The Northern Neuroscience Centre Chiangmai University

NNC MU
Movement disorder
tutorial
Surat Tanprawate, MD, MSc (London), FRCP(T)
Division of Neurology, Chaingmai University
Neurological symptoms

• Conscious and cognitive • Dysfunction of motor system
functions

• alteration of conscious • weakness
• higher cortical function • ataxia
disorder • movement disorder
• dementia • Dysfunction of sensory system
• Cranial nerve functions • numbness
• anosmia, visual loss, • pain
diplopia, CN V dysfunction,
facial weakness, hearing • Autonomic dysfunction
loss, tinnitus, dysphