduction and left arm pronator after a motorcycle accident. Vital signs were
unremarkable. Head CT scan showed minimal subarachnoid hemorrhage (SAH) at anterior
interhemisphere falx, and tripod fracture at left zygomatic bone. Hand, humeral and antebrachii
X-ray showed no fracture nor dislocation. Electromyography (EMG) test showed no nerve
conduction velocity of left axillary nerve injury. Patient underwent physiotherapy and given
neuroprotectors. After 7 days, there was a significant improvement in shoulder abduction and
left arm pronation. Patient was then discharged and scheduled for further physiotherapy.
Discussion: Isolated lesions of the axillary nerve occur most commonly after a fracture or
dislocation around the shoulder. Most recover spontaneously. In this case, patient had a speed
recovery a week after the injury. Electrodiagnostic study shows a physiologic block of nerve
conduction in the axillary nerve. At the end of the week, patient showed significant strength.
This might be due to temporary interruption of conduction without loss of axonal continuity
(neuropraxia).

Conclusion: Isolated axillary nerve injury is often a low-grade injury which progresses to full
recovery without intervention. Electrodiagnostic study might reveal no nerve conduction.
However, recovery of nerve conduction deficit is full, and requires days to weeks.

228
PN003. REFRACTORY CHRONIC INFLAMMATORY DEMYELINATING
POLYNEUROPATHY TREATED WITH SUBCUTANEOUS BORTEZUMIB

Chong Li Jie*1; Chia Yuen Kang1


1Neurology, Hospital Queen Elizabeth, Malaysia

Email Address: lijie_chong@hotmail.com

Introduction: A 73-year-old man with underlying diabetes presented in May 2017 with
progressive bilateral upper and lower limb weakness and numbness for 3 months. Examination
revealed power of 2-3 on lower limbs and 3 on upper limbs. Reflexes were absent with gloves
and stocking sensory loss. NCS showed diffused sensorimotor demyelinating polyneuropathy.
Monoclonal IgG kappa was detected. Urine free light chain showed raised kappa. However,
BMAT showed no evidence of myeloma. Abdominal fat pad biopsy showed no evidence of
amyloidosis. Anti-ganglioside and paraneoplastic antibodies were not detected. Sural nerve
biopsy showed axonal loss. CT NTAP was unremarkable. He responded initially with IVIG
therapy but In July 2017, he had a relapse with worsening lower limb weakness. He was given
IVIG 1 g/kg maintenance together with dexamethasone 4 weekly pulse therapy with some
improvement. In Oct 2017, he presented again with worsening weakness. Besides, there was
dysphagia and dysphonia. He developed type 2 respiratory failure requiring NIV and ICU
admission, requiring 2 weekly plasmapheresis to rescue him from recurrent respiratory failure.
IV Rituximab was tried but with little success even after 1 month of completion. Subcutaneous
Bortezumib was given for 2 courses; Feb 2018 and Aug 2018.

Results: After first course of Bortezumib, he improved significantly; with no more dysphagia
and respiratory failure. He was subsequently discharged in Apr 2018, since then on monthly
maintenance plasmapharesis and later 3 monthly. Upon latest review in Nov 2019, he was
able to ambulate independently; muscle power improved with upper limbs proximal 5, distal 3;
lower limbs proximal 5, distal 4. NCS showed improvement of motor CMAP especially in upper
limbs.

Conclusion: Bortezumib can be considered in refractory CIDP despite limited experience in


the literature. However it should be reserved after conventional therapies have failed due to its
neurotoxic side effects.

229
PN005. THE CLINICAL CHARACTERISTICS AND INCIDENCE OF
HYPOALBUMINEMIA IN PATIENTS WITH GUILLAIN BARRE SYNDROME IN
YOGYAKARTA, INDONESIA

Indra Sari Kusuma Harahap*1 ; Desby Juananda1 ; Wikan Pambudi Nuroso1 ; Ahmad
Asmedi1
1Department of Neurology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah

Mada, Yogyakarta, Indonesia


Email Address: iin_999@yahoo.com

Introduction: GBS is an acute polyradiculoneuropathy typically presenting with sensory


symptoms and weakness. Approximately 70% of patients report a recent preceding
inflammation process. Investigators of large, worldwide, collaborative studies of the spectrum
of GBS are accruing data for clinical databases to inform the development of outcome
predictors and disease biomarkers. The low of serum albumin level, as the most abundant
protein in plasma, may be related to acute systemic inflammatory reaction. In this study, we
investigate the clinical characteristics and incidence of hypoalbuminemia in patients with GBS.

Methods: We retrospectively analyzed the clinical data of adult GBS, between 2014 to 2019,
from medical record of the patients hospitalized at Dr.Sardjito Hospital, as central district
hospital in Yogyakarta. Serum albumin concentration was determined before treatment.

Results: There were 73 patients were enrolled in this study. We found that male was slightly
more affected than female (53,4% vs 46,6%). The elderly patients (60 years) were less than
adult non-elderly patients (19% vs 81%). The adult age varied from 18 year to 78 years. During
hospitalization, the mean length of stay (LOS) were 24 days (range from 4 days to 154 days).
The in-hospital mortality rate was 9.6% (7 of 73 patients), while 66 (90,4%) patients with GBS
were alive at discharge. The mean day of death were 40 days, longer than the mean of LOS,
indicating the complication during treatment may happened. Before treatment, the mean serum
albumin level was 4.03 g/dL with hypoalbuminemia (albumin<3.5 g/dL) in 10 (14%) of 73
patients.

Conclusion: The incidence of GBS decreased with age. The mortality may happen in long-
term of hospital treatment. There is a low incidence of hypoalbuminemia in acute GBS. Further
studies are required to confirm the correlation between hypoalbuminemia and the severity of
clinical course in patients with GBS.

230
PN006. GUILLAIN BARRE SYNDROME TREATMENT RELATED
FLUCTUATION (GBS-TRF) FOLLOWING MENINGOCOCCUS VACCINATION:
A CASE REPORT

Indra Sari Kusuma Harahap*1; Ismail Setyopranoto1


1Department of Neurology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah

Mada, Yogyakarta, Indonesia


Email Address: iin_999@yahoo.com

Introduction: GBS is an autoimmune neurological disorder, characterized by acute limb


weakness and areflexia. The pathogenesis of GBS is triggered mostly by infection process.
However, the consequence of vaccination can cause GBS has been widely discussed. Some
studies address the potential relationship of GBS to influenza vaccination, while other
vaccination is rarely described. In this study, we reported a case of GBS-TRF after
administration of meningococcus vaccination. Case presentation: A 64-year-old man
presented with glove-stocking hypoesthesia followed by flaccid tetraparesis, which occurred
two weeks after meningococcus vaccination. There were no history of any infectious diseases
preceding his symptoms. He and his family were administered these vaccinations, as the
prerequisite for going to Umrah. After coming back of his Umrah, he was diagnosed with GBS
based on history, clinical features and test results. Neurological examination revealed absent
of knee and ankle reflexes. Nerve conduction studies indicated the decreased of sensory
nerve action potential, amplitudes and conduction velocity, also prolongation of distal latency.
He had six times therapeutic plasma exchange (TPE) and the symptoms slightly resolved at
discharge. One month after plasma exchange, he was readmitted due to dyspnea, worsening
of his weakness and severe hyponatremia. He was transferred to intensive care unit with
mechanical ventilation. He was diagnosed as GBS-TRF. The treatment was intravenous
immunoglobulin (IVIG) with full dose of 400 mg/kg/day. Unfortunately, the patient developed
some complications during intensive care. He did not survive, suffering from cardiac arrythmia
and septic shock, after IVIG treatment.

Conclusion: Meningococcus vaccination may cause GBS as an unexpected abnormal


autoimmune response. It is important to note the possibility of neurological complications after
vaccine administration.

231
PN007. A CASE PRESENTING CLINICAL AND LABORATORY
CHARACTERISTICS OF FISHER SYNDROME AND LAMBERT-EATEN
MYASTHENIC SYNDROME (LEMS)

Kazuna Ikeda*1 ; Bungo Hirose2 ; Daisuke Yamamoto1 ; Shun Shimohama1 ; Hirokazu


Shiraishi3 ; Masakatsu Motomura4 ; Tomihiro Imai5
1Neurology, Sapporo Medical University School of Medicine, Japan, 2Neurology, Sunagawa

City Medical Center, Japan, 3Neurology and Strokology, Nagasaki University Hospital,
Japan, 4Medical Engineering Course, Department of Engineering, Faculty of Engineering,
Nagasaki Institute of Applied Science, Japan, 5Disability Studies of Peripheral Nerve and
Muscles, Sapporo Medical University Graduate School of Health Sciences, Japan
Email Address: kazuna@sapmed.ac.jp

Introduction: Although Fisher syndrome and LEMS are classified into autoimmune
neuromuscular diseases, we cannot find the coincidence of them in a single patient in the
previous literature.

Results: A 70-year-old female was admitted to our hospital because of bilateral hand
numbness, double vision and unsteadiness which had developed 2 weeks. She had no history
of preceding infection and/or related illness. On admission, her condition was characterized by
mydriasis, extraocular muscle weakness, mild proximal muscle weakness and truncal ataxia.
In addition, the tendon reflexes were absent at rest, and improved with brief exercise. Motor
nerve conduction studies showed significantly low compound muscle action potentials
(CMAPs) and increments of more than 200% after brief exercise (post-exercise facilitation) in
several nerves. Sensory nerve conduction studies showed significantly low sensory nerve
action potential (SNAPs) of median and ulnar nerves, and normal SNAPs of sural nerves. We
also demonstrated the impairment of neuromuscular transmission in several muscles based on
abnormal decrements in the repetitive nerve stimulation (RNS) test and abnormal jitter in the
single-fiber electromyography (SFEMG). Both anti-GQ1b and GT1a ganglioside antibodies and
P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in her sera. Her
conditions improved spontaneously. She accomplished walking alone in six weeks, and
paralysis of the eye muscles recovered in five months. Median and ulnar SNAPs returned to
normal in accordance with recovery of clinical findings. On the other hand, low CMAPs with
post-exercise facilitation remained after complete recovery. Positron emission tomography and
gastrointestinal endoscopy did not show any malignancy.

Conclusion: Some electrophysiological studies revealed the impairment of neuromuscular


transmission using RNS and/or SFEMG in patients with Fisher syndrome. Also, it has been
known that autoimmune neurological phenotypes are diverse among patients with P/Q-type
VGCC antibodies. This may be the first case who showed clinical and laboratory
characteristics of Fisher syndrome and LEMS simultaneously.

232
PN008. ELECTROPHYSIOLOGICAL SUBTYPES OF GUILLAIN-BARRE
SYNDROME (GBS) CASES AT TERTIARY GENERAL HOSPITAL, MYANMAR

Kyawt Oo Kay Thi Htay*1 ; Kyaw Phyo Hlaing1 ; Khine Yee Mon1 ; Zin Phyu Tun1 ; Yan Lynn
Aung1 ; Moe Zaw Myint1
1Neurology, University of Medicine 1 Yangon, Myanmar.

Email Address: whitelucent@gmail.com

Introduction: Nerve conduction study (NCS) plays an important role in Guillain-Barre


syndrome (GBS) diagnosis and subtypes classification. GBS subtypes were reported to vary
with different geography. We aim to study the electrophysiological subtypes of GBS cases at
the biggest tertiary general hospital, Myanmar.

Methods: It was hospital-based prospective study at Neurology ward, Yangon General


Hospital over 1.5-year period from June 2017 to January 2019. At least 2 nerve conduction
studies (NCS) were done.

Results: A total of 50 patients with GBS were recruited, with mean age 32.66 ± 17.93 years.
Male-to-female ratio was 2.6:1. History of preceding illness was present in 58% (diarrhea in
8%, respiratory infection in 14% and unspecified fever in 13%). 32% had facial palsy, 36%
bulbar, 8% ataxia, and 6% had ocular involvement. Various antiganglioside antibodies were
detected in 44%. Good outcome was noted in 88% and poor outcome in 12%. 44% met criteria
for acute inflammatory demyelinating polyneuropathy (AIDP) whereas 56% were found to have
axonal type. Among axonal types, 40% were acute motor axonal neuropathy (AMAN) and 16%
were acute motor and sensory axonal neuropathy (AMSAN). 77% of GBS post Mycoplasma
infections were axonal subtypes. 57.1% of Campylobacter positive GBS were demyelinating
and the rest axonal types. All CMV positive and those with positive influenza serology were
electrophysiologically AMAN subtypes. Antiganglioside antibodies were positive in 27.3% of
AIDP, 55% of AMAN, 75% of AMSAN. 27.3% of AIDP and 60.7% of axonal subtypes were
associated with antiganglioside antibodies. Among poor outcome patients, AIDP was 83% and
axonal 17%.

Conclusion: The most common electrophysiological type among GBS patients in Myanmar
was axonal (56%), and axonal type was associated with positive anti-ganglioside antibodies (P
= 0.042). In contrast to other studies, our poor outcome patients were more of demyelinating
type.

233
PN009. PHOTOBIOMODULATION PROMOTING RECOVERY FROM
TRAUMATIC NERVE INJURY SECONDARY TO SUPRACONDYLAR
FRACTURE OF HUMERUS: A CASE REPORT

Lee Hwee Ming*1 ; Chung Tze Yang1 ; Tunku Sara Binti Tunku Ahmad Yahya2
1Rehabilitation Medicine, University Malaya Medical Center, Malaysia, 2Orthopedic, University

Malaya Medical Center, Malaysia.


Email Address: drmingness@gmail.com

Introduction: Photobiomodulation using low level laser therapy (LLLT-produced by low to mid-
powered coherent lasers ranging from 1 mW to 500 mW) has been researched extensively in
animal experiments and is shown to enhance nerve healing; however, few clinical studies
report effectiveness of photobiomodulation in humans. Recent understanding suggests that
LLRT-induced mitochondrial activity leads to increased ATP production, transcription factor
induction, pro and anti-inflammatory gene expression causing cell biostimulation, cytokine
modulation, growth factors release and increased tissue oxygenation ameliorating nerve injury.
This clinical case report aims to highlight clinical nerve injury recovery after LLLT in a 7-year-
old girl following a traumatic Gartland type 3 supracondylar fracture of the right humerus
sustained in May 2019. Clincal examination of patient NS suggested right median and ulnar
axonometsis evidenced by wasting of intrinsic muscles, thenar and clawing of all digits. After
undergoing Closed Manual Reduction and K-wiring there was minimal recovery of motor
function below the right wrist.

Results: Center based physio and occupational therapy was instituted together with home
based therapy - NMES (Neuromuscular electrical stimulation) immediately following injury and
surgical management. Typically 86-100% of supracondylar fracture associated nerve injuries
resolve spontaneously within 6 months with recovery mean time between 2 and 3 months and
in this case, there was some recovery (index finger extension) but stagnated at 5 months. At
University Malaya Medical Center (UMMC) she underwent 10 sessions of LLLT at the
Rehabilitation Medicine clinic, over a 2 week period resulted in remarkable improvement in
motor function especially wrist extension (beyond 90 degrees), extension of 3rd to 5th fingers
and 5th finger abduction.

Conclusion: Our findings suggest that a structured, intensive course of LLLT has the potential
to enhance nerve recovery. Further research is required to determine the duration, intensity
and dosage of LLLT to streamline prescription and clinical outcomes.

234
PN010. CRITICAL ILLNESS POLYNEUROPATHY

Luther Theng*1 ; Hasnawi Haddani2 ; Zulkifli3 ; Zen Ahmad4


1Neurology Department, Dr. H. Abdul Moeloek General Hospital Lampung Province,

Indonesia, 2Neurology, Dr. Mohammad Hoesin General Hospital Palembang, Indonesia,


3Anesthesiology, Dr. Mohammad Hoesin General Hospital Palembang, Indonesia, 4Internal

Medicine, Dr. Mohammad Hoesin General Hospital Palembang, Indonesia


Email Address: dr.luther.theng@gmail.com

Introduction: Critical Illness Polyneuropathy (CIP) is a frequent complication of critical illness,


acutely and primarily affecting the motor and sensory axons. CIP delays weaning from
mechanical ventilation, increases the length of stay at the intensive care unit, compromises
rehabilitation and may result in a lifelong loss of function and in a reduction in quality of life.
Greater than 50% of patients mechanically ventilated for more than 7 days will develop
electrophysiological abnormalities with 25-33% developing clinically overt weakness. Until
recently, no therapeutic measures had been proven to affect its incidence, and apart from
supportive treatment.

Results: A 52-year-old chinese lady presented to ED with shortness of breath due to SIRS et.
causa pneumonia was consulted to neurology department because of loss of conciousness.
After being intubated, patient awakened within stable condition. On neurological examination,
there were bilateral ptosis, bilateral ophthalmoplegia, flaccid over all extremities, decrease of
tendon reflexes and sensory were intact. There were leukocytosis and respiratory acidosis on
laboratory findings. Chest X-ray noticed bilateral minimal pleural effusion and cardiomegaly.
Bronchoscopy and Thorax CT-scan found inflammation. Abdominal ultrasound and head CT
scan were within normal limits. Lumbar puncture was not done due to rejection from the family.
On EDX findings, NCS found axonal loss pattern in motor and sensory, RNS was within
normal limits and needle EMG findings noted distal pattern of decreased recruitment with
denervation. After 18 months being hospitalised in ICU and using mechanical ventilator, the
patient died.

Conclusion: There is no direct treatment for CIP. CIP results in substantial morbidity,
mortality, costs and could likely be improved with earlier diagnosis and more timely
intervention. To achieve this goal, however, more awareness on the part of medical
establishment is needed, as well as further research into the causes of treatments for CIP.

235
PN013. CHARCOT-MARIE-TOOTH WITH TRANSIENT LEUKODYSTROPHY:
A CASE REPORT

Nguyen Kien Minh*1 ; Nguyen Tran Thanh Thao2, Do Ngoc Han2, Tran Thanh Truc1, Nguyen
Thi Thuy Van1, Ly Hien Khanh1
1Department of Infectious Diseases and Neurology, Children's Hospital 1, Vietnam,
2Department of Infectious Diseases and Neurology, Medical Genetics Institute, Vietnam.

Email Address: kienminhn@yahoo.com

Introduction: Charcot-Marie-Tooth (CMT), a chronic hereditary peripheral neuropathy, in


some rare cases is associated with transient leukodystrophy, making its diagnosis difficult.

Results: A 14-year-old boy, after playing football, had a sudden onset of crisis of stiff, numb
jaw and tip of tongue, causing drooling, difficulty swallowing and speaking; this numbness
spread to his arms. This crisis lasted for 1 to 2 hours; with the daily frequency of 2-3 attacks, at
fixed hours. Sometimes he had incontinence. Diagnosed as having epilepsy, he was admitted.
Symptoms self ameliorated 3 days later, then discharged. No symptoms recurrence during 4
months follow-up. Two years before admission, his feet were overturned while walking; he was
unable to sit straight and had to raise his buttocks. Family history was unremarkable.
Neurology examination: Normal sensory function, slight distal weakness of feet, absent tendon
reflexes, no pyramid signs, no bladder sphincter disorder. He had pes cavus, and he said he
had this deformity for 3 years. Laboratory tests and imaging diagnosis: Normal CBC, CRP,
ionogram, glycemia, CPK, and CSF, chest X-ray, and cardiac ultrasound. Brain MRI showed
high T2–weighted signal in splenium area of corpus callosum, in white matter behind bilateral
centrum ovale, parietal, and occipital regions. These lesions improved on follow-up brain MRI
performed 2 months later. Electrodiagnostic studies (EDx) highlighted sensorimotor
polyneuropathy, with low SNAP and CMAP of upper and lower limbs. Needle test showed
fibrillation, MUP polyphases. Gene panel (Diagsure) for typing CMT shows a homozygous
missence mutation (c589 G>C, p.Ala197Pro) in the GJB1 gene of chromosome X. This
mutation related to Charcot-Mare-Tooth, has not been reported in ClinVar.

Conclusion: Based on history, clinical symptoms, EMG, and gene panel, this is a typical case
of CMT1X causing peripheral symptoms and signs. Though transient central symptoms might
cause confusion, typing of CMT1X supported transient leucodystrophy.

236
PN014. DEMOGRAPHIC AND CLINICAL CHARACTERISTICS AMONG
PATIENTS WITH MYASTHENIA GRAVIS

Nurul Fajri*1; Syahrul2


1Department of Neurology Faculty of Medicine Syiah Kuala University, Dr. Zainoel Abidin

Public Hospital, Banda Aceh, Indonesia, 2Department of Neurology Faculty of Medicine Syiah
Kuala University, Dr. Zainoel Abidin Public Hospital, Banda Aceh, Indonesia
Email Address: neurology.nurul@gmail.com

Introduction: Myasthenia gravis is a rare, clinically heterogeneous, autoimmune disorder of


the neuromuscular junction characterized by fatigable weakness of voluntary muscles with an
average prevalence of 15-20 per 100,000 in the US population. In Indonesia, this disease is
still considered rare.

Methods: This study applied a retrospective cross-sectional study design. Data were gathered
from patients who were diagnosed and treated for myasthenia gravis admitted in the
neuroemergency of hospital during January to November 2019. The clinical information at
follow-up was also analyzed.

Results: 12 cases of myasthenia gravis were collected with females predominant (75%), and
one male (25%) with the average age of 30-40 years. The majority (75%) had onset < 35
years. The patients were from various districts, 75% referral from the district and 58% was
housewives. Clinically, 8% patients had ocular and 92% had the generalised form of
myasthenia. Out of all patients, 13.7% belonged to Osserman’s classification class 1-3 (33%)
and class 4-5 (67%). The severity of each crisis was calibrated in terms of Myasthenia Gravis
Foundation of America (MGFA) grading and it was noticed that 17% crisis was of milder
degree and 83% was of moderate to severe nature. The presence of thyroid disorder was
found in 1 of our patients (8.3%). Laboratory findings were 75% patients with the increase in
leukocyte count. The treatment was only 25% anticholinesterase, 50% anticholinesterase and
steroid, and 25% anticholinesterase and therapeutic plasma exchange (TPE). The hospital
stay was >7 days in 83% of patients.

Conclusion: 12 cases of myasthenia gravis were reported. These findings will be useful for
resource allocation and planning in health services. Many regions worldwide have few or no
epidemiological data on the myasthenia gravis, and more studies are required to create
estimation that are more accurate.

237
PN015. RITUXIMAB THERAPY IN POST THYMECTOMY REFRACTORY
MYASTHENIA GRAVIS: A CASE REPORT

Sari PM*1 ; Wijaya A2 ; Aryatama2 ; Djojoatmodjo S2 ; Liman J2 ; Freshimona R1


1General Practitioner, Premier Jatinegara Hospital, Jakarta, Indonesia, 2Neurologist, Premier

Jatinegara Hospital, Jakarta, Indonesia


Email Address: putrimaulia@gmail.com

Introduction: Myasthenia gravis (MG) is one of the autoimmune diseases caused by


disruption of synaptic transmission at the neuromuscular junction. An estimated 10–20%
patients with MG will still experience frequent relapses even after receiving the standard MG
treatment with pyridostigmine and immunosuppressants. Rituximab can be considered as a
therapeutic option in MG patients that show unsatisfactory response to standard therapy. Case
report: A female patient, 31 years old, was diagnosed with myasthenia gravis in 2017, and
tested positive for acetylcholine receptor antibody (AChR antibody). Upon diagnosis, her
Myasthenia Gravis Composite (MGC) score was 22. She was administered with pyridostigmine
4x60 mg per oral (PO) and methylprednisolone 3x16 mg PO. She was hospitalized three times
in two months due to MG relapses. In addition, the patient suffered an episode of myasthenic
crisis with mechanical ventilator support one year after diagnosis, after which she had
thymectomy. Post thymectomy, MGC score was 13, with medication pyridostigmine 4x60 mg
PO, methylprednisolone 1x8 mg PO, and azathioprine 1x200 mg PO. The patient was still
experiencing MG relapses and was admitted to hospital five times in two months. She was
then administered rituximab. After rituximab therapy, her MGC score was 4, she only had
relapse once within a year. Pyridostigmine 2x60 mg and methylprednisolone 1x8 mg PO were
still continued.

Results: A recent systematic review shows that rituximab administration gives satisfactory
results in 72% of MuSK patient, 30% of AChR antibody patients, and 44% in combined both
groups. In this patient, it was found that initial treatment with thymectomy surgery had
unsatisfactory improvement, and relapses were remained relatively frequent. However, with
additional treatment with rituximab, clinical improvement was significant.

Conclusion: Rituximab is an effective therapeutic choice for refractory MG patients who fail to
attain good disease control even after thymectomy.

238
PN018. THE CO-OCCURRENCE OF MYASTHENIA GRAVIS AND GUILLAIN-
BARRE SYNDROME: A CASE REPORT

Freshimona R*1 ; Wijaya A2 ; Aryatama2 ; Djojoatmodjo S2 ; Liman J2 ; Sari PM1


1General Practitioner, Premier Jatinegara Hospital, Jakarta, Indonesia, 2Neurologist, Premier

Jatinegara Hospital, Jakarta, Indonesia


Email Address: resistafreshimona@gmail.com

Introduction: Myasthenia gravis (MG) is an autoimmune disease of neuromuscular junction


with a prevalence of approximately 20 per 100,000 in the US and between 15 and 179 per
million worldwide. Guillain-Barre Syndrome (GBS) is an autoimmune-related disease leading
to generalized paralysis, characterized by acute severe ascending weakness. The co-
occurrence of myasthenia gravis (MG) is uncommon with a few reported cases in the literature.
Case report: A 34-year-old male with history of bilateral ophthalmoplegia, bilateral ptosis,
areflexia and lower motor neuron tetraparesis. The symptoms seemed to be fluctuating in the
early phase and progressive within 3 weeks. Patient was diagnosed with myasthenia gravis.
The Acetylcholine Receptor Antibody (AChR) was positive. The Pyridostigmine therapy was
administered but did not improve the symptoms fully. The repetitive nerve stimulation was
positive and nerve conduction study showed axonal degeneration. Cerebrospinal fluid (CSF)
analysis showed cytoalbuminergic dissociation. The patient was diagnosed with GBS Miller
Fisher variant. The clinical symptoms improved after being administered with intravenous
immunoglobulin (IVIg) therapy. A month later, the patient readmitted with worsening symptoms
that was triggered by respiratory tract infection. These symptoms improved after administration
of pyridostigmine, intravenous corticosteroid, and intravenous antibiotic.

Results: This patient admitted to our hospital with symptoms fit with relapsing myasthenia
gravis, and the course of the disease was also fit with Miller Fisher variant of Guillain Barre
Syndrome. In this case, the MG diagnosis was confirmed with the presence of AChR antibody
and positive repetitive nerve stimulation test. While, GBS diagnosis was supported by the CSF
analysis profile, and nerve conduction study results.

Conclusion: Although extremely rare, MG and GBS may present in the same patient with
various characteristics. This case is unique and rarely occur, additional research needs to be
conducted to understand the mechanism.

239
PN019. PNEUMONIA IN A PREGNANT WOMAN WITH EXCACERBATED
MYASTHENIA GRAVIS

Shierly Novitawati Sitanaya*1; Dodo Sambodo1


1Department of Neurology, Dr. Iskak General Hospital, Tulungagung, Indonesia.

Email Address: shierlynovitawati@yahoo.co.id

Introduction: Myasthenia gravis (MG) occurs in 10-20 persons per 1 million people per year
and in women twice as much as men. Pitfalls in MG therapy include numerous drugs and
conditions that could worsen muscle weakness, leading to myasthenic crisis. One of the most
common exacerbating conditions is infection, especially pneumonia. This case report will
discuss the management of exacerbated MG with pneumonia in a pregnant woman.

Results: Mrs. I, 35 years old and 12 weeks pregnant, visited Emergency Room of Dr. Iskak
General Hospital with the chief complaint of shortness of breath for two days. There were
fever, productive cough, and nausea. A week prior, she forgot to take her pyridostigmine.
There was history of dyspnea in her first pregnancy. Her vital signs showed tachypnea and
fever, peripheral saturation was normal, and patient was fully alert. There was neither
weakness in extremities nor difficulty in swallowing. Remarkable laboratory results were
leucocytosis and neutrophilia. She was diagnosed as pneumonia and exacerbated MG.
Treatments during hospitalization included cefadroxil 2x500 mg, pyridostigmine 6x60 mg,
paracetamol 3x500 mg, O2 nasal cannule 3 L/m, Ventolin nebulization, and ambroxol 3x30 mg.

Conclusion: Mrs. I’s clinical features pointed towards pneumonia, but MG exacerbation
cannot be fully excluded since pneumonia can worsen the muscle weakness. Therefore, the
main purposes of treatment should be to eradicate the bacteria and prevent MG from
worsening to myasthenic crisis. Cefadroxil was chosen since it is not teratogenic and does not
induce myasthenic crisis. Pyridostigmine was given twice the normal dose, which was in the
safe range for not causing microcephaly. In short, dyspnea in a MG patient should be
assessed like any other dyspnea case with several differential diagnoses instead of focusing to
the MG itself. Treatment of this patient should consider the teratogenic effect to the pregnancy
and the exacerbating effect to the MG.

240
PN020. ACUTE FLACCID PARALYSIS: GUILLAIN-BARRÉ SYNDROME
MIMICS

Si-Lei Fong*1 ; Mohamad Imran Idris1 ; Stefanie Kar-Yan Hung2 ; Benjamin Han-Sim Ng2 ;
Kheng-Chiew Chooi3 ; Shireene Ratna Vethakkan3 ; Cheng-Yin Tan1
1Division of Neurology, Department of Medicine, University Malaya Medical Centre, Malaysia,
2Department of Neurology, Kuala Lumpur General Hospital, Malaysia, 3Division of

Endocrinology, Department of Medicine, University Malaya Medical Centre, Malaysia


Email Address: fongsilei@gmail.com

Introduction: Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid


paralysis. However, clinicians should not miss the other causes of acute flaccid paralysis which
may mimic GBS.

Results: We present 3 cases of flaccid areflexic paralysis which presented acute/subacutely


resembling GBS. Case 1: A 29-year-old lady presented with 2-week history of bilateral lower
limb weakness with left hip pain for 3 years. Investigations revealed hypercalcaemia,
hypophosphataemia, elevated ALP, vitamin D deficiency, hyperparathyroidism with right
parathyroid adenoma from parathyroid ultrasonography and scintigraphy. She had plasma
exchange for presumed GBS, but her lower limb weakness worsened and progressed to
involve upper limbs and facial muscles. Her weakness improved after parathyroidectomy and
the diagnosis was revised to hyperparathyroidism-related polyneuropathy. Case 2: A 28-year-
old lady with underlying epilepsy and intellectual disability presented with progressive
weakness of extremities over 3 weeks after prolonged fasting. Her weakness did not improve
after treatment with immunoglobulin for GBS. Investigations showed positive urine
porphobilinogen and raised urine delta-aminolaevulinic acid. A diagnosis of acute intermittent
porphyria was considered most likely and her weakness improved after continuous glucose
loading. Case 3: A 52-year-old lady who had bariatric surgery presented with bilateral lower
limb weakness over 4 weeks after recurrent bouts of vomiting and excessive weight loss.
Investigations revealed vitamin D deficiency, hypoalbuminaemia, hyperhomocysteinemia, low
folate and low normal serum thiamine. As her weakness continued to progress, a diagnosis of
acute nutritional neuropathy was considered more likely. Her weakness improved following
multivitamins replacement. All 3 patients had normal CSF analysis except Case 1 who showed
cyto-albuminologic dissociation. Sensorimotor axonal polyneuropathy was the electrodiagnosis
demonstrated in all the cases.

Conclusion: Clinicians should have high index of suspicion when treating acute flaccid
paralysis with progressive disease and null response to immunotherapy as the neurology is
reversible with treatment of the underlying cause.

241
PN021. A CASE SERIES OF CRYOGLOBULINEMIA WITH DIFFERENT
CLINICAL PRESENTATIONS AND DIFFERENT NERVE CONDUCTION
STUDIES (NCS) FINDINGS

Soe Thinzar Oo*1 ; Htet Htet Lin1 ; Khine Yee Mon1 ; Yan Lynn Aung1 ; Ohnmar1
1Neuromedical Department, University of Medicine 1, Yangon, Myanmar

Email Address: soethinzaroo@gmail.com

Introduction: Neuropathy in cryoglobulinemia can be due to hyperviscosity (type I) or non


obstructive immune complex mediated inflammatory vasculitis (type II and III).

Methods: Case series: Our first patient was a 72-year-old gentleman with 5-month duration of
severe asymmetrical neuropathic pain and rapidly progressive weakness in right peroneal and
bilateral ulnar distribution associated with significant weight loss and pain, but no sphincter and
autonomic dysfunction. He denied fever, arthritis, Raynaud’s phenomenon, rash and edema.
His NCS revealed bilateral ulnar and right peroneal neuropathy. Left sural nerve biopsy
revealed vasculitis, and cryoglobulin was found to be positive. Myeloma panel was suggestive
of Kappa monoclonal gammopathy with normal flow cytometry and bone marrow. We
concluded it as cryoglobulinemic vasculitis with Kappa light chain MGUS presenting with
mononeuritis multiplex. Second patient was a 56-year-old man, HCV treated one year ago with
undetectable viral load currently, and had generalized Myasthenia Gravis, presented with
burning sensation of bilateral feet and erythematous macules, blanching papules on ankles,
arms with normal power and reflexes. Nerve conduction study was normal and supported the
clinical diagnosis of small fiber neuropathy due to cryoglobulinemia with treated HCV. Third
case, a 70-year-old lady, presented with tingling sensation of both lower limbs with impaired
sensation of up to ankles, distal toes weakness (3/5) and absent ankle reflexes for 2 months.
Her NCS showed distal sensorimotor axonal polyneuropathy. She was found to be anti-HCV
positive and cryoglobulinemia with viral load of 1 × 106 IU/ml. All our patients had no history of
exposure to heavy metals, metabolic, infection screening and ENA, ANCA, anti-CCP were
negative and cryoglobulin was positive.

Conclusion: Nerve conduction study can add to the clinical for diagnosis of various types of
polyneuropathy (mononeuritis multiplex, large fiber peripheral neuropathy, small fiber
neuropathy) as depicted in our cases.

242
PN022. TRANSTHYRETIN FAMILIAL AMYLOID POLYNEUROPATHY IN
MULTI-ETHNIC MALAYSIANS

Low Soon Chai*1 ; Tan Cheng Yin1 ; Nor Ashikin binti Md Sari1 ; Azlina binti Ahmad Annuar2 ;
Wong Kum Thong3 ; Lin Kon-Ping4 ; Nortina Shahrizaila1 ; Goh Khean Jin1
1Medicine, University of Malaya, Kuala Lumpur, Malaysia, 2Biomedical Science, University of

Malaya, Kuala Lumpur, Malaysia, 3Pathology, University of Malaya, Kuala Lumpur, Malaysia,
4Neurology, Taipei Veterans General Hospital, Taiwan

Email Address: lowsc@ummc.edu.my

Introduction: Familial amyloid polyneuropathy (FAP) is a rare autosomal dominant peripheral


neuropathy. We report a case series of Malaysian FAP patients.

Methods: Patients with definite (genetically confirmed) FAP seen at the University of Malaya
Medical Centre between 2008 till 2019 were included in the series. The patients’ demographic
and clinical features and investigations including nerve conduction studies and
electromyography (NCS/EMG), echocardiography, sural nerve biopsy and hATTR mutation are
reported.

Results: A total 21 index cases were seen. Nineteen (90%) were ethnic Chinese, one ethnic
(5%) Malay and one (5%) ethnic Indian; Fourteen (67%) were men. Mean age of onset of
symptoms was 56.1 ± 10.5 years (range: 31-73). Nineteen (90%) patients presented with
numbness and weakness of the limbs while two had postural hypotension. Fifteen (71%)
patients had positive family history. NCS/EMG showed sensorimotor polyneuropathy in 19
(90%) patients; fifteen (71%) showing axonal neuropathy. Amyloid deposits were seen on
nerve biopsy in ten patients and rectal, leptomeningeal and endomyocardial biopsies in 1
patient respectively. The latter was diagnosed as familial amyloid cardiomyopathy (FAC).
Sixteen patients (76%) had abnormal cardiac echocardiogram with amyloid deposits. Fifteen
Chinese patients (71%) carried the Ala97Ser hATTR mutation while the FAC patient has
Asp39Val mutation. Our Malay patient had Glu54Lys mutation and Indian patient had Gly67Val
mutation. Two patients carried Val30Met mutation. Autonomic function test was abnormal in all
7 patients in whom they were carried out.

Conclusion: FAP in Malaysians is seen mainly among ethnic Chinese, in whom Ala97Ser may
be a common mutation. This is also the reported “hotspot” mutation for FAP reported in Taiwan
but not in China. Similar to Taiwan, Chinese-Malaysians are descended from immigrants from
Southern China. Similar to Taiwanese patients, our FAP patients with Ala97Ser mutation,
present with late-onset progressive sensorimotor polyneuropathy and cardiac involvement was
common.

243
PN023. CHILDHOOD-ONSET DEMYELINATING POLYNEUROPATHY:
CHALLENGES IN DIFFERENTIATING ACQUIRED FROM GENETIC DISEASE

Toh Tsun Haw*1 ; Lau Kar Foo2 ; Tay Chee Geap3 ; Chung Tze Yang4 ; Nortina Shahrizaila1 ;
Tan Cheng Yin1
1Neurology, University Malaya Medical Centre, Malaysia, 2Neurology, University of Tunku

Abdul Rahman, Malaysia, 3Paediatric Neurology, University of Malaya, Malaysia,


4Rehabilitation Medicine, University of Malaya, Malaysia

Email Address: yuy987@hotmail.com

Introduction: Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) can be


misdiagnosed for the more common genetic neuropathies such as Charcot-Marie-Tooth (CMT)
disease. We present a case of childhood-onset demyelinating polyneuropathy who was initially
diagnosed as CMT before a revised diagnosis of CIDP was made.

Results: A 14-year-old boy with bilateral pes cavus presented with progressive history of
ataxic gait, generalized areflexia and proprioceptive sensory loss. Nerve conduction studies
showed demyelinating features including markedly slow motor conduction velocities and
prolonged distal motor latencies resembling CMT1. Despite the absence of a family history of
genetic neuropathies, a diagnosis of CMT1 was considered most likely. The patient
represented two years later with an acute onset of worsening instability and muscle weakness.
A detailed history revealed functional improvement following the last presentation along with
two separate episodes of exacerbations suggesting a relapsing-remitting form of neuropathy.
Cerebrospinal fluid analysis showed cytoalbuminergic dissociation. Nerve ultrasound
demonstrated enlarged peripheral nerves, particularly in the proximal and non-entrapment
sites. Genetic testing was negative for known mutations in common CMT genes. A course of
intravenous immunoglobulin resulted in clinically significant improvement.

Conclusion: This report highlights the diagnostic challenges in childhood-onset demyelinating


neuropathies and the importance of not missing a potentially treatable immune-mediated
neuropathy.

244
PN024. THE PROFILE OF CEREBROSPINAL FLUID ANALYSIS IN PATIENTS
WITH GUILLAIN-BARRÉ SYNDROME

Wikan Pambudi Nuroso*1 ; Indarwati Setyaningsih1 ; Kusumo Dananjoyo1 ; Indra Sari


Kusuma Harahap1
1Departement of Neurology, Faculty of Medicine, Public Health and Nursing, Universitas

Gadjah Mada, Indonesia


Email Address: wikanpambudi@gmail.com

Introduction: Guillain-Barré Syndrome (GBS) is a clinical syndrome of an acute inflammatory


polyradiculoneuropathy with ascending muscle weakness. One of the criteria to diagnose GBS
is including the cerebrospinal fluid examination. Several parameters had been analysed from
this examination. During the acute phase of GBS, characteristic findings on CSF analysis
include albuminocytologic dissociation, which is an elevation of CSF protein (>0.55 g/L) without
an elevation of white blood cells. In this study, we describe the profile of cerebrospinal fluid
analysis in patients with GBS.

Methods: Thirty-three adult patients with GBS were retrospectively analysed from medical
record in Dr.Sardjito General Hospital, Yogyakarta, collected from January 2016 to September
2019.

Results: The age of patients ranged from 18 to 77 years. The proportion of male was higher
than female (21 vs 12 patients). The cerebrospinal fluid analysis found that the mean white
blood cells count was 17,82 ± 44,965 cells/µL, range from 0 to 200 (normal result 0-5 cells/µL).
The mean protein count was 0,13242 ± 0,147098 g/dL, ranged from 0,010 to 0,800 (normal
result 0,02-0,05 g/dL). The albuminocytologic dissociation result was found in 21 of 33 patients
(63,64%). The mean of glucose level was 91,4848 ± 22,439 mg/dL, ranged from 68 to 188
(normal result 50-80 mg/dL).

Conclusion: Cerebrospinal fluid analysis showed the elevation of white blood cells count,
protein count and glucose level in GBS patients. More than half of the results showed
albuminocytologic dissociation.

245
PN025. PECULIARITIES OF DIFFERENTIAL DIAGNOSIS AND TREATMENT
OF UPPER LIMB TUNNEL NEUROPATHIES

Yury Merkulov*1 ; A.M. Magomedova2 ; A.A. Pyatkov3 ; D.M. Merkulova2


1Medical Rehabilitation, Moscow Centre for Research and Practice in Medical Rehabilitation,

Restorative and Sports Medicine, Russia, 2Neurology, N.A. Semashko Central Clinical Hospital
No2 JSC Russian Railways, Russia, 3Neurology, Moscow Centre for Research and Practice in
Medical Rehabilitation, Restorative and Sports Medicine, Russia
Email Address: 4181220@gmail.com

Introduction: It is well-known that numbness in hands is associated with peripheral nerves


compression or “tunnel neuropathies” (TN). According to numerous authors, TN account for up
to 40% of all neurological diseases. However, TN diagnosing in outpatients is practically
uncommon.

Methods: 597 cases of hand numbness were selected on analysis of 2495 outpatient records
in two remote ambulatories during 2016-2017, with the share of TN being 0.7% of all initial
diagnoses. 92 patients with mild to moderate TN symptoms were included in the study, and
divided into main (n=50) and control (n=42) groups. Both groups received B vitamins and
thioctic acid as baseline medications. Ipidacrine (Ipigrix by Grindex, Latvia) was added in the
main group: 15 mg I.M. q.d. for a week and 20 mg p.o. q.d. for 3 more weeks. Thorough
clinical and ENMG examinations were carried out before and after treatment.

Results: All initial diagnoses were associated with cervical osteochondrosis, including
radiculopathy, lacking any signs of its objectification. The Kruskal-Wallis test revealed sample
homogeneity regardless of the diagnoses, and no statistically significant inter-group
differences by clinical or ENMG markers of TN were originally detected. A 4-week follow-up
analysis compared with the baseline results demonstrated that the control group had a positive
trend, but no significant differences before and after treatment. Meanwhile, in the main group,
additionally receiving ipidacrine, there was a statistically confirmed improvement of
hypoesthesia, paresthesia, provocative test results, SNAP amplitudes and conduction time
within carpal and pronator TN, as well as CMAPs in the cubital TN (p<0.05).

Conclusion: The study describes typical "diagnostic masks" of TN, traditionally associated
with osteochondrosis, the role of which in the occurrence of hand numbness is greatly
exaggerated. Furthermore, the results suggest the efficacy of a short course of ipidacrine in
the complex treatment of patients with mild and moderate TN.

246
PN027. SYMPATHETIC SKIN RESPONSE IN DIABETIC NEUROPATHY WITH
AND WITHOUT PERIPHERAL ARTERIAL DISEASE

Nurul Fadli*1 ; Manfaluthy Hakim1 ; Ahmad Yanuar Safri1 ; Dono Antono2


1Neurology, Faculty of Medicine University of Indonesia, Indonesia, 2Cardiovascular, Faculty of

Medicine University of Indonesia, Indonesia.


Email Address: kick.fadli@gmail.com

Introduction: Diabetic neuropathy (DN) is a common complication of diabetes. Symptoms can


be tingling, pain or numbness in the lower limb. Peripheral arterial disease (PAD) is also a
complication of diabetes which can cause pain in the lower limb. The presence of DN and PAD
affect each other, resulting in worsening of patient condition. Autonomic nervous system
disturbance, shown by sympathetic skin response (SSR) abnormalities, is an independent
finding that appear early in patient with PAD.

Methods: A cross sectional study with 46 DN patients based on clinical and nerve conduction
study (NCS) examination. Patient were evaluated with toe-brachial index (TBI) and SSR then
were categorized to PAD and non-PAD group.

Results: In our study, the mean age of the study population was 63,09 years (±9,98). The
mean duration of diabetes in the study population was 13,57 years (±10,43). Most of the
patients had poorly controlled diabetes with a median HbA1C of 7,35 (min-max: 5,6 – 12,2).
Twenty two patients (47,8%) have PAD. Ten patients (45,45%) in PAD group and 8 patients
(33,33%) in non-PAD group had absent SSR in the lower limb. There was no statistically
significant association between SSR abnormalities and the presence of PAD (p = 0,40).

Conclusion: SSR abnormalities are more common in DN with PAD group.

247
PN028. CLINICAL AND ELECTROPHYSIOLOGICAL PROFILE OF ARSENIC
NEUROPATHY

Seshagiri D V*1 ; Nagappa M1 ; Safwan A1 ; Ganaraja VH1 ; Sinha S1


1Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), India

Email Address: drgiri061@gmail.com

Introduction: Arsenic toxicity though rare can affect multiple organs including peripheral
nerves. We aim to describe the clinical and electrophysiological characteristics of two patients
with arsenic neuropathy

Methods: A retrospective chart review of two patients with neuropathy and markedly elevated
arsenic levels in blood and hair.

Results: Patient 1, a 33-years-old male, developed acute gastroenteritis after consuming


locally made liquor, followed by febrile encephalopathy, which improved over one week. Two
weeks later, he developed painful ascending paraesthesias involving legs up to knees and
hands up to wrists. This was associated with difficulty in walking independently. The patient
also used to smoke illicit imported cigarettes. He was provisionally diagnosed to have chronic
inflammatory demyelinating polyradiculoneuropathy (CIDP). Examination during the fourth
week of illness revealed Mee’s lines, hypotonia, and weakness of all limbs distally, global
areflexia, and graded sensory loss to all modalities of sensation. Electrophysiological testing
showed reduced motor conduction velocities and absent sensory potentials suggesting
demyelinating neuropathy. The patient was treated with d-penicillamine. At three month follow
up he improved significantly and could resume his job. Patient 2, a 35-year-old gentleman,
painter by occupation, developed painful paraesthesias of distal extremities, imbalance while
walking in the dark, and slippage of footwear of two months duration. He had a febrile illness
10 days prior to the onset of neurological symptoms. He was provisionally diagnosed to have
immune-mediated neuropathy. Examination revealed Mee’s lines, generalised
hyperpigmentation, raindrop pigmentation, and palmoplantar hyper-keratosis. He had normal
power, but global areflexia, impaired kinesthetic sensation in all limbs and sensory ataxic gait.
Electrophysiological testing showed reduced amplitude of sensory potentials suggesting
sensory axonopathy

Conclusion: Neuropathy arising from arsenic toxicity can have acute/subacute/chronic course
and may be mistaken for other acquired inflammatory neuropathies. A high index of suspicion
is required to make an early diagnosis and to initiate appropriate preventive measures.

248
PN029. CLINICAL AND ELECTROPHYSIOLOGICAL PROFILE OF PATIENTS
WITH GUILLAIN-BARRE SYNDROME WITH CONDUCTION BLOCKS.

Praveen Sharma P*1 ; Madhu Nagappa1 ; Donaparthi Venkata Seshagiri1 ; Sumanth


Shivaram1 ; Sanjib Sinha1 ; Arun B Taly1
1Neurology, National Institute of Mental Health and Neurosciences, India

Email Address: drpraveensharma04@gmail.com

Introduction: Clinical data is limited in patients with Guillain Barre syndrome (GBS) who show
conduction block (CB) on nerve conduction study (NCS). We aim to describe the clinical and
electrophysiological profiles of GBS with CB.

Methods: Between 2017 and 2020, 111 patients fulfilling the National Institute of Neurological
Disorders and Stroke (NINDS) criteria for GBS and Brighton level of certainty one or two were
seen in a single neurology unit. NCS of three motor (median, ulnar and common peroneal
(CP)) and three sensory (median, ulnar and sural) nerves were performed. Definite and
probable CBs were defined as proximal/distal latency of 20% of lower limit of normal (LLN).

Results: Mean interval from symptom-onset to NCS was 9.4±6.5 days. Twenty patients had
CB (one nerve: 15, two nerves: 4, three nerves: 1). This included probable CB in median (n=3),
ulnar (n=2) and CP (n=4) nerves, and definite CB in median (n=5), ulnar (n=6) and CP (n=6)
nerves. Electrophysiological subtypes included demyelinating (n=11), axonal (n=4) and
equivocal (n=5). Demographic and clinical features included M:F of 3:1, mean age of
38.5±19.9 years, facial weakness (n=8), ophthalmoparesis (n=1), respiratory muscle weakness
(n=2) and antecedent infection (n=12). At the time of peak neurological deficits, the mean
Medical Research Council (MRC) sum score was 35.4±14.9 and Hughes disability score
(HDS) was 3.5±0.8. The mean duration of hospital stay was 14.7±15.9 days. At the time of
discharge, the mean MRC sum score was 39.8±15.2 and mean HDS was 3.1±0.7.

Conclusion: We report CBs across non-entrapment sites in both demyelinating and axonal
sub-types of GBS. Interruption of saltatory conduction due to distortion of paranodal myelin
leads to reversible conduction failure and rapid recovery as noted in the present study.

249
PN030. RECURRENT GUILLAIN BARRE SYNDROME: CLINICAL AND
ELECTROPHYSIOLOGICAL PROFILES.

Praveen Sharma P*1 ; Madhu Nagappa1 ; Donaparthi Venkata Seshagiri1 ; Sumanth


Shivaram1 ; Sanjib Sinha1 ; Arun B Taly1
1Neurology, National Institute of Mental Health and Neurosciences, India

Email Address: drpraveensharma04@gmail.com

Introduction: Guillain-Barre syndrome (GBS) is an autoimmune disease characterized by


rapidly progressive ascending flaccid paralysis. Recurrence of GBS has been rarely reported.
This study aims to describe the clinical and electrophysiological characteristics of a group of
patients with recurrent GBS.

Methods: The clinical features and nerve conduction studies (NCS) of patients with recurrent
GBS seen in a single neurology unit study from 2017 to 2020 were analyzed. These patients
met the National Institute of Neurological Disorders and Stroke (NINDS) criteria and had
Brighton level of certainty of 1 or 2.

Results: Nine out of 111 patients seen during the study period had recurrent GBS. One patient
had 3 episodes while the rest had 2 episodes each. The interval between episodes ranged
from 4 years to 17 years. Demographic and clinical features included M:F ratio of 2:1. mean
age of 35.4±12.9 years, facial weakness (n=3), and respiratory muscle weaknees (n=1). Four
patients had antecedent illnesses (fever: 2, respiratory infection: 1 and acute gastroenteritis:
1). At the peak of illness, the mean Medical Research Council (MRC) sum score was 25.8±18
and mean Hughes disability score (HDS) was 3.5±0.9. The median duration of hospital stay
was 11 days. MRC sum score at discharge was 31.5±0.7 and HDS was 3.3±0.7.
Electrophysiological sub-types based on Hadden’s criteria included demyelinating (n=6),
equivocal (n=2) and inexcitable (n=1). Based on Rajabally’s criteria, the electrophysiological
sub-types included demyelinating (n=5), axonal (n=2), equivocal (n=1) and inexcitable (n=1).
One patient had definite conduction block while another had probable conduction block.

Conclusion: We describe nine patients with recurrent GBS. There were long intervals
between the episodes. All patients were asymptomatic in between the episodes and none
required long-term alternative immunomodulatory therapy. NCS showed predominantly
demyelinating pattern however conduction block was not common. All patients recovered
following treatment with mild residual deficits.

250
PN031. MEDICINAL IMPORTANCE AND HEALTH BENEFICIAL EFFECT OF
TANGERETIN: THERAPEUTIC ROLE IN NEURODEGENERATION AND
INFLAMMATORY RESPONSES

Dinesh K Patel*1
1Shalom Institute of Health and Allied Sciences, Prayagraj, India

Email Address: dineshpatel23shiva@gmail.com

Introduction: Citrus flavonoids are important polyphenolic compounds having huge biological
potential. Therapeutic potential of various flavonoidal compounds in obesity, cancer, diabetes
and Alzheimer’s has been well documented in the scientific literature. Demand of natural
products and their derived products including dietary supplements has been increased in the
medical field. Alzheimer’s and Parkinson's disease are most common Neurodegenerative
disorders affecting huge population in the world. Prevention and treatment of
neurodegenerative disorders by use of natural products is warranted. Tangeretin is important
polymethoxylated flavonoids derived from the peel and other parts of Citrus fruits. Tangeretin
has been proven for their antiinflammatory and neuroprotective effects in several disorders.

Methods: To know the therapeutic importance of tangeretin in the medicine, various scientific
databases have been searched and evaluated for their role in neurodegeneration and
inflammatory responses. Medicinal importance and therapeutic effect of tangeretin in
neurodegeneration and related disorders have been analyzed through different scientific
database. Therapeutic importance of tangeretin in neurodegeneration and inflammatory
disorders has been analyzed in the present study and correlated with other biological potential.

Results: From the analysis of the scientific databases of tangeretin it was found to have
chemopreventive and neuroprotective potential. Scientific data analysis revealed that
tangeretin reduced expression of inflammatory mediators. Experimental databases suggest
that tangeretin is an effective candidate for prevention and treatment of neuroinflammation and
dementia. Other experimental study suggests that tangeretin decreased nitric oxide.
Tangeretin is also effective in colitis through suppression of IL12 and TNF-α expression and
nuclear factor kappa-B activation.

Conclusion: From the analysis of the scientific research work it was found that tangeretin
have potential medicinal value for the treatment of neurodegeneration and inflammatory
medicated disorders.

251
PN032. PERIPHERAL NERVE HYPEREXCITABILITY SYNDROME-CASE
SERIES FROM TERTIARY NEUROSPECIALITY CENTRE

Dr Mayura P Dhonde*1; Dr Pramod V Dhonde2


1Neuroelectrophysiology, Dhonde Neurocare Centre, Nanded, India, 2Neurology, Dhonde

Neurocare centre, India


Email Address: Mayuradhonde@gmail.com

Introduction: Peripheral nerve hyperexcitability (PNH) syndrome is a wide spectrum disorder


characterized by spontaneous and continuous muscle fiber activity of peripheral nerve origin.
We are presenting five cases with this rare syndrome.

Methods: We studied five cases of PNH syndrome cases at our single neurocare centre from
2016-2020. There were 4 males with age 15, 35, 55, 40 years and one female 32 years. All
patients were examined clinically and evaluated using nerve conduction and EMG on Viking
Quest EMG machine with detailed evaluation.

Results: The common presentation feature were severe pain in lower limbs (VAS score 8/10)
and insomnia in all patients. Fasciculations seen in 4 patients with grade 3 severity and grade
1 in female patient. Autonomic involvement was seen as excessive sweating, tachycardia and
hypertension in two patients. Suicidal tendencies due to severe pain were seen in three cases.
Nerve conduction revealed repetitive compound muscle action potential (CMAP) and repetitive
F waves were seen in all patients. On electromyography (EMG), doublets, triplets in two
patients, myokymic discharges in one patient, and neuromyotonic discharges seen in one
patient. Laboratory testing revealed three patients were CASPR2 positive and one LGI1
positive. Female patient report was not available. Thymoma was not seen in any case. Four
patients were treated with steroids followed by mycophenolate mofetil and all showed
significant improvement over 3 months but required treatment for at least two years. Three
patients went into remission till last follow up. Three were drug free till date. Two patients were
on steroids currently.

Conclusion: PNH is a syndrome with most common presentation of lower limb pain, then
suicidal tendencies and fasciculations with typical neuroelectrohysiological features with good
response to immunomodulation. We did not find thymoma in our case series as this is a short
case series.

252
PN033. CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY
IN HIV INFECTED PATIENT RESPONDING TO PLASMAPHERESIS: A CASE
REPORT

Rakhian Listyawan*1 ; Yudiyanta1 ; Tommy Rachmat1 ; Rinaras Anggraini1


1Neurology Department, Faculty of Medicine, Public Health, and Nursing Universitas Gadjah

Mada, Yogyakarta, Indonesia


Email Address: rakh.list@gmail.com

Introduction: Chronic Inflammatory Demyelinating Polyradiculoneuropathy or CIDP is a


neurological disorder characterized by chronic progressive weakness and impairment of
sensory function of the limbs and arms, accompanied by decreased tendon reflexes. CIDP can
occur in the setting of HIV, as are other immune-mediated neurologic disorders including a
wide spectrum of neuropathies.

Results: Male, 27 years old, with thick and tingling complaints in both feet and hands followed
by ascending, symmetrical, progressive weakness of all four limbs for 3 months. Physical
examination showed decreased muscle strength, areflexia, and hypoesthesia in the distal four
extremities. In addition, there was oral candidiasis in the patient so there was a suspicion of an
immunodeficiency condition. Repeat HIV antibody testing returned positive. The ENMG results
shows demyelinating and axonopathy in peripheral nerve. Six sessions of plasmapheresis
measures were performed in this patient as therapy. Muscle strength and complaints of
numbness improved with therapy, patient was expected to continue to improve with intensive
rehabilitation after discharge. The diagnosis of CIDP meets the EFNS clinical criteria and
EFNS definite electrophysiologic criteria. This case can be distinguished from HIV-related
distal sensory polyneuropathy because of motor nerve involvement.

Conclusion: CIDP may occur in early stages of HIV infection, is probably autoimmune in
etiology, clinically shows sensorimotor neuropathy and nerve conduction studies show
demyelination. This case report shows HIV-infected patients with CIDP displayed a
progressive course of sensorimotor involvement. Plasma exchange showed a good
therapeutic response in this case.

253
PN034. FACIAL PARALYSIS IN RAMSAY HUNT SYNDROME: A CASE
REPORT, LITERATURE REVIEW ON THERAPY AND PROGNOSIS

Agus Nugroho Andhi Saputro*1 ; Sekar Satiti1 ; Atitya Fithri Khairani1 ; Tommy Rachmat1
1Neurology, Neurology Department Gadjah Mada University, Indonesia

Email Address: nugroho.andhi85@gmail.com

Introduction: Ramsay hunt syndrome is a rare disease caused by infection by varicella zoster
virus. The disease causes facial paralysis and painful ear rash. Proper therapy is required for a
good prognosis.

Results: A 28-year-old female came to hospital with complaints of right-sided facial weakness
and right ear pain, hearingloss, tinnitus and vertigo. On physical examination we found 7th
cranial nerve palsy, vesicle on right periauricular, and horizontal unidirectional nystagmus.
Laboratory examination results show IgM anti-HSV1 1.9 U/ml, IgG anti-HSV-1 10.4 U/ml, IgM
anti-HSV-2 1.7 U/ml, IgG anti-HSV-2 6.7 U/ml were all within normal limits. Brain MRI with
contrast showed right internal otitis that has affected the right facial nerve. Patient was
diagnosed with Ramsay Hunt Syndrome and was given steroid, betahistine and acyclovir
therapy. Patient was hospitalized for nine days. Dizziness, tinnitus and ear pain improved fully.
Facial weakness slightly improved. Discussion: Several therapeutic formulas are proposed to
be the most appropriate therapy in the treatment of Ramsay Hunt syndrome. This paper
discusses a comparison of several of these therapeutic formulas in order to get the best
outcome.

Conclusion: The combination of steroids and acyclovir reach better recovery rates than
steroids in monotherapy despite many variations in formula dosage.

254
PN035. TH17 PATHWAY-RELATED CYTOKINE ABNORMALITIES IN THE
PLASMA AND CEREBROSPINAL FLUID IN GUILLAIN BARRÉ SYNDROME
(GBS): A CORRELATION ANALYSIS

Monojit Debnath*1 ; Madhu Nagappa2 ; Debprasad Dutta1 ; Rahul Wahatule2 ; Arun B Taly2
1Human Genetics, National Institute of Mental Health and Neurosciences, India 2Neurology,

National Institute of Mental Health and Neurosciences, India


Email Address: monozeet@gmail.com

Introduction: Guillain Barré Syndrome (GBS) is a monophasic immune-mediated disease of


the peripheral nervous system, often triggered by antecedent gastrointestinal or respiratory
infection. Compelling evidence suggests inflammatory cytokines as the critical drivers of the
onset and progression in GBS. Elevated levels of pro-inflammatory cytokines were reported
both in the plasma and cerebrospinal fluid (CSF) of patients with GBS. However, whether there
exists a correlation between the levels of proinflammatory cytokines in plasma and CSF is
inadequately explored.

Methods: In this study, we recruited 37 (21 male and 16 female) patients with GBS from the
emergency services of the National Institute of Mental Health and Neurosciences, Bangalore,
India. Mean age of the patients was 36.65 ± 12.2 years. We analysed the levels of four Th17-
pathway related cytokines, IL-6, IL-17A, IL-22 and IL-23, in both the plasma and CSF of
patients with GBS by Multiplex Suspension Assay. The levels of these cytokines were
correlated between plasma and CSF and also with various clinical features and
electrophysiological subtypes.

Results: Spearman’s correlation analysis suggests that there was no correlation in the levels
of the Th17-pathway related cytokines, IL-6 (p = 0.11), IL-17A (p = 0.85), IL-22 (p = 0.45) and
IL-23 (p = 0.76) between plasma and CSF in patients of GBS.

Conclusion: Elevated levels of Th17 pathway related cytokines were observed both in the
plasma and CSF in our earlier studies. Immune molecules in the CSF play pathogenic roles in
various nervous system disorders. CSF analysis also plays important role in establishing the
diagnosis of GBS. Though Th17 pathway-related cytokines are upregulated in both the plasma
and CSF, however, they do not show any correlation. This may suggest that immune
molecules in plasma and CSF may drive pathogenetic processes in GBS independently.

255
PN036. LOSS OF FUNCTION MUTATION IN HINT1 GENE PRESENTING
WITH NEUROMYOTONIA AND HEREDITARY NEUROPATHY

Dr. Leena Shingavi*1 ; Seena Vengalil2 ; Kiran Polavarapu2 ; Veeramani Preethish-Kumar2 ;


Saraswathi Nashi2 ; Atchayaram Nalini2
1Clinical Neurosciences, National Institute of Mental Health and Neurosciences (NIMHANS),

India, 2Neurology, NIMHANS, India


Email Address: leenashingavi@gmail.com

Introduction: Histidine Triad Nucleotide binding protein 1 (HINT1) also known as Protein
kinase C inhibitor 1 (PRKCNH-1); Protein kinase C interacting protein 1 (PKCI-1), mapped to
chromosome 5q31, is a purine phosphoramidase homodimer and is ubiquitously expressed.
HINT1 is a tumour suprresor gene and is involved in several apoptotic pathways. Biallelic
variants in the HINT1 gene are known to cause Neuromyotonia with Axonal Neuropathy
(NMAN). Zimon et al. have identified 8 HINT1 variants in 33 families of NMAN.

Results: A 13-year-old male child, born of consanguineous parentage; presented for the first
time in 2018 with of bilateral foot drop of 2 years duration. This was followed one and half year
later by bilateral forearm and hand muscle weakness in the form of finger and wrist drop with
ulner deviation of the wrist. Hyperpigmentation of the knuckles was noted on the examination.
Investigations were negative for screening of inborn errors of metabolism by Tandem mass
spectrometry. His serum vitamin B12 level was 692 pg/ml (180-914 pg/ml). ENMG
demonstrated neurogenic myotonia. NGS of the proband reported a pathogenic start-loss
variation c.3G>C_NM005340.7 (p.Met1Ile) in the HINT1 gene aligned to human reference
genome (GRCh37/hg19). The variant is not reported in the ClinVar and the Human gemone
mutation (HGMD) databases. A variant at c.2T>C has been reported as likely pathogenic for
NMAN in the ClinVar database. The variant is reported as damaging by SIFT, possibly
damaging by PolyPhen2, and disease causing by MutationTaster2. CADD score is 24. The
variant is not reported in gnomAD, 1000 genome and ExAC population frequency databases.

Conclusion: Rare genetic causes of CMT are important contributions to the full allelic
spectrum of the disease. Here we report an ultra-rare NMAN case of Indian origin; mediated by
a novel, pathogenic, biallelic start-loss variant in the HINT1 gene.

256
Neuromuscular Ultrasound
NU001. ULTRASOUND ASSESSMENT FOR SHOULDER PAIN: EVALUATION
OF ETIOLOGY IN URBAN AREA OF JAKARTA

Anne Dina Soebroto*1 ; Vika Ardianto Laksono2 ; Rio Hermawan3


1Neurology, MRCCC Siloam Hospitals Semanggi, Indonesia, 2Resident Medical Officer,

MRCCC Siloam Hospitals Semanggi, Indonesia, 3Radiology, MRCCC Siloam Hospitals


Semanggi, Indonesia
Email Address: annesoebroto@gmail.com

Introduction: Shoulder pain is the third most common cause of musculoskeletal problems with
chronicity and high recurrence rate. Due to its superficial location, Ultrasound imaging (US)
can be used to assess associated pathological conditions. We try to identify the frequency of
US-diagnosed shoulder disorders in our clinical practice.

Methods: We reviewed and analysed 49 shoulder ultrasound reports of patients with shoulder
pain (sub-acute and chronic) from our neurology department in MRCCC Siloam Hospitals
Jakarta. All of the patients were referred to radiology department after initial assessment from
neurologist. Patients were assessed for their biceps tendon, subscapularis tendon,
supraspinatus tendon, infraspinatus and teres minor tendon and also evaluated for
acromioclavicular joint and posterior structures such as posterior glenohumeral joint recess.

Results: The most frequently diagnosed pathology was calcified (chronic) tendinopathy
(30.6%), followed by non-calcified tendinopathy (22.4%), subacromial-subdeltoid bursitis
(22.4%), acromioclavicular (AC) joint stenosis (14.3%), and miscellaneous disorders such as
lipoma and fibroma (4.1%). There were 3 cases (6.2%) where no specific disorder was found.
In the tendinopathy group (calcified and non-calcified), long biceps tendon was the most
frequently affected tendon (46.4%), followed by supraspinatus tendon (39.3%) and
subscapularis tendon (14.3%).

Conclusion: Among patients presenting with shoulder pain, tendinopathy is the most common
disorder found using ultrasound assessment of shoulder, followed by bursitis, and
acromioclavicular joint stenosis.

257
NU002. MUSCLE ULTRASOUND IS A HIGHLY SENSITIVE NON-INVASIVE
TOOL TO DETECT FASCICULATIONS IN LIMBS, TONGUE AND
PARASPINAL MUSCLES IN AMYOTROPHIC LATERAL SCLEROSIS

Rahul Reddy Rajula*1 ; Seena V1 ; Saraswathi N1 ; Niranjan M1 ; Kiran P1 ; Preethish V1 ;


Keerthipriya M1 ; Sanita Raju1 ; Saini J2 ; Nalini A1
1Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India,
2Neuroradiology, National Institute of Mental Health and Neurosciences, Bengaluru, India

Email Address: rahulreddyrajula@gmail.com

Introduction: Fasciculations are characteristic feature of Amyotrophic Lateral Sclerosis (ALS)


and their identification by Muscle Ultrasound (MUS) is an important non-invasive tool and
allows studying the major bulk and more inaccessible muscles. This could potentially replace
painful Electromyography (EMG) procedure to identify fasciculations. Our aim is to utilize MUS
to identify and also detect fasciculations in unaffected muscles in early stages of ALS and
correlate with EMG findings.

Methods: In 30 cases (7 bulbar and 23 limb onset) with suspected ALS, we performed EMG in
3 muscles (biceps brachii, quadriceps and thoracic paraspinal) and MUS in 9 muscles (biceps,
triceps, deltoid, abductor digiti minimi, quadriceps, hamstrings, tibialis anterior, thoracic
paraspinal and tongue). MUS was performed using linear transducer (3-13 MHz, Esaote Mylab
Gamma Scanner). Fasciculations were observed in each muscle for one minute. Frequency of
fasciculations detected by MUS was compared with EMG findings in same muscles.

Results: The M:F ratio was 4:1, age of onset was 50.1±12.8 years, age of presentation was
50.9±12.9 years. Duration of illness was 1.21±1.08 years. Of the total muscles studied, 176
muscles showed wasting while 334 were normal. Out of 284 muscles without clinical
fasciculations, 206 (72.5%) showed fasciculations on MUS. Of 90 muscles examined by EMG,
20 (40.8%) of 49 muscles without clinical fasciculations showed fasciculations. Out of 23 pure
limb onset cases, 16 cases showed extrinsic tongue muscle fasciculations on MUS.
Fasciculations as detected by MUS vs. EMG: biceps brachii (90% vs 76.6%), quadriceps (80%
vs 50%) and thoracic paraspinal (66.6% vs 40%)

Conclusion: MUS is a promising tool in diagnosis of ALS. MUS is more sensitive in detecting
fasciculations compared to EMG, can substitute it as it is noninvasive, safer, easily accessible,
can access larger muscle areas in shorter examination time. Further, the test can be repeated
for any longitudinal/sequential assessments.

258
NU003. ESTIMATION OF CROSS-SECTIONAL AREA REFERENCE VALUES
OF NERVES IN THE UPPER AND LOWER EXTREMITIES USING
ULTRASONOGRAPHY IN THE INDIAN POPULATION

Sindhu DM*1 ; Seena Vengalil1 ; Saraswathi Nashi1 ; Jitender Saini2 ; Lokesh Bhatala1 ; Sanita
Raju1 ; Nalini A1
1Neurology, NIMHANS Bangalore, India, 2Neuroimaging and Interventional Radiology,

NIMHANS, India
Email Address: drsindhudm@gmail.com

Introduction: High resolution ultrasonography (HRU) permits direct assessment of the


morphology of the nerve, unlike electrophysiology that assesses the functionality of the nerves.
Normative data of nerves in a population is crucial in differentiating from pathological
conditions and hence this study was conducted.

Methods: The demographics, physical characteristics and HRU of median, radial, ulnar,
fibular, tibial, sural, and superficial fibular nerves at defined sites of 60 adult healthy volunteers
were evaluated and recorded. Nerve conduction study (NCS) of ulnar and common peroneal
nerves were done to rule out subclinical neuropathy. The estimated cross-sectional area (CSA)
reference values and their correlations with the age, weight, height, body mass index (BMI)
were evaluated.

Results: The mean age of the population was 35.6 (SD-11.1) years, height = 161.4 (SD-8.3)
centimeters, weight = 57.4 (SD-9.5) kilograms and BMI = 21.99 (SD-2.9) kilograms/m2. The
M:F ratio = 1.2:1. The NCS of ulnar and common peroneal nerves were within normal limits.
The normal reference values for each nerve were as follows: median nerve at wrist = 7.1±2.8
mm2, at mid forearm level = 4.8±1.6 mm2, at elbow = 7.4±2.8 mm2, ulnar nerve at wrist =
4.3±1.4 mm2, at mid forearm level = 4.4±1.4 mm2, at elbow = 5.3±2.1 mm2, radial nerve at
spiral groove = 4.2±1.3 mm2, tibial nerve at popliteal fossa = 19.5±8.6 mm2, at the medial
malleolus = 9.3 ± 3.8 mm2, common peroneal nerve at fibular head = 9.2±4.2 mm 2, sural nerve
above the lateral malleolus = 1.8±0.8 mm2. Positive correlation was seen between height and
median nerve at wrist and elbow, radial nerve at spiral groove, tibial nerve at popliteal fossa
and medial malleolus, between weight and median nerve at elbow, ulnar nerve at forearm and
elbow, tibial nerve at popliteal fossa and medial malleolus and common peroneal nerve at
fibular, between BMI and median nerve at elbow, tibial and common peroneal nerves.

Conclusion: The reference values for the upper and lower limb peripheral nerves were
recorded. These values could be used for comparison of normal versus abnormal nerves in
peripheral neuropathies and could also help in selecting the nerve for biopsy.

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NU004. NERVE ULTRASOUND IN MULTIFOCAL MOTOR NEUROPATHY:
CORRELATION WITH ELECTROPHYSIOLOGY AND FUNCTIONAL
DISABILITY

Cheng-Yin Tan*1 ; Khean-Jin Goh1 ; Mohd Azly Yahya1 ; Nortina Shahrizaila1


1Neurology, University of Malaya, Malaysia

Email Address: cytan@ummc.edu.my

Introduction: Increased nerve cross-sectional area (CSA) on ultrasound is associated with


conduction block in multifocal motor neuropathy (MMN). We investigated the ultrasound
features of peripheral nerves in MMN and their correlation with electrophysiology and
functional disability.

Methods: MMN patients fulfilling the EFNS/PNS criteria were recruited from University Malaya
Medical Centre, Kuala Lumpur, Malaysia. All patients underwent neurological assessment with
MRC sumscore, grip strength, INCAT disability score, and had nerve conduction studies. Each
patient also underwent nerve ultrasound study using a standardized protocol. Age- and
gender-matched healthy controls were recruited for comparison.

Results: Three MMN patients (2 men) aged 46.3±10.6 years and three normal controls were
included. Mean disease duration was 4.3±2.5 years (ranged 1.5-6.0). CSF protein ranged
0.25-0.50 g/L with a mean 0.40±0.13 g/L. One patient was positive for anti-GM1 IgM. Mean
MRC was 55.0±5.3 (range 49-59), handgrip (right: 13.2±8.1 kg; left: 21.7±11.9 kg) and INCAT
3.7±1.1 (range 3-5). Nerve CSAs were significantly larger in MMN patients than normal
controls in median (forearm: p=0.043), ulnar (forearm: p=0.047), tibial (knee: p=0.002), radial
(p=0.002) and sural (p<0.001) nerves. The side-to-side difference ratio of intranerve CSA
variability (ICSAV) for ulnar nerve (p=0.046) was significantly larger in MMN patients. The
nerve CSAs were significantly correlated with MRC [median midarm/forearm ratio (p=0.004);
ulnar ICSAV (p=0.037)] and INCAT [median: elbow (p=0.029), midarm (p=0.030) and
midarm/forearm ratio (p=0.007); ulnar ICSAV (p=0.029)]. Correlation analysis with
electrophysiology found increased nerve CSAs significantly correlated with distal motor latency
[median: wrist (p=0.042), ICSAV (p=0.005)]; and inversely correlated with motor conduction
velocity [median ICSAV (p=0.042)] and proximal/distal CMAP ratio [tibial knee (p=0.005)].

Conclusion: In MMN, nerve enlargement was focal, at non-entrapment sites, asymmetrical


and can involve the sensory nerve. Nerve ultrasound significantly correlates with functional
disability and electrophysiological parameters suggesting that it may be useful as an additional
diagnostic biomarker in MMN.

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NU005. NERVE CROSS-SECTIONAL AREA AS A SURROGATE MARKER OF
PERIPHERAL NERVE DEGENERATION IN AMYOTROPHIC LATERAL
SCLEROSIS

Toh Tsun Haw*1 ; Nur Adilah Abdul Aziz1 ; Mohd Azly Yahaya1 ; Ee Chin Loh2 ; David
Capelle2 ; Khean Jin Goh1 ; Lydia Abdul Latif3 ; Nortina Shahrizaila1
1Neurology, University of Malaya, Malaysia 2Palliative Care Unit, University of Malaya,

Malaysia, 3Department of Rehabilitation Medicine, University of Malaya, Malaysia


Email Address: yuy987@hotmail.com

Introduction: In Amyotrophic Lateral Sclerosis (ALS), there is progressive motor neuron


degeneration leading to progressive muscle weakness. Nerve ultrasound has the potential to
detect progressive nerve atrophy through the evaluation of nerve cross-sectional area (CSA).
We aimed to longitudinally study the peripheral nerves CSA in ALS patients.

Methods: ALS patients fulfilling the revised El-Escorial and Awaji criteria presenting to
University Malaya Medical Centre were consecutively recruited. Two sets of peripheral nerve
ultrasound were performed at six-month intervals, documenting the nerve CSAs at proximal
and distal segments of median, ulnar, peroneal and tibial nerves. These findings were
correlated with disease duration and the revised ALS Functional Rating Scale (ALSFRS-R).

Results: A total of 40 patients (mean onset age 56.5± 8.8 years) were recruited. The diagnosis
of ALS was definite in 42.5%, probable in 40.0% and possible in 17.5%. Most patients had
limb-onset ALS (77.5%). The mean ALSFRS-R was 33.7±9.7 (baseline) and 28.3±11.0 (6
months). All ALS patients had at least one nerve segment CSA reduction on longitudinal
evaluation at 6 months. Progressively smaller CSA was seen in 26/40 (65.0%) common
peroneal nerves, 23/40 (57.5%) tibial nerves, 18/40 (45.0%) ulnar nerves and 17/40 (42.5%)
median nerves. In 38/40 (95.0%) patients, there was a decline in the proximal:distal CSA at 6
months, suggesting proximal nerve segment atrophy. This decline was more marked in the
tibial nerve compared to the median and ulnar nerves. At 6 months, the disease duration
negatively correlated with median nerve CSA at elbow (R=-0.331, p=0.040) while the ALSFRS-
R correlated with ulnar nerve midarm CSA (R=0.359, p=0.025).

Conclusion: We detected a decline in nerve size in the majority of ALS patients at 6 months.
Our findings suggest that longitudinal changes in nerve CSA may be helpful as a surrogate
biomarker of progressive nerve degeneration in ALS.

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NU006. LONGITUDINAL EVALUATION OF FASCICULATION THROUGH
ULTRASONOGRAPHY IN AMYOTROPHIC LATERAL SCLEROSIS

Toh Tsun Haw*1 ; Nur Adilah Abdul Aziz1 ; Mohd Azly Yahaya1 ; Ee Chin Loh2 ; David
Capelle2 ; Khean Jin Goh1 ; Lydia Abdul Latif3 ; Nortina Shahrizaila1
1Neurology, University of Malaya, Malaysia 2Palliative Care Unit, University of Malaya,

Malaysia, 3Department of Rehabilitation Medicine, University of Malaya, Malaysia


Email Address: yuy987@hotmail.com

Introduction: Muscle fasciculation in amyotrophic lateral sclerosis (ALS) is typically evaluated


through needle electromyography. In recent years, ultrasound has been increasingly used to
detect fasciculation particularly as this procedure is non-invasive. We aimed to longitudinally
evaluate muscle fasciculation in ALS patients through neuromuscular ultrasound.

Methods: ALS patients fulfilling the revised El-Escorial and Awaji criteria presenting to
University Malaya Medical Centre were consecutively recruited. Neuromuscular ultrasound
was performed at 6-monthly intervals. Two sets of 30-second recordings were obtained from
three cervical region muscles (biceps brachii, brachioradialis, first dorsal interosseus) and two
lumbosacral region muscles (vastus medialis, tibialis anterior). Fasciculation was considered
present when there were ≥ two involuntary contractions. These findings were correlated with
disease duration and the revised ALS Functional Rating Scale (ALSFRS-R).

Results: A total of 40 patients were recruited. The diagnosis of ALS was definite in 42.5%,
probable in 40.0% and possible in 17.5%. The mean onset age was 56.5±8.8 years. Most
patients were limb-onset ALS (77.5%). The mean ALSFRS-R was 33.7±9.7 (baseline) and
28.3±11.0 (6 months). At baseline, 17/40 (42.5%) patients had fasciculations in one region,
19/40 (47.5%) in two regions while 4/40 (10.0%) had none. At 6 months, the regions affected
remained unchanged in 21 (52.5%) patients. In eight (20.0%) patients, fasciculation was
further detected in additional region/s. In five (12.5%) patients, fasciculation could no longer be
detected. The disease duration was negatively correlated with the number of muscles with
fasciculation detected (R=-0.319 p=0.045 at baseline, R=-0.361 p=0.020 at 6 months). There
was, however, no correlation between ALSFRS-R and the number of muscles with
fasciculation.

Conclusion: Our findings support the role of neuromuscular ultrasound as a diagnostic tool in
detecting fasciculation in ALS. However, its role as a prognostic biomarker remains less clear
as fasciculation can be less frequently detected with progressive motor neurons loss.

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Muscle Disorder
MU002. MUTATION SPECTRUM OF CONGENITAL MUSCULAR
DYSTROPHIES: A CASE SERIES FROM INDIA

Seena Vengalil*1 ; Kiran Polavarapu2 ; Saraswati Nashi1 ; Veeramani Preethish-Kumar2 ;


Niranjan Prakash Mahajan1 ; Maddasu Keerthipriya1 ; Chevulu Pradeep Chandra Reddy1 ;
Gautham Arunachal3 ; Narayanappa Gayathri ; Atchayaram Nalini1
1Neurology, National Institute of Mental Health and Neuro Sciences, India, 2Clinical

Neurosciences, National Institute of Mental Health and Neuro Sciences, India, 3Human
Genetics, National Institute of Mental Health and Neuro Sciences, India, 4Neuropathology,
National Institute of Mental Health and Neuro Sciences, India
Email Address: seenavengalil@gmail.com

Introduction: Congenital muscular dystrophies (CMD) are a group of neuromuscular disorders


with clinical and genetic heterogeneity with onset at birth or infancy. Little is known about the
genetic spectrum of CMD in India.

Methods: A retrospective study on genetically confirmed CMDs (2015- 2019). Detailed


phenotyping and Next Generation Sequencing (NGS) were done.

Results: A total of 19 patients were classified into 5 subgroups based on the protein and gene
defect. Ullrich CMD group=three patients, mean age of onset of 1year, M:F=2:1, clinically had
motor delay and limb girdle weakness, proximal contractures, distal hypotonia, joint
hyperextensibility, prominent calcaneum and velvety palms/soles. Homozygous mutations
were found in COL6A2[c.310C>T(p.Gln104Ter), (c.2098G>A(p.Gly700Ser)),
COL6A3[c.79C>T(p.Gln27Ter)] gene respectively. Bethlem myopathy=6 patients, mean age of
onset=10 years, M:F= 2:4, had limb girdle weakness, prominent calf hypertrophy and
contractures. Heterozygous mutations in COL6A1[c.805G>A; p.Gly269Arg],
COL6A2{[c.655A>C(p.Met219Leu)], [c.1690G>C (p.Gly564Arg)]}; COL6A3{[c.6309+3A>G 5'
splice site], [c.175C>T(p.Arg59Ter)]}; COL12A1[c.2944G>A (p.Gly982Arg)] respectively.
Merosin deficient=5 patients, mean age of onset=4 years, M: F=2:3, had motor delay with limb
girdle weakness, bifacial weakness, proximal and distal contractures, calf hypertrophy.
Neuroimaging showed leukoencephalopathy with cerebellar cysts. Mutations were
homozygous in LAMA2 gene in two patients [c.6350A>G] and exon 13 (c.
1823_1824del(p.Tyr608Ter) respectively and compound heterozygous in three [Exon,36-
c.5176A>T(p.Lys1726Ter); Exon,56-c.7810C>T(p.Arg2604Ter)], (exon
8c.1176_1177del)p.Cys393TyrfsTer2), exon38(c.5476C>T(p.Arg1826Ter), [Exon,49-
c.6955C>T(p.Arg2319Ter), CNVvariation(deletion)- exon,34-37]. Emery-Dreifuss Muscular
dystrophy=3, mean age of onset of 10 years, M:F=1:2, had limb girdle pattern of weakness,
elongated facies, neck and elbow contractures, kyphoscoliosis, scapular winging.
Heterozygous mutations in LMNA gene seen in all 3 [c.115A>C (p.Asn39His)],

263
[c.1357C>T(p.Arg453Trp)] and c.305T>C. CMD dystroglycanopathy with or without mental-
retardation=two with onset in infancy, had motor delay, genetically had homozygous mutation
in FKRP gene [c.1343C>T(p.Pro448Leu)] and POMT1(c.193G>A(p.Gly65Arg)

Conclusion: This study describes a large cohort of genetically confirmed CMDs from a single
center in India and demonstrates the genetic heterogeneity.

264
MU003. THE IMPAIRMENT OF EXCITATION-CONTRACTION COUPLING IN
ICU-ACQUIRED WEAKNESS

Bungo Hirose*1 ; Kazuna Ikeda2 ; Daisuke Yamamoto2 ; Emiko Tsuda3 ; Rika Yamauchi1 ;
Takayoshi Hozuki3 ; Shun Shimohama2 ; Yuta Asada4 ; Tomihiro Imai4
1Neurology, Sunagawa City Medical Center, Japan, 2Neurology, Sapporo Medical University

School of Medicine, Japan, 3Neurology, Sapporo Shirakabadai Hospital, Japan, 4Disability


Studies of Peripheral Nerve and Muscle, Sapporo Medical University Graduate School of
Health Sciences, Japan
Email Address: bungorianchop@gmail.com

Introduction: The aim of this study is to elucidate the impairment of excitation-contraction (E-
C) coupling in ICU-acquired weakness (ICUAW) using a unique electrophysiologic method in
patients received the intensive care in ICU.

Methods: We examined 13 patients with ICUAW and 11 normal controls. We performed


successive recordings of electrophysiological tests after ICU admission. First, CMAPs of
abductor hallucis muscle (AH) and sural SNAPs were recorded using a conventional method.
In addition, the tibial nerve was stimulated at the popliteal fossa using a supramaximal
rectangular pulse to record CMAPs from soleus muscle (SOL). Simultaneously, movement-
related potential (MRP) was recorded using an accelerometer (SV1101; NEC, Tokyo Japan)
taped at the base of hallux and calculated E-C coupling time (ECCT) based on the latency
differences between soleus CMAP and MRP.

Results: We recorded significantly decreased CMAP amplitudes in 6 AHs and 8 SOLs and
significantly longer CMAP durations in 2 SOLs. Also, we recorded significantly decreased
MRPs in 9 patients and significantly prolonged ECCTs in 10 patients. These abnormal findings
were detected even in very short durations of ICU stay such as 2-3 days. The durations of ICU
stay seemed to be correlated with involvement of CMAP amplitudes of AH/SOL, MRPs and
ECCTs. Ten patients showed decreased SOL-CMAP amplitude concomitant with decreased
MRP and/or prolonged ECCT in spite of preservation of SOL-CMAP duration. In addition,
CMAP amplitudes of AH/SOL and MRP were significantly smaller in patients than in controls.
Furthermore, CMAP durations of AH and ECCTs were significantly longer in patients than in
controls.

Conclusion: The E-C coupling may be impaired in the very early stages of CIM. Noninvasive
measurement of MRP and ECCT is easy to perform, and may be useful to detect the
impairment of E-C coupling in the early stages of ICUAW.

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MU004. A RARE TREATABLE CAUSE OF DROPPED HEAD SYNDROME

Nurul Maisarah Sainuddin*1 ; Christopher Chua1 ; Sibi Sunny1 ; Joy Vijayan1 ; Kay Ng1
1Neurology, National University Hospital

Email Address: maisarah_sainuddin@nuhs.edu.sg

Introduction: Dropped head syndrome results from neck extensor weakness, and is
commonly due to neuromuscular disorders such as inflammatory myopathies, myasthenia
gravis, or amyotrophic lateral sclerosis. We describe, with neck muscle imaging findings, a
man presenting with neck extensor weakness due to hypothyroidism that resolves with
thyroxine replacement.

Results: A 60-year-old man without any past medical history of note presented with neck pain
for 7 years. On examination, he was noted to have head drop, without limb weakness, bulbar
symptoms nor fatiguability. Neurologic examination was otherwise normal. Creatine kinase
(CK) was raised at 2480 U/L. Thyroid-stimulating hormone (TSH) was high at 64.50 mIU/L
(Normal 0.45-4.50 mIU/L), and T4 was low at < 3.2 pmol/L (Normal 8-16 pmol/L). Thyroid
peroxidase and anti-thyroglobulin antibodies were positive. Antiacetylcholine receptor and
myositis-specific antibodies were negative. Repetitive nerve stimulation was negative. Needle
electromyography showed evidence of an irritable myopathy in the deltoid, iliopsoas and neck
extensors. Computed Tomography of the neck showed hypertrophy of the splenius capitis. He
was diagnosed with myopathy due to hypothyroidism and started on levothyroxine
replacement. 6 months’ later, there was resolution of his dropped head with normalization of
his CK levels.

Conclusion: Hypothyroidism more commonly presents with proximal limb weakness or raised
CK. Associated axial myopathies are very rarely described. Hypothyroidism is a treatable
cause of dropped head syndrome that reverses with thyroxine replacement. It is a rare cause
of neck extensor muscle hypertrophy and irritable myopathy. Recognizing this entity is
important to avoid unnecessary invasive investigations, such as muscle biopsy, or
unnecessary immunomodulatory treatment in a neck extensor myopathy that will self-resolve
with normalization of thyroid function. Muscle imaging can be helpful in its diagnosis.

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MU005. MYOPATHY IN PRIMARY SJÖGREN’S SYNDROME IN DR.
MOEWARDI HOSPITAL INDONESIA

Dody Wahyu Lestya Nugraha*1; Baarid Luqman Hamidi1


1Neurology Department, Universitas Sebelas Maret, Indonesia

Email Address: dodyda888@gmail.com

Introduction: Myopathies are disorders affecting skeletal muscle. There is a term 'overlap
syndrome' when myopathies are associated with another well-defined connective tissue
disease. Sjögren’s syndrome is one of the connective tissue disease characterized by hypo-
function of salivary and lacrimal glands and possible systemic multi-organ manifestations.
Although muscular manifestations are very common with Sjögren’s syndrome, true myopathy
is very rare. Myopathy inflammatory has been reported in less than 3% of patients. The aim of
the case report is to report a case of myopathy in primary Sjögren’s syndrome in Dr. Moewardi
hospital Surakarta.

Results: 22 years old female, with the weakness of all four extremities, arthralgia, myalgia,
and shortness of breath due to respiratory failure. The weakness was heavier in the proximal.
Schirmer’s test ≤5 mm/5 min in left eye. From the results of laboratory examination obtained
increased CK, SGOT, and SGPT. From cerebrospinal fluid examination, no dissociation of
cytoalbumin was found. There was a positive (strong) result on La / SS-B antigen. Muscle
biopsy showed hyalinized muscle. Plasmapheresis was performed on this patient. The patient
received intravenous methylprednisolone, azathioprine, and folic acid therapy. The patient did
not improve after the plasmapheresis and immunosuppressant administration.

Conclusion: Skeletal muscle involvement in primary Sjögren’s syndrome is a relatively rare


complication. Some signs, symptoms, and laboratory results support the appearance of
myopathy in this case. Muscle biopsy is the gold standard examination for diagnosed
myopathy. In Sjögren’s syndrome, the type of myopathy that is often found is myositis. In this
case, there was a hyalinized muscle that supports myopathy but no inflammatory cells were
found on the muscle biopsy. Myopathy in Sjögren’s syndrome responded favorably to high-
dose steroids. In this case, there was no improvement after immunosuppressant administration

267
Pain
PA001. PARSONAGE TURNER SYNDROME

Pricilla Yani Gunawan*1 ; Fitri Octaviana2 ; Ahmad Yanuar Safri2 ; Rocksy Situmeang1 ;
Yusak Mangara Tua Siahaan1
1Neurology, Pelita Harapan University, Indonesia, 2Neurology, University Of Indonesia,

Indonesia
Email Address: pricilla.gunawan@uph.edu

Introduction: Parsonage Turner Syndrome (PTS) is a rare syndrome that may occur in
healthy subjects. The clinical presentation might not be typical and risk factors attributed to the
development of disease may not be easily depicted. We present a case of abrupt onset of
unilateral shoulder pain, followed by progressive neurologic weakness.

Result: A 54-year-old male presented with abrupt onset of shoulder pain mainly on the right
side. Pain was felt in both shoulder and thigh which progressed to weakness, dysesthesia, and
numbness in the right upper arm. In 4 days, he could barely lift his right upper arm. There was
no history of trauma nor infection on the affected area. On physical examination, there were
limitation of shoulder abduction and external rotation. His right arm strength from proximal to
distal was 1344. Other extremities showed no significant weakness. Sensory involvement did
not match dermatomal and peripheral sensory distribution. Nerve Conduction Velocity (NCV)
showed axonal motor neuropathy of right axillary nerve. Electromyography (EMG) showed no
Motor Unit Action Potential (MUAP) on deltoid muscle. His CK was 457, and there was an
increase in LDH and CRP. Cervical MRI was unremarkable. Steroids were immediately given,
and the response was fast and satisfactory. After 1 day, his motor strength increased, and the
pain subsided. EMG showed single MUAP on deltoid. After 10 days, his motor strength
returned to near normal. NCV study showed decreased amplitude of right axillary nerve, and
recruitment was slightly decreased on right deltoid muscle. He underwent physical therapy and
was making marked progress.

Conclusion: PTS generally involves one upper limb, and axillary nerve is one the most
commonly involved. PTS should be considered as a differential diagnosis in cases of shoulder
pain and arm weakness without any clear etiology.

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PA002. CHRONIC LOW BACK PAIN TREATED WITH PERINEURAL
SUBCUTANEOUS INJECTION OF DEXTROSE 5% IN WATER (D5W): A
CASE SERIES

Budhi Febrian Purwo Jatmiko*1 ; Indra Sari Kusuma Harahap1 ; Wahyu Wihartono2 ; Whisnu
Nalendra Tama1
1Departement of Neurology, Faculty of Medicine, Public Health and Nursing, Universitas

Gadjah Mada, Yogyakarta, Indonesia, 2Departement of Neurology, RSPAU Dr.S.Hardjolukito,


Yogyakarta, Indonesia
Email Address: dr.budhifebrian@gmail.com

Introduction: Most people will experience low back pain (LBP) problem minimally once in their
lifetime. Chronic LBP (CLBP) is a primary source of incapacitation, struggling and expense,
commonly resulting in significant anxiety and disturbance with daily activities leading to a
significant level of disability. Several studies showed pain reduction with Perineural
Subcutaneous Injection (PSI) over related sensory nerve pathways in LBP. PSI is an injection
of D5W close to subcutaneous nerves to reduce neuropathic inflammation, treating the nerves
and restore their normal function. This study presents the outcomes of PSI for CLBP
treatment.

Methods: The subject in this study were six patients with CLBP who had failed conventional
therapies in the neurological outpatient clinic in Dr. Sardjito General Hospital Yogyakarta
Indonesia. They were administered PSI treatment, injected with D5W about 0,5 cc each in the
subcutaneous tissue on 5-8 tender points of the low back region. Visual Analogue Scale (VAS)
score was assessed before treatment. Two to four weeks after treatment the patients were
asked about their VAS score.

Results: Two males, and 4 females from 45 until 65 years old were treated with PSI. All
patients reported pain reduction of 50% - 70% as measured by VAS scores.

Conclusion: PSI of D5W has potential benefits for CLBP and needs to be considered in
clinical practice and further research is required.

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PA004. SEVERE HEADACHE POST COITAL: A CASE REPORT

Suanton*1 ; Yudiyanta1 ; Indarwati Setyaningsih1 ; Whisnu Nalendra Tama1


1Neurology, Gadjah Mada University, Indonesia

Email Address: schevanton12@gmail.com

Introduction: Headache associated with sexual activity (HAS) is an acute headache that is
often related to sexual intercourse. It may also be classified as benign or malignant. Though
largely benign, the headache may seldom be a symptom of an underlying life threatening
neurologic disorder such as aneurysmal subarachnoid hemorrhage.

Results: We report a case of 40-year-old Indonesian man, admitted to hospital because of


sudden onset of severe headache followed by nausea and vomiting during sexual intercourse,
particularly at the time of orgasm. This complaint had been repeated three times a month. The
patient had history of hypertension and diabetes mellitus. There was no history of head
trauma. Neurological examination was normal. There were no signs of coagulopathy on the
results of laboratory tests. A cerebral magnetic resonance imaging (MRI) and magnetic
resonance angiography (MRA) showed a chronic active subdural hematoma and suspected
left transverse sinus thrombosis. The patient underwent surgery and the complaints improved.

Conclusion: Headache associated with sexual activity (HAS) could be primary or secondary.
HAS is classified as secondary when it is associated with an underlying intracranial pathology,
notable among which are cerebral artery aneurysms and other vascular disorders. Based on
clinical criteria and neuroimaging result, these patients met the diagnostic criteria suspected
for secondary HAS. To the best of our knowledge, this is the first case of subdural hematoma
due to suspected left transverse sinus thrombosis in association with the physical exertion
associated with sexual intercourse. Headache after sexual intercourse should always be
carefully evaluated by cerebral imaging. The case highlights the importance of paying attention
to post coital headache.

270
Basic Neuroscience
BN002. MORPHINE-, MITRAGYNINE- AND THC-INDUCED ALTERATIONS
OF CANNABINOID CB1 RECEPTOR IMMUNOREACTIVITY IN BRAIN
HIPPOCAMPAL CA1 REGION OF SWISS ALBINO MICE

Nurul Iman W Ismail*1 ; Nur Aimi Zawami Ahmad1 ; Ummi Nasrah Talib1 ; Nurul Aiman Mohd
Yusof2 ; Sharif Mahsufi Mansor3 ; Christian P. Muller4 ; Mustapha Muzaimi1
1Department of Neurosciences, School of Medical Sciences, Universiti Sains Malaysia (Health

Campus), Malaysia, 2Department of Anatomy, School of Medical Sciences, Universiti Sains


Malaysia (Health Campus, Malaysia, 3Centre for Drug Research, Universiti Sains Malaysia,
Pulau Pinang, Malaysia, 4Section of Addiction Medicine, Department of Psychiatry
Psychotherapy, Friedrich-Alexander-University, ErlangenNuremberg, Germany
Email Address: imanismail87@gmail.com

Introduction: Problems pertaining to the large and growing abuse of plant-derived


psychoactive substances are on the rise. Literature indicates that uncontrolled use of
phytochemicals, such as opioids, Δ-9-tetrahydrocannabinol (THC) and mitragynine (main
alkaloid of Mitragyna speciosa or Ketum plant) pose considerable harms and addictive
burdens, including behavioural and neurological alterations in brain reward system. This study
aimed to evaluate the neuronal changes in hippocampal CA1 region of brain mesolimbic
system, particularly involving cannabinoid (CB1) receptor, induced by chronic exposure to
morphine, mitragynine and THC.

Methods: Twenty-four (n=24) male Swiss albino mice were subjected to daily administration of
Tween 20-vehicle (1 ml/kg, ip, n=6), morphine sulphate (5 mg/kg, sc, n=6), mitragynine (5-25
mg/kg, ip, n=6), or THC (2 mg/kg, ip, n=6) for 28 days. On Day-29, mice brains were removed
and processed for immunohistochemistry and Western Blot analyses for the expression of CB1
receptor protein.

Results: Chronic treatment with morphine and mitragynine induced an up-regulation of CB1
receptor immunoreactivity in mice hippocampal CA1 region (p<0.05), a key brain reward area.
In contrast, THC group showed significant down-regulation of CB1 receptor binding sites in
CA1 hippocampal region (p<0.05).

Conclusion: These findings suggest the involvement of brain CB1 receptors in the abuse
liability of chronic morphine, mitragynine and THC intake.

271
BN004. NEUROPROTECTIVE EFFECT OF EDIBLE BIRD'S NEST ON
CHRONIC CEREBRAL HYPOPERFUSION INDUCED
NEURODEGENERATION IN RATS

Rehab Ismaeil*1 ; Khairunisa Binti Ahmad Affandi1, Chua Kien Hui1, Wael Mohamed1, Mohd
Fadly2
1Basic Medical Science, IIUM, Malaysia, 2BMS, IIUM, UKM, Malaysia

Email Address: rehabpharma81@gmail.com

Introduction: Alzheimer’s disease is marked by insufficient blood supply to the brain, leads to
progressive loss of memory and cognitive skills. Continuous reduction of cerebral blood flow as
a result of chronic cerebral hypoperfusion leads to overproduction of reactive oxygen species
that may cause cognitive decline. Until now there is no available curative treatment for
Alzheimer’s disease (AD) and the only available option is symptomatic treatment. Recent
alternative medicines have underscored the neuroprotective and antioxidant ability of the
edible bird’s nest (EBN). The current study evaluates the effects of EBN on hippocampal
neurons specifically in the CA1 hippocampal region by using chronic cerebral hypoperfusion-
induced neurodegeneration in rat model.

Methods: Chronic cerebral hypoperfusion was induced by permanent bilateral common carotid
arteries occlusion (2VO) in rats that triggers the neuroinflammatory processes. The rats were
divided into 4 groups: Sham group, 2VO group, and two 2VO groups treated with 2 different
doses (60,120 mg/kg) of EBN, which was administered daily by oral gavage. After 8
consecutive weeks, rats were euthanized and the hippocampi were examined
histopathologically by counting the viable neuronal cells and the level of F2 Isoprostane in
hipoocampal tissue was measured by ELISA.

Results: A significant decrease in the neuronal cell death and significant decline in F2
Isoprostane level was shown in the group of rats treated with EBN.

Conclusion: CCH, which was triggered by 2VO, causes reduction in viable neurons in the
hippocampal CA1 region. This damage could be ameliorated by administration of EBN, which
exhibits a neuroprotective effect via increasing the viable neuronal cell count and decline in
oxidative stress level. Our results underscored the possible effects of EBN in delaying the
progression of dementia in AD patients if used early in the disease. it is safe supplement that
could be used prophylactically for a long time.

272
BN005. MATERNAL VITAMIN B12 DEFICIENCY IS ASSOCIATED WITH
INCREASED OXIDATIVE STRESS AND DNA DAMAGE IN BRAIN REGIONS
OF C57BL/6 MOUSE OFFSPRING

Shampa Ghosh*1 ; Jitendra Kumar Sinha2 ; Manchala Raghunath1


1National Institute of Nutrition, ICMR, India, 2Amity Institute of Neuropsychology and

Neurosciences, Amity University UP, India


Email Address: g.shampa17@gmail.com

Introduction: Maternal micronutrient deficiencies are predominant in developing countries.


Vitamin B12 deficiency is common in women, especially during child-bearing age. Maternal
vitamin B12 deficiency is known to have a profound impact on the developing fetus and
programs it to a number of complex adult-onset disorders like cardiovascular diseases,
diabetes, neurological disturbances, etc. We hypothesized that increased oxidative stress and
reduced activity of antioxidant enzymes along with DNA damage in the brain are associated
with the etiology of these complications. As little is known about the effects of maternal vitamin
B12 restriction on brain regions of the offspring, we have focused particularly on the cerebral
cortex and hippocampus in our study.

Methods: Female, weaning C57BL/6 mice received ad libitum for 4 weeks a control diet
(American Institute of Nutrition-76A) or the same with restriction of vitamin B12. After
confirming the deficiency, the mice were allowed to breed with control males to obtain the F1
generation offspring. The different parameters related to oxidative stress and DNA damage
were assessed at 3 months of age of the offspring.

Results: Interestingly, offspring born to mice fed on vitamin B12 restricted diet had a
significantly higher degree of oxidative stress in both the cerebral cortex and hippocampus as
reflected by their increased levels of lipid peroxidation and protein oxidation. Also, the activity
of antioxidant enzymes (superoxide dismutase and catalase) was diminished in both the brain
regions of offspring born to mice fed on vitamin B12 restricted diet. DNA damage in the form of
single and double-stranded DNA breaks were also increased in the cerebral cortex and
hippocampus of mice offspring born to vitamin B12 restricted dams.

Conclusion: To conclude, maternal vitamin B12 deficiency is associated with increased


oxidative stress in the brain of young offspring, which further leads to DNA damage in the
brain.

273
Miscellaneous (Others)
OT001. POLYMYXIN-INDUCED NEUROTOXICITY MASQUERADING AS
MYASTHENIA GRAVIS

Amanda XY Chin*1 ; Benjamin YQ Tan1 ; Yihui Goh1 ; Sangeetha V1 ; Teu Chun Pin1 ; Yee
Cheun Chan1 ; Rahul Rathakrishnan1
1Neurology, National University Hospital, Singapore

Email Address: amanda_chin@nuhs.edu.sg

Introduction: We describe a patient with polymyxin-induced neurotoxicity manifesting with a


myasthenia-like syndrome. Objective: With the emergence of multi-drug resistant gram-
negative bacilli, the use of polymyxins have renewed relevance, especially in the treatment of
nosocomial infections. Frequently reported neurological adverse effects of polymyxin include
paraesthesias, polyneuropathy and ataxia. Rarely, it can cause weakness secondary to
neuromuscular blockade.

Methods: We present a patient who had ventilation-dependent respiratory failure following


polymyxin use.

Results: A 62-year-old male was admitted for cholecystitis complicated by multi-drug resistant
Escherichia Coli bacteraemia. Five days after initiation of polymyxin B, he developed two
episodes of hypercarbic respiratory failure leading to recurrent cardiac arrests. Neurological
examination revealed bilateral ptosis and fatiguable proximal weakness (proximal power MRC
grade 1, distal power MRC grade 4), suggestive of a neuromuscular junction pathology.
Repetitive nerve stimulation (RNS) revealed a decremental response and a characteristic U-
shaped envelope pattern. The patient was started on a course of intravenous immunoglobulin
due to concerns of myasthenic crisis unmasked by polymyxin, which was also promptly
discontinued given the differential diagnosis of polymyxin-related neurotoxicity. Within 48
hours, he rapidly improved. Five days after cessation of polymyxin, he had full strength and
complete resolution of ptosis. Interval RNS performed at this juncture normalised and
acetylcholinesterase receptor antibodies returned negative. Further history taken after
recovery revealed that he did not have history of droopy eyelids, diplopia or weakness prior to
this presentation.

Conclusion: Whilst few studies have highlighted weakness and respiratory failure from
neuromuscular blockade as adverse effects of polymyxin, majority of these cases were
reported between 1962 and 1973. With the renewed use of polymyxin in the era of
antimicrobial resistance, physicians should have heightened awareness of this potentially
serious complication. The clinical features of polymyxin-induced neurotoxicity may be difficult
to distinguish from myasthenic crisis. Recognition and prompt discontinuation of polymyxin is
essential.

274
OT002. ALCOHOL CONSUMPTION ALTERS THE AMYGDALAR PROTEIN
EXPRESSION OF MTORC2 IN MALE WISTAR RATS

Athirah Hanim Binti Che Rahim*1 ; Mohd Helmy Mokhtar1 ; Rashidi Mohamed Pakri2 ; Isa
Naina Mohamed3 ; Jayakumar1
1Physiology, University Kebangsaan Malaysia, Malaysia, 2Family Medicine, University

Kebangsaan Malaysia, Malaysia, 3Pharmacology, University Kebangsaan Malaysia, Malaysia


Email Address: trahtyrah1990@gmail.com

Introduction: Mechanistic target of rapamycin complex 2 (mTORC2) mediates binge intake of


ethanol in rodents and thereby facilitates behavioural sensitization towards the effects of
ethanol. Generally, mTORC2 acts as an upstream kinase by phosphorylating other
downstream kinases through its sub-components, mTOR and sin1. Amygdala is the brain
region that regulates emotion and has been associated with various behavioural changes in
alcohol use disorder (AUD). To date, little is known about the effects of ethanol on mTORC2,
especially in amygdala. Therefore, this study was undertaken to evaluate the changes in
protein expression of mTOR and sin1 in the amygdala of male Wistar rats.

Methods: Seven-weeks-old male Wistar rats (n=18) were allocated into three different groups:
control, acute, and chronic ethanol. The control group was fed solely with Modified Liquid Diet
(MLD) (without ethanol) which included low fat milk powder, maltodextrin and sucrose for 27
days. The acute group was given control MLD for 27 days and ethanol (2.5 g/kg, 20% v/v in
saline) was administered once, on day 28 via intra peritoneal route. For chronic group, MLD
without ethanol was given for first 7 days and followed by gradual introduction of ethanol at
2.4% (3 days), 4.8% (3 days) and 7.2% (14 days). Parameters such as body weight (g), MLD
intake (ml), ethanol intake (g/kg) and serum ethanol level (mg/dl) were measured. At the end,
amygdala was dissected bilaterally and the target protein expressions were analysed using
Western Blot.

Results: The relative comparison of chronic group with control showed significant increase in
the protein expression of mTOR and sin1. However, no relative difference was noticed for
acute ethanol.

Conclusion: Collectively, our data indicate changes in the protein expression of mTORC2
components following chronic ethanol intake, suggesting their probable roles in AUD at chronic
stage rather than acute in the amygdala.

275
OT003. A CASE SERIES OF FAMILIAL NEUROMYELITIS OPTICA
SPECTRUM DISORDER (NMOSD)

Chai Liang Ang*1 ; Jie Ping Schee1 ; Eng Kian Ng1 ; Yuen Kang Chia2
1Medical, Hospital Tawau, Malaysia, 2Neuromedical, Hospital Queen Elizabeth, Malaysia

Email Address: eric1990leong@hotmail.com

Introduction: Evidence of familial neuromyelitis optica spectrum disorder (NMOSD) have


been limited, reported to comprised of 3% of total cases (Matiello et al., 2010). We report a
case series of possible familial NMOSD involving only 1 generation, namely 3 out of the 6
female siblings.

Results: All 3 patients were female Malaysian Chinese with age of disease onset of 39, 56,
and 57 years respectively. Two of them demonstrated AQP4 seropositivity while one passed
away without being tested. Their presenting features were optic neuritis (ON) plus transverse
myelitis (TM) in Patient 1 and TM in both Patient 2 and Patient 3. Subsequent relapses
involved both ON and TM over years. They demonstrated relapsing disease course with
annualized relapse rates of 0.67, 0.55 and 0.20 respectively. Both Patient 2 and Patient 3
demonstrated longitudinally extensive transverse myelitis on spinal MRI with normal MRI brain.
Apart from serum anti-Ro positivity hence Sjogren’s Syndrome in Patient 3, comprehensive
workups for other aetiologies of myelitis were negative. Both Patient 2 and Patient 3
responded well with intravenous methylprednisolone (IVMP) during acute attacks/relapses,
followed by long term maintenance therapy of prednisolone plus Mycophenolate Mofetil
(MMF). Patient 3 received 1 cycle of add-on therapeutic plasma exchange once during a
relapse with severe LETM. During the last follow-up, (i) Patient 1 demonstrated right eye
blindness, (ii) Patient 2 demonstrated impaired colour vision of right eye, and (iii) Expanded
Disability Status Scale was 1 in all 3 patients. Both Patient 2 and Patient 3 fulfilled the 2015
international consensus diagnostic criteria for NMOSD. Patient 1 passed away in 1989 without
undergoing detailed para-clinical and neuroimaging investigations.

Conclusion: Familial NMOSD may still be recognized despite an onset within the same
generation with heterogenous phenotypic presentations, provided fulfilment of the international
consensus diagnostic criteria.

276
OT004. RECURRENT DIRECT CAROTID CAVERNOUS FISTULA AFTER
EMBOLIZATION, A CASE REPORT

Dotti Inggrianita*1; Eko Arisetijono2


1Neurology, Faculty of medicine Brawijaya University, Indonesia, 1Neurovascular Division,

Neurology, Faculty of Medicine, Brawijaya University, Indonesia


Email Address: dotti_i@student.ub.ac.id

Introduction: Carotid cavernous fistula (CCF) is a rare case defined as an abnormal


communication between carotid artery and cavernous sinus. Anatomically, CCF is classified as
direct and indirect. Radiological imaging with Head CT scan and CT angiography (CTA) has an
important role in CCF’s diagnosis and management. Endovascular embolization technique is
one of the effective management for CCF.

Results: A 16-year-old male complained left cephalgia and progressive visual lost on his left
eye after endovascular embolization 3 years ago. Patient was diagnosed with CCF since he
was 12. From physical examination, we found negative light perception on the left eye. From
the former CT angiography, there was vascular malformation, suspected as multiple
arteriovenous fistula at level carotid cavernous level urged bulbus oculi for 10 mm and also at
left jugular external carotid. We performed the second head CT scan and CTA, with conclusion
that there was vascular malformation which urged bulbus oculi for 17 mm. Patient was
administered with clopidogrel 75 mg.

Conclusion: Endovascular embolization as CCF’s management is effective but recurrence


can happen.

277
OT005. A RARE CAUSE OF MULTIPLE CYSTIC BRAIN LESIONS: BRAIN
METASTASIS MIMICKING NEUROCYSTICERCOSIS (NCC)

Beh Hui Chien*1


1Medical, Hospital Sultan Abdul Halim, Malaysia.

Email Address: huichienb@gmail.com

Introduction: Lung malignancy is one of the leading cause of brain metastasis.

Results: A case of multiple cystic metastasis brain lesions which mimicked NCC was reported.
A 71 years old lady with no previous medical illness, presented with progressively worsening
memory and deterioration in self-care for the past 3 weeks. She became drowsy and unable to
ambulate 1 week ago. She was a non-smoker and had history of travelling to India 1 month
ago. Clinically, GCS of E2M4V2, pupils symmetrically 2 mm, sluggish reactive. Neurological
examination showed bilateral upper limbs and lower limbs hypertonia with brisk reflexes,
plantar reflexes up-going. Lumbar puncture was performed with opening pressure of 6 cmH2O,
cerebral spinal fluid (CSF) showed protein of 0.23 g/L, glucose ratio 0.6, cell count zero,
absent of malignant cells and Mycobacterium tuberculosis. Serum toxoplasma IgG was
negative. There was a single solitary nodule size 1 cm x 2 cm at right upper zone found in
chest radiograph. She was empirically treated for NCC with oral Albendazole 400 mg bd for 2
weeks due to magnetic resonance imaging (MRI) findings of extensive cystic lesions scattered
at both hemispheres, cerebellum and within brain stem. However her condition did not
improve. We proceeded with computerized tomography (CT) thorax, abdomen, pelvis which
showed spiculated mass size 2.0 x 2.1 x 2.2 cm at superior segment of right middle lobe with
multiple hypodense lesions of varying sizes at segment VII and VIII of liver. Cysticercosis
serology was negative. Family was counselled for CT guided lung biopsy but refused. Her
condition remained the same and discharged home with follow-up for palliative care.

Conclusion: The findings of multiple cystic brain lesions is commonly seen in NCC in an
endemic country. However, in patient with no clinical improvement after treatment and
negative NCC serology warrant further investigations for other rarer causes such as brain
metastasis.

278
OT006. VERTEBRO-BASILAR DOLICHOECTASIA: A CASE REPORT OF
RECURRENT STROKE WITH TREATMENT DILEMMA

Yin Yin Tan*1 ; Md Yusri Md Yusof2 ; Kenny Tan3


1Department of Internal Medicine, Hospital Sultanah Maliha, Malaysia, 2Department of

Radiology, Hospital Sultanah Maliha, Malaysia, 3Department of Neurology, Loh Guan Lye
Specialist Centre, Penang, Malaysia
Email Address: tanyinyin2006@gmail.com

Introduction: Vertebro-basilar dolichoectasia (VBD) has been associated with stroke and all-
cause mortality. Subsequent stroke risk is also higher in patients with VBD. The incidence of
the disease is low, and most patients are asymptomatic. Howbeit this disease carries
significant mortality and disability rate in symptomatic patients. We report a case of recurrent
ischemic stroke in a patient with VBD in Hospital Sultanah Maliha.

Results: A 79-year-old man with underlying hypertension suffered from two strokes within six
months duration. His first stroke was lacunar infarction with left hemiparesis and he was
commenced on antiplatelet. He had modified Rankin Scale (mRS) score of 3 upon discharge.
Six months later, he was readmitted for generalised tonic clonic seizure with worsening left
hemiparesis. His computed tomography (CT) brain revealed hypodensity in right basal ganglia
and right occipital, in line with recurrent ischemic stroke. His CT angiography of brain showed
tortuous, elongated and dilated vertebral and basilar arteries with wall calcification which are
consistent with VBD. Family members opted for conservative management and he was
discharged with mRS score of 5.

Discussion: VBD is recognized as an important independent risk factor for recurrent stroke.
There are currently no effective measures to manage patients with VBD who present with
ischemic symptoms. Dolichoectasia is an arteriopathy rather than an atherosclerosis; thus, the
role of antiplatelet or anticoagulant therapy is unclear, but it may increase the risk of VBD
rupture.

Conclusion: VBD is considered a challenging lesion without an ideal treatment modality.


Despite the unfavourable course of VBD, there is still uncertainty in the optimal treatment of
VBD.

279
OT007. IS IT GBS TREATMENT RELATED FLUCTUATION OR INADEQUATE
TREATMENT OF GBS? A CASE REPORT

Sujarot Dwi Sasmito*1; Badrul Munir2


1Department of Neurology, Faculty of Medicine, University of Brawijaya, Indonesia, 2Staff of

Neuroimmunology Division in Department of Neurology, Faculty of Medicine, University of


Brawijaya, Indonesia
Email Address: sujarot.sasmito@gmail.com

Introduction: Guillain-Barre Syndrome (GBS) is an autoimmune disease with acute ascending


paralysis of the limb. Intravenous Immunoglobulin (IVIG) and plasmapharesis are still the
effective treatment for GBS. In some cases, recurrence and treatment related fluctuation (TRF)
of GBS can occur. TRF is defined as improvement in the GBS disability scale of at least one
grade after completion of therapy followed by a worsening of the disability scale of at least one
grade within the first 2 months after disease onset. Meanwhile, recurrence of GBS is defined
as two or more episodes that fulfilled the criteria for GBS, with either a minimum interval
greater than 4 months between episodes if the patient did not recover completely or greater
than 2 months when there was a complete or near complete recovery after the previous
episode.

Results: A 47-year-old male with acute ascending paralysis accompanied with paresthesia in
his leg. EMG result showed moderate axonal demyelinating motor sensory polyneuropathy
(AMSAN) and diagnosed with GBS AMSAN type. He had plasmapharesis 2 times and his
condition was improved (GBS Disability scale from 4 before plasmapharesis and became 3
after treatment). One month after onset, he complained his condition became worse again
(GBS Disability scale 4). We assessed this condition as TRF. We conducted plasmapharesis
again but only 2 times, because of limitation in national health insurance claim. Patient was
discharged from hospital with no improvement. He routinely underwent physiotherapy and not
on any pharmacotherapy. His condition improved in six months.

Conclusion: We assumed that TRF in this patient was caused by inadequacy of


plasmapharesis. This problem arises because of high cost of plasmapharesis while the
national health insurance does not cover all serial plasmapharesis.

280
OT008. NON-INVASIVE 3-D IMAGE ANALYSIS OF MICROCIRCULATION
VASCULAR INTEGRITY IN ASYMPTOMATIC CEREBRAL SMALL VESSEL
DISEASE

Yuri Na*1 ; Che Mohd Nasril Che Mohd Nassir2 ; Muzaimi Mustapha2 ; Linqing Feng1
1Brain Science Institute, Korea Institute of Science and Technology (KIST), Korea, 2School of

Medical Sciences, Hospital Universiti Sains Malaysia, Malaysia


Email Address: yurisaur@gmail.com

Introduction: Today, people worldwide are living longer than before. The pace of population
aging is also increasing dramatically in the world. Cerebral small vessel disease (CSVD) is a
neurological disease resulted from pathological aetiologies affecting small vessel
(microcirculation) in the brain parenchyma and often diagnosed in the old generation.
Alarmingly, CSVD causes dementia, risk of incident stroke, Alzheimer’s disease (AD), and
depression. Image analysis plays a pivotal role, which gives a clue in identifying the different
manifestation of CSVD. This study intended to explore the microcirculation vascular integrity of
individuals with and without CSVD.

Methods: A neural tracing software neuTube was used to accurately reconstruct the blood
vessel network of brains from noninvasive 3D-time-of-flight Magnetic Resonance Angiography
(MRA) images. The quantitative analysis of the reconstructed blood vessel network provides
potentially valuable information for the diagnostic and monitoring of CSVD.

Results: neuTube provides a set of functions that enable us to delineate and reconstruct the
fine details of the blood vessel network. With ten human MRA images, the microcirculation
vascular integrity of individuals with and without CSVD was explored. Further quantitative
analysis will aid future clinical studies using this method for patients with CSVD.

Conclusion: This study opens a window to provide a non-invasive technique assessing the
integrity of small vessel in the brain.

281
OT011. POST EXPOSURE OF ANTI-DIABETIC AGENTS EXERTS
NEUROPROTECTION AND IMPROVE MEMORY AND LEARNING IN
ENDOTHELIN-1 INDUCED FOCAL ISCHEMIA IN ADULT ZEBRAFISH

Vishal K Chavda*1
1Department of Pharmacology, Nirma University, India

Email Address: chavdavishal2@gmail.com

Introduction: Stroke is the recurrent threat of mortality worldwide among normal population
and 3 fold more in diabetics. Current study was designed to elucidate the neuroprotective role
of anti-diabetic agents in endothelin-1 induced focal ischemia in adult zebrafish.

Method: In our study, pink zebra-Daniorerio was used for induction of stroke. Diabetes was
induced with 111 mM D-glucose for 14 days and zebrafish having more than 100 mg/dl blood
glucose level were included in study. The experimental stroke was induced with single oral
administration of Endothelin-1 3 µl/gm of zebrafish. Post treatment with anti-diabetic agents
was carried out for 7 days after stroke. The swimming, behavioural-patterns and cognitive
performance (Y-maze, T-maze) was recorded and analysed with UMA Tracker. The brains
were extracted for histopathological investigations.

Results: Administration of ET-1 in normal and diabetic group showed statistically significant
(p<0.001) change in behavioural swimming patterns and movements (circulatory, irregular,
disturbed, rotating) and delayed recovery time from stroke to normal swimming. Behavioural
study of ET-1 treated groups showed disturbed cognitive profile and locomotors coordination
(p<0.001) by increasing in the number of mistakes and reducing total distance travelled in
neurocognitive assessment paradigm. Histopathological analysis of ET-1 treated groups
indicated cortical lesions, shrinked neuronal cells and activated thrombocytes with disturbed
normal flora of brain compared to normal control. Anti-diabetic treatment with voglibose and
sexagliptin showed significant improvement upon post treatment in all neurological and
behavioural assessment (p<0.005).

Conclusion: Upon the data of this investigation, we concluded that among all the anti-diabetic
agents (voglibose, saxagliptin, repaglinide and dapaglifozin) voglibose and sexagliptin showed
significant improvement in neurological fitness and improved learning and cognitive profile in
post stroke adult zebrafish.

Keywords: Adult zebrafish, cerebrovascular stroke, neuroprotection, cognitive decline.

282
OT013. PAROXYSMAL TONIC SPASMS AND RIGHT FOOT DROP AS
INITIAL PRESENTATIONS OF NEUROMYELITIS OPTICA

Sin Hong Chew*1; Yuen Kang Chia1


1Neurology, Queen Elizabeth Hospital, Malaysia

Email Address: sinhong06@gmail.com

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune


inflammatory disorder commonly presents with acute myelitis or optic neuritis.

Results: We report the case of a middle age lady presented with paroxysmal tonic spasms
(PTS) of bilateral lower limbs and right foot drop, without any upper motor neuron weakness or
sensory level. EMG done confirmed a right peroneal neuropathy at the fibula head. MRI spine
showed longitudinal extensive transverse myelitis and her serum aquaporin-4 antibody was
positive, consistent with NMOSD. Incidentally, the patient also had persistent leucopenia and
positive anti-nuclear antibody, secondary to concomitant systemic lupus erythematosus, which
accounted for the foot drop at the onset.

Conclusion: This case highlights that tonic spasms can be the initial manifestation of NMOSD,
in the absence of overt myelitis or optic neuritis. Treatment with carbamazepine usually results
in prompt resolution of PTS.

283
OT014. NEUROBEHAVIOR DISORDERS AND CHRONIC FATIGUE
ASSOCIATED WITH AUTOIMMUNE DISEASE: A RARE CASE OF MULTIPLE
SCLEROSIS

Gyan Adytya*1 ; Astuti1 ; Abdul Gofir1 ; Sudarmanta2


1Neurology Gadjahmada University, Gadjah Mada University, 2Department Of Radiology

Gadjah Mada University, Gadjah Mada University, Indonesia


Email Address: gyanadytya@gmail.com

Introduction: Neurobehavior disorders and chronic fatigue are symptoms that should be
explored. The causes of neurobehavior disorders and chronic fatigue can be various, one of
which is autoimmune causes. Among the autoimmune diseases that cause these symptoms,
the most common is SLE and Multiple Sclerosis. To establish a diagnosis of multiple sclerosis,
MRI examination can be done because of its very high sensitivity and specificity and then
appropriate therapy is carried out on the patient.

Results: A 26-year-old male with a history of cognitive impairment in the attention domain,
memory, depression and chronic fatigue for 7 years came to the neurological clinic. Previous
examination history was suspected of being SLE but on a more detailed examination, there
was a suspicion of Multiple Sclerosis. Then, a cerebal MRI examination was performed and a
suspicion of early multiple sclerosis was noted in juxtacortical frontal lobe bilaterally, bilateral
occipitalis, periventricular right anterior horn, with white matter lesion score (Fazekas) 1.
Conservative therapy with analgesics and corticosteroids in patients ended good results
without significant complications.

Conclusion: Neurobehavior disorders and chronic fatigue are symptoms of Multiple Sclerosis
that must be considered, the cerebral MRI diagnostic approach can help make the diagnosis
because of its high sensitivity and specificity.

284
OT015. MONITORING PREFRONTAL CORTICAL ACTIVATION WHILE
DRIVING A VEHICLE: A FUNCTIONAL NEAR-INFRARED SPECTROSCOPY
STUDY

Daisuke Hirano*1 ; Naotoshi Kimura2 ; Hana Nozawa1 ; Miku Enoki3 ; Yoshinobu Goto4 ;
Takamichi Taniguchi1
1Department of Occupational Therapy, International University of Health and Welfare, Japan,
2Department of Rehabilitation, International University of Health and Welfare Ichikawa

Hospital, Japan, 3Department of Rehabilitation, International University of Health and Welfare


Shioya Hospital, Japan, 4School of Medicine, International University of Health and Welfare,
Japan.
Email Address: dhirano@iuhw.ac.jp

Introduction: Functional near-infrared spectroscopy (fNIRS) is a non-invasive method to


measure changes in oxygenated- and deoxygenated-hemoglobin concentrations in response
to neural activation during specific tasks and stimuli. In this study, we monitored the brain
activity of the prefrontal cortex in subjects while driving to investigate the effects of speed on
brain activity.

Methods: Twenty healthy subjects with driving experience drove their own vehicles about 100
meters at any speed (5 trials) and at highest acceleration speed (5 trials) and were told to stop
at the stop line. Sixteen channels of fNIRS recording were placed to overlie the prefrontal
cortex of the subjects during driving. The distance between the stop line and the vehicle when
the vehicle comes to a stop and oxygenated hemoglobin concentration was compared
between driving at any speed and driving at the highest acceleration.

Results: Variation in the distance between the stop line and the vehicle was significantly
greater when driving at the highest acceleration compared to driving at any speed.
Oxygenated hemoglobin concentration recorded in the prefrontal cortex was significantly
greater while driving a car to a stop at the highest acceleration than at free speed.

Conclusion: Our findings suggest that driving at highest acceleration speeds requires more
attention resources than driving at any speed. We have shown that monitoring brain activity
using fNIRS has advantages and usefulness of in real driving.

285
OT016. THE COMPARISON OF ROBOTIC GLOVE TRAINING AND
CONVENTIONAL TRAINING IN POST-STROKE HAND IMPAIRMENT
PATIENTS: A PILOT RANDOMIZED CONTROLLED TRIAL PROTOCOL

SA Albart*1 ; HC Loh1 ; M Chin2 ; I Looi3


1Clinical Research Centre (CRC), Seberang Jaya Hospital, Malaysia, 2Physiotherapy Unit,

Seberang Jaya Hospital, Malaysia, 3Medical Department, Seberang Jaya Hospital, Malaysia
Email Address: annstephenie27@gmail.com

Introduction: Rehabilitation in stroke patients is crucial in minimizing the neurological deficit,


improving their quality of life and reducing the caretakers’ burden. Post-stroke hand
impairment is common and improving hand function is a core element of rehabilitation. For
such patients, robotic glove training might be a promising tool. This trial aims to determine the
efficacy, safety, feasibility and cost effectiveness of Robotic Glove Training (RGT) compared
with Conventional Training (CT) among post-stroke unilateral hand impairment patients.

Methods: This is a pilot, randomized, assessor blinded, controlled trial. Adult patients with first
onset of stroke with unilateral poststroke hand impairment (Medical Research Council Grading
≤ 3) will be selected. Subjects will be randomized into RGT or CT group in a 1:1 ratio. Both
groups also will undergo the usual daily home hand exercises. The endpoints will be measured
after 6 weeks of training.

Results: For efficacy assessment, hand grip strength; tip, key and palmar pinch strength; and
hand motor function will be measured using Hand Dynamometer; Pinch Gauge; Motor
Assessment Scale and Fulg Meyer Assessment. For safety assessment, hand spasticity and
pain will be assessed using Modified Ashworth Scale and Ministry of Health Pain Scale. For
feasibility assessment, adverse event and level of operator difficulty in handling the robotic
glove device will be assessed. For cost effectiveness, the average cost per hour of a
physiotherapist spend during training will be analyzed. Independent t-test or Mann-Whitney
test will be used to compare the two mean scores and a P value of < 0.05 is considered
statistically significant.

Conclusion: We hypothesized that RGT group will show better improvement in terms of hand
grip strength, pinch strength and hand motor function compared to CT group among post-
stroke unilateral hand impairment patients. We also expected that RGT will be safe, feasible
and cost effective.

286
OT017. NEUTROPHIL-TO-LYMPHOCYTE RATIO, MONOCYTE-TO-
LYMPHOCYTE RATIO, AND PLATELET-TOLYMPHOCYTE RATIO
BIOMARKERS IN SEVERE HEMORRHAGIC STROKE PATIENTS: WHICH
RATIO TO CHOOSE AS A MORTALITY PREDICTOR?

Adhi Hermawan Saputro*1 ; Abdul Gofir1 ; Paryono1 ; Astuti1


1Departement of Neurology, Faculty of Medicine, Public Health And Nursing, Universitas

Gadjah Mada, Yogyakarta, Indonesia


Email Address: adhiher.dr@gmail.com

Introduction: Several biomarkers are now being developed to predict the prognosis of stroke.
Ratios of the white blood’s components are widely studied as short-term and long-term
prognostic factors in patients with intensive care. Stroke, particularly severe hemorrhagic
stroke, is a condition that needs intensive care that has the potential to worsen anytime in its
acute period and eventually leads to death. Here, we studied the neutrophil-to-lymphocyte ratio
(NLR), monocyte-to-lymphocyte ratio (MLR), and platelet-to-lymphocyte ratio (PLR) on-
admission as potential predictors of in-hospital mortality in severe hemorrhagic stroke patients.

Methods: It is a retrospective observational study in severe hemorrhagic stroke with


conventional treatment. We reviewed the medical records to obtain clinical and laboratory data
and divided the patients into survivors vs non-survivors. The laboratory data were searched for
white blood cell (WBC) count, neutrophil count, lymphocyte count, monocyte count, and
platelet count. The NLR, MLR, and PLR were then manually estimated.

Results: Of the 30 eligible patients, 25 patients were survivors and 5 patients were non-
survivors. In the laboratory examinations, the neutrophil, lymphocyte, and monocyte counts
showed a significant difference between survivors and non-survivors. Furthermore, we also
found that NLR and MLR were two ratios that had significant difference statistically. Also, if we
used the NLR cut-off point of 5 to separate high and low NLR, we found that NLR on-
admission is associated with in-hospital mortality.

Conclusion: NLR and MLR on-admission were significantly different between survivors and
non-survivors of severe hemorrhagic patients that were treated conventionally. Also, NLR on-
admission > 5 could be associated with in-hospital mortality.

287
OT018. DEMOGRAPHIC CHARACTERIZATION OF PATIENTS WITH
COGNITIVE IMPAIRMENT

Monica Danial*1 ; Ng Li Yun1 ; Alan Ch'ng Swee Hock1 ; Irene Looi1


1Clinical Research Center, Hospital Seberang Jaya, Malaysia

Email Address: monicadanialjsm@gmail.com

Introduction: As Malaysia undergoes population ageing, the prevalence of dementia is


expected to increase, imposing a major public health issue and serious burden to patients,
their caregivers, and the government. However, data on dementia prevalence is lacking and
little is known about the demographic characteristics of dementia in Seberang Perai area.

Methods: A cross-sectional observational study was conducted at the Neuro-geriatric Clinic,


Hospital Seberang Jaya from November 2018 to February 2020. Case notes were reviewed
and demographic characteristic and diagnosis of either MCI or types of dementia were
recorded

Results: A total of 173 patients with cognitive impairment included in this study. The majority
were Chinese (66%), male (63%), aged 71-80 years old (32%), and diagnosed with
Alzheimer's disease (AD) (35%). Among male patients, Parkinson’s Disease Dementia (PDD)
(37%) was the commonest, followed by Alzheimer disease (AD) (26%), Vascular Dementia
(VaD) (24%), MCI (11%) and Lewy body disease (3%). On contrary, among female patients,
AD (52%) was the commonest, followed by PDD (17%), VaD (13%), MCI (7%) and Lewy body
disease (2%). Based on race category, majority of the patients were diagnosed with AD with
Malay (31%), Chinese (37%) and Indian (33%) compared to other types of cognitive
impairment. In terms of age category highest number of AD were associated with age group of
71-80 years old (52%).

Conclusion: Among patients with cognitive impairment on follow-up at the Seberang Jaya
Hospital Neuro-geriatric clinic, the majority were 71-80 years old, Chinese, male and
diagnosed with AD. Diversity in health literacy and health seeking behaviour among ethnic
groups and gender needs to be further explored.

288
OT019. CLINICAL CHARACTERISTICS OF PATIENTS WITH BRAIN
METASTASES IN DR SARDJITO GENERAL HOSPITAL YOGYAKARTA,
INDONESIA

Briliansy*1 ; Ahmad Asmedi1 ; Kusumo Dananjoyo1


1Neurology, Sardjito General Hospital, Indonesia

Email Address: briliansy@gmail.com

Introduction: Dissemination of systemic carcinoma to the brain continues to present a poor


prognosis. Knowledge of the metastatic patterns and limited survival associated with specific
tumor types may be useful for guiding future therapeutics.

Methods: This study is non-analytical quantitative descriptive with cross-sectional approach,


retrospectively taking secondary data from medical record of brain metastases patients.

Results: There were 278 cases of brain metastases. The most frequent distribution of age was
age < 55 years (n=171; 61,5%), with average of 50.6 years, sex was female (n=182; 65,7%),
the most common primary tumor was breast cancer (n=49; 28%) and NSCLC (n=33; 18,8%),
the most common characteristic of lesion found on imaging was single lesion (n=159; 72,6%),
focal edema (n=151; 66,8%), and supratentorial location (n=138; 71,8 %)

Conclusion: In our population, brain metastases was most frequent in age < 55 years, female;
the most common primary tumors were breast and lung cancer, with most common lesion was
single lesion involving supratentorial region with focal edema.

289
OT020. PROFILE AND MORTALITY RATE OF CEREBROVASCULAR
ACCIDENT IN PATIENT WITH ACUTE MYELOID LEUKEMIA IN DR.
SARDJITO HOSPITAL YOGYAKARTA

Rizka Ibonita*1 ; Ismail Setyopranoto2 ; Indarwati Setyaningsih2 ; Siti Farida Setyaningrum3


1Resident At Department Of Neurology, Gadjah Mada University, Indonesia, 2Staff At

Department Of Neurology, Gadjah Mada University, Indonesia, 3Neurologist, RSU Banyumas,


Indonesia
Email Address: rizkaibonita@gmail.com

Introduction: The incidence of cerebrovascular accident (CVA) in Acute Myeloid Leukemia


(AML) patients tends to increase worldwide. AML was 50-fold increase the risk of CVA. In a
previous study, CVA patients with AML were found to have significantly higher inpatient
mortality. It has serious implications both medically and financially and reduces the patient's
quality of life. The aim of this study was to determine the CVA profile in AML patients and the
incident of mortality.

Methods: A cross sectional descriptive study was conducted based on secondary data from
the medical records inpatient Dr. Sardjito Hospital Yogyakarta from January 2015 until
December 2019. Samples were part of the population that fulfilled inclusion criteria. CVA was
diagnosed based on clinical examination findings and head computed tomography (CT) scan.

Results: There were 31 subjects, consisted of 17 (54.8%) males and 14 (45.2%) females. The
mean age was 48 (± 16.72) years old. There were 22 (71%) subjects diagnosed as
intracerebral hemorrhage and 9 (29%) as brain infarction. There were 22 (71%) with high
leukocytosis and 26 (83%) with very low thrombocyte count. In-hospital mortality in
intracerebral hemorrhage subjects were 48.4% (15 of 22) and brain infarction were 19.4% (6 of
9), with the highest percentage of mortality in very low count of thrombocyte (58.1%) and high
leukocytosis (51.6%).

Conclusion: The most common type of CVA was intracerebral hemorrhage, with higher
incidence in middle age because of the highest incidence of AML in that age group. The
highest mortality was mostly found in intracerebral hemorrhage and very low count of
thrombocyte because of disturbances in intrinsic factors and high leukocytosis, which can lead
to leukostasis in the endothelial wall causing rupture of the vessel endothelium.

290
OT021. INTRACEREBRAL HEMORRHAGE IN INFECTIVE ENDOCARDITIS,
WITH DRAMATIC RESPONSE TO DEXAMETHASONE: A CASE REPORT

Distya Nugrahening Pradhani*1 ; Ismail Setyopranoto2 ; Indarwati Setyaningsih2 ; Sri Sutarni2


1Resident of Neurology Department, Faculty of Medicine, Public Health and Nursing,

Universitas Gadjah Mada, Indonesia 2Staff of Neurology Department, Faculty of Medicine,


Public Health and Nursing, Universitas Gadjah Mada, Indonesia
Email Address: distya.np@gmail.com

Introduction: Intracerebral hemorrhage (ICH) in infective endocarditis (IE) patient is a rare


condition. This case describes the clinical features and management of ICH due to IE with
favorable outcome.

Results: A 19-year-old woman, diagnosed for IE and ventricular septal defect (VSD)
undergoing treatment with Levofloxacin 2x500 mg orally, presented to our hospital with
decreased level of consciousness for 2 days following weakness of right limbs and acute
communication difficulty. Physical examination: stupor, right lateralization, positive Babinski
sign of right extremity. A non-contrasted head computerized tomography scan (HCTS) image
revealed 20 cc ICH in the left frontotemporoparietal lobe with subfalcine herniation.
Echocardiography: multiple vegetation in tricuspid valve and VSD. Streptococcus
mitis/Streptococcus oralis were confirmed by the blood cultures. We prescribed 5 mg
dexamethasone injection every 6 hour, tapered off after 3 days and replaced the antibiotics
with Cefotaxime injection 1 gram every 8 hours. On day 14, HCTS evaluation showed
improvement with 4.2 cc ICH in the left frontotemporoparietal lobe with significant improvement
of the clinical state. Patient was discharged on day 39 with sequelae of UMN paresis of the 7th
and 12th right cranial nerves.

Conclusion: ICH in IE may be caused by hemorrhagic transformation, mycotic aneurysm


rupture, pyogenic/immune complex arteritis, and/or septic emboli that causes erosive arteritis
with ruptured vassels, requiring comprehensive management. Clinical improvement after
administration of steroid explained the neurologic deficit was due to a vasculitis mechanism,
although there are no guidelines regulate the use of steroids in IE patients with ICH. Cardiac
surgery may consider to be delayed at least 4 weeks if ICH is found except there is an urgent
indication.

291
OT022. NEUROIMAGING FEATURES OF HIV-POSITIVE PATIENTS WITH
CENTRAL NERVOUS SYSTEM INFECTION IN DR SARDJITO HOSPITAL,
YOGYAKARTA, INDONESIA

Hanifah Fajarisna Hayati*1 ; Sekar Satiti1 ; Cempaka Thursina Srie Setyaningrum1 ; Milasari
Dwi Sutadi1
1Department Of Neurology, Gadjah Mada University, Indonesia

Email Address: hanifahfh@gmail.com

Introduction: Central Nervous System (CNS) infection, including meningitis and encephalitis,
is a life-threatening disease, especially in developing countries. The aetiologies are bacterial,
viral, fungal, or parasite needing different treatments. Patients with HIV positive are at higher
risk for the development of CNS infections. Neuroimaging, such as Head CT scan and MRI, is
important to help the diagnosis. This study is to describe the features of neuroimaging related
to the diagnosis in HIV-positive patients with CNS infection.

Methods: A retrospective descriptive design was used in this study. Sixty adult patients
confirmed for HIV-positive with CNS infection were described for the neuroimaging features
(Head CT scan and MRI) and related diagnoses. The data was collected from January 2019
until September 2020 in Dr Sardjito hospital.

Results: The prevalence of HIV-positive in CNS infection was 36% (2019 in Dr Sardjito
Hospital). The most common neuroimaging features was pathological enhancement found in
30 cases (50%). The second feature was cerebral edema (27 cases, 45%), followed by
abscess (18 cases, 30%). The other features were focal mass lesion 25%, hydrocephalus
21.6%, cerebral atrophy 13.3%, cerebral infarct 13.3%, tuberculoma 13.3%, midline shift
13.3%, hemorrhagic 1.6%, and normal finding in 11.6%. The diagnoses found in pathological
enhancement were cerebral toxoplasmosis (13 patients, 43%), cerebral abscess (8 patients,
26.6%), bacterial meningitis/encephalitis (6 patients, 20%), and unspecified
meningitis/encephalitis. The same diagnoses presented in feature of cerebral edema.
However, the abscess feature was mostly found in cerebral abscess (9 patients, 50%),
cerebral toxoplasmosis (6 patients, 33%), and bacterial meningitis/encephalitis (3 patients,
17%). The other features showed a variety of diagnoses.

Conclusion: HIV-positive infection showed various features of neuroimaging. The most


common features were pathological enhancement, cerebral edema, and abscess. Cerebral
toxoplasmosis, cerebral abscess, and bacterial meningitis/encephalitis were the most common
diagnoses in HIV-positive patients with CNS infection.

292
OT023. INTRACRANIAL SUBDURAL EMPYEMA MIMICKING ACUTE ON
CHRONIC SUBDURAL HEMATOMA: A CASE REPORT

Mawaddah Ar Rochmah*1 ; Abdul Gofir1 ; Sekar Satiti1 ; Tommy Rachmat Setyawan1 ;


Adimas Endro Wibisono1 ; Endro Basuki1
1Department of Neurology, Faculty of Medicinie, Public Health, and Nursing Universitas

Gadjah Mada Yogyakarta, Indonesia


Email Address: mawaddah_ar@ugm.ac.id

Introduction: Intracranial subdural empyema is a rare localized intracranial infection that


forms between dura and arachnoid membrane of the meninges. Its unspecific clinical
presentations as well as neuroimaging findings that might resemble subdural hematoma make
the definitive diagnosis of subdural empyema difficult. This report aims to highlight the
importance of making a differential diagnosis of subdural empyema in a suspected case of
subdural hematoma.

Results: A 71 years old male presented with acute mild to moderate intermittent headache
that was followed by sudden difficulty in word findings and right limbs weakness. No history of
fever, trauma, seizure, or prior history of ear, nose, throat, tooth infections, and intracranial
surgical procedure. Increased white blood cells count was not found despite the increased
neutrophil to lymphocyte ratio as well as C-reactive protein. Non-contrast head CT-scan
showed crescentic hyperdense lesion within a wider hypodense lesion in the left
frontotemporoparietal region, characterizing acute on chronic subdural hematoma. During burr
hole drainage, pus was found, consistent with subdural empyema. An intralesion antibiotic
treatment was given after complete removal of the pus.

Conclusion: Intracranial subdural hematoma and subdural empyema with the involvement of
neurological deficits are emergency conditions. However, it is difficult to differentiate these two
conditions even after the examinations of clinical, laboratory, and neuroimaging with non-
contrast head CT-scan, particularly without the triad of subdural empyema: fever, headache,
vomiting. Our case showed that even without noticeable port d’entry, intracranial subdural
empyema could be formed.

293
OT024. STROKE SUBTYPES AND IN-HOSPITAL MORTALITY IN PATIENTS
WITH SYSTEMIC LUPUS ERYTHEMATOSUS IN DR. SARDJITO HOSPITAL
YOGYAKARTA

Ima Damayanti*1 ; Ismail Setyopranoto1 ; Paryono1 ; Anton Darmawan2


1Department of Neurology, Faculty of Medicine, Public Health, and Nursing, Universitas

Gadjah Mada, Indonesia, 2Neurologist, RSUD Wates, Indonesia


Email Address: ima.damayanti89@gmail.com

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that


associated with an increased risk of stroke especially in young patient. Individuals with SLE
have a two-fold higher risk of stroke infarct and three-fold higher for intracerebral hemorrhage.
Survival after stroke in SLE patient is lower compared to the general population. This study
was conducted to determine the subtypes of stroke and in-hospital mortality in patients with
SLE.

Methods: A descriptive cross-sectional study. Subjects were SLE patients that had stroke and
hospitalised at Dr. Sardjito Hospital from January 1st, 2015 until December 31st, 2019.
Patients were diagnosed with SLE based on American College of Rheumatology Classification
Criteria and stroke diagnosis was made by neurological examination and imaging finding (head
CT scan/MRI).

Results: All of the subjects were female with total 23 patients. The mean age was 33,65
(12,86) years old. There were 16 (69,6%) subjects having stroke infarct, 6 (26,1%) subjects
having intracerebral hemorrhage and only one (4,3%) having subarachnoid hemorrhage. The
most common subtypes of stroke infarct based on Oxfordshire Community Stroke Project
(OCSP) classification was partial anterior circulation infarction (PACI) that was found in 11
(68,8%) subjects. In-hospital mortality was found in 10 (43,5%) subjects which 7 (30,4%) of
them have had stroke infarct and 6 (26,0%) of them were categorized into age group below 40
years old.

Conclusion: Subject in this study was female predominance. It was because SLE typically
affects females rather than males. The most common subtypes of stroke was partial anterior
circulation infarction. SLE could trigger the formation of blood clots that leads to inflammatory
changes in blood vessels, which create a prothrombotic state, and increase risk of
thromboembolism. In-hospital mortality was mostly found in stroke infarct and young subjects.

294
OT025. A RARE CASE OF DENGUE ENCEPHALOPATHY IN PREGNANT
WOMAN

Fahreza Hanifa Akbar*1 ; Kusumo Dananjoyo1 ; Amelia Nur Vidyanti1 ; Subagya1


1Resident Department of Neurology, Faculty of Medicine, Public Health, and Nursing

Universitas Gadjah Mada, Indonesia


Email Address: fahrezahanifa@gmail.com

Introduction: Dengue fever has been known for more than two decades as a tropical endemic
infection caused by the dengue virus that affected from Aedes aegypti. Complications of
dengue fever are common and usually related to renal and hepatic dysfunction.
Encephalopathy is a rare complication. Here we report an unusual case of pregnant woman
with dengue encephalopathy.

Results: A 28-year-old female, G1P0A0, consulted by the anesthetic division with loss of
consciousness. Prior to that, she had acute fever with history of acute progressive cephalgia
and positive results of IgG and IgM dengue antibody. She was 20 weeks of pregnancy. On
examination she was stuporous (E2V2M5). Lateralization, pathological reflex, and meningeal
signs were not found. Laboratory examination showed thrombocytopenia 23 X 103 /µl,
increased transaminase enzyme, hypokalemia 2.53 mmol/L, and hypoalbuminemia 2.35 gr/dL.
Head CT Scan showed cerebral edema. During hospital care, she was monitored closely.
Careful fluid management was carried out with additional treatment consisted of thiamine 100
mg/12hours, piracetam 800 mg/12hours, folic acid 2x1 mg, and transfusion of albumin and
premix KCL. Patient condition was improved after 7 days of hospital care. The encephalopathy
lasted for 5 days. Patient was discharged with full of consciousness and the baby was safe.

Conclusion: Encephalopathy is a rare manifestation of DHF. However, it is a significant


diagnostic challenge when it presents in atypical form, especially during pregnancy. It was first
reported in 1976, and worldwide literature search shows the various incidence of dengue
encephalopathy from 0.5% to 6.2% with a mortality rate up to 40%. The complications of
dengue in pregnancy have been scarcely studied. There are increased risks of maternal
hemorrhage, preterm labor and stillbirths. Dengue should be the differential diagnosis even a
pregnant women presents with fever and altered sensorium especially in endemic areas like
Indonesia.

295
OT026. EPIDEMIOLOGICAL TRENDS ON THE RISK FACTORS AND
OUTCOMES OF STROKE

Lim Soo Ping*1 ; Loh Hong Chuan2 ; Kurubaran Ganasegeran2 ; Zariah Abdul Aziz3 ; Irene
Looi4
1Hospital Seberang Jaya, Malaysia, 2Clinical Research Centre, Hospital Seberang Jaya,

Malaysia, 3Medical, Clinical Research Centre, Sultanah Nur Zahirah Hospital,


Malaysia, 4Medical, Clinical Research Centre, Hospital Seberang Jaya, Malaysia
Email Address: soopinglim@yahoo.com

Introduction: Malaysia observed a declining trend of stroke related deaths since 2009. Age
adjusted death rates for stroke was 71.5 deaths per 100,000 population. This study aimed to
determine the epidemiological trends on the risk factors and outcomes of stroke based on the
World Health Organization (WHO) classification of stroke.

Methods: This observational hospital-based study was conducted at the Seberang Jaya
Hospital (HSJ) from January 2013 to December 2018. A total of 1373 stroke patients upon
admission to the hospital that were registered under the National Neurology Registry of Stroke
were recruited.

Results: The mean (SD) age of the patients was 61.6 (12.5) years. Younger aged population
were predominantly being afflicted with stroke over the past few years. The majority of the
patients were men (62.9%) and Malays (53.7%). Hypertension was the main risk factor
(69.2%), with the number of cases decreasing over the years. Diabetes mellitus was showing a
similar trend. Hyperlipidaemia and ischaemic heart disease accounted for about 20% of the
total cases and plateaued over the years. It was observed that smoking was on the rise over
the course of the study period. Ischaemic stroke (80%) and first-ever stroke (77.9%) were
more commonly encountered as compared to haemorrhagic stroke and recurrent stroke
respectively.

Conclusion: Up-to-date public health actions, especially on primary prevention is crucial in


order to reduce the impact of stroke on people’s health.

296
OT027. APPROACH AND MANAGEMENT OF SUSPECTED ENCEPHALITIC
PATIENTS IN OMDURMAN TEACHING HOSPITAL

Mohammed Eltahier Abdalla Omer*1


1Neurology, Gadarif University, Sudan

Email Address: Mohammedeltahier100@gmail.com

Introduction: The aim of this study was to assess the approach and management of
suspected encephalitic patients in Omdurman teaching hospital.

Methods: This was a cross-sectional descriptive study, conducted on 84 patients who are
suspected of encephalitis in neurology department. The data was analyzed using SPSS.

Results: The commonest group of age affected (21 - 40) representing about 66.7% with
84.5% female predominant and most of the patients from Khartoum state (58.3%). Regarding
the main presenting symptoms, 100% presented with fever and headache, 36.9% with seizure
and 28.6% with agitation, other symptoms like abnormal behavior, memory disturbance,
photophobia, neck stiffness and nausea / vomiting ranging from (22.6% - 8.3 %). 1.2% has a
history of SLE and also 1.2% presented with bilateral 6th cranial nerve lesion. 28.6%
presented with unconsciousness and disoriented. 13.1% comatose with 20.2% had personality
changes and memory disturbance. Regarding upper and lower limbs examination 13.1%
hemiparesis, 4.8% quadriparesis and 1.2% paraparesis. 100% did a CBC showed lymphocyte
predominant about 70.2%, 100% elevation in ESR. 71 patients did a CSF analysis with 83.1%
showed abnormality, increasing lymphocyte cells, others were normal. MRI/CT scan was done
in 50 patients;, in 63.2% MRI showed temporal hyperintense lesion, whereas CT scan was
normal in majority of cases (71%). EEG was abnormal in 3.6% of cases. Most of the cases
were managed empirically with antiviral and antibiotics, 100% and 94% respectively. About
85.7% received antiepileptic. The most common complication was seizure (85.7%). 26.2%
ended up with motor deficit. Fortunately, most of the cases completely recovered (65.5%).

Conclusion: The diagnostic approach to patients with encephalitis must include CSF,
neuroimaging (MRI) plus or minus EEG. Empirical antimicrobial therapy for patients with
suspected encephalitis should include rapid administration of intravenous acyclovir at
appropriate dosages if clinically suspected despite unobtainable CSF result. We must identify
the exact etiology and once an etiologic agent of encephalitis is identified, antimicrobial
therapy should be targeted to that infectious agent, or therapy should be discontinued if
treatment directed against the etiologic agent is not available.

297
OT028. CLINICAL FEATURES OF HIV PATIENTS WITH TOXOPLASMOSIS
INFECTION AT DR. SARDJITO GENERAL HOSPITAL YOGYAKARTA

Deslita Purnamasari*1 ; Sekar Satiti2 ; Desin Pambudi2 ; Djoko Kraksono2


1Neurologist Trainee, Department of Neurology, Sardjito General Hospital, Yogyakarta,

Indonesia, Faculty of Medicine, Public Health, and Nursery Universitas Gadjah Mada,
Indonesia, 2Staff Department of Neurology, Sardjito General Hospital, Yogyakarta, Faculty of
Medicine, Public Health, and Nursery Universitas Gadjah Mada, Indonesia
Email Address: desti.deslita@gmail.com

Introduction: Toxoplasma gondii is an obligate intracellular protozoan parasite presenting as


a zoonotic infection distributed worldwide. In HIV-positive individuals, it causes severe
opportunistic infections, which is of major public health concern as it results in physical and
psychological disabilities. The number of HIV sufferers in Yogyakarta is high, from 1993 to
2019 there were a cumulative number of 4990 cases in Yogyakarta. The objective of this study
is to determine the clinical features of HIV patient with toxoplasmosis infection at Dr. Sardjito
General Hospital Yogyakarta.

Methods: This cross-sectional observational descriptive study was carried out at Dr. Sardjito
General Hospital Yogyakarta from October 26, 2018 to October 18, 2020, with purposive
sampling method. The subjects were HIV inpatient with positive serology tests for IgM and IgG
anti-toxoplasma, we collected data about the patient's history and clinical condition.

Results: Ninety-one subjects included in this study consisted of male 62 (68.1%) and female
29 (31.8%) with a mean age (36.6 ± 12.5 years) having complaints and clinical conditions such
as headache 66 (72.5%), decreased consciousness 23 (25.3%), fever 23 (25.3%), disability 21
(23.1%), aphasia 19 (20.9%), changes in behavior 16 (17.6%), seizures 9 (9.9%), neck
stiffness 9 (9.9%), sensory disturbances 5 (5.5%), dementia 5 (5.5%), blurred vision 3 (3.3%),
and autonomic dysfunction 2 (2.2%).

Conclusion: The most common signs of HIV patients with toxoplasmosis was headache, while
the most severe clinical condition was decreased consciousness. HIV sufferers with
headaches should be suspected of having toxoplasmosis.

298
OT029. CLINICAL NEUROPHYSIOLOGY SOCIAL MEDIA ENGAGEMENT
DIFFERS BETWEEN FACEBOOK AND TWITTER USERS

Tuhfatul Tasnim*1 ; Hirotaka Iwaki1 ; Eishi Asano1


1Pediatrics, Wayne State University, United States

Email Address: go3699@wayne.edu

Introduction: In an age where social media is prevalent and long hours are spent browsing
through the internet, it is crucial to understand audience engagement. Two of the most
common platforms used worldwide are Twitter and Facebook. Both have provided a unique
opportunity for the science world; it broadened the horizons for spreading information on
current endeavors in the research world. Clinical Neurophysiology and Clinical
Neurophysiology Practice, the Official Journals of the International Federation of Clinical
Neurophysiology, opened the Twitter and Facebook pages on April 1, 2020. We determined
what factors would alter users’ engagement.

Methods: We employed a multivariate regression analysis. The tested predictor variables


included ‘days elapsed since the opening (range: 1-100)’, ‘posting during weekend’,
‘introduction of a 2020 article’, ‘author interview’, ‘inclusion of guidelines or review article’,
‘COVID19-related information’, ‘number of hashtags’, ‘number of attachments’, ‘presence of
colored attachment’, and ‘attachment of movie’. The dependent variable was the proportion of
engaged users, defined as the number of link clicks divided by users who saw the post.

Results: Posts including author interviews (p<0.001) and those introducing guidelines/review
articles (p=0.005) independently increased the proportion of engaged Facebook users.
Conversely, posts including a larger number of colored attachments (p<0.001) and those
introducing COVID19-related articles (p=0.007) independently increased the proportion of
engaged Twitter users.

Conclusion: Different types of posts attracted Facebook and Twitter users. Facebook users
were specifically engaged with interview-based posts. Facebook users might be willing to read
through lengthy posts to understand the authors’ stories behind the study. In contrast, Twitter
users might want to absorb essential information with a glance.

299

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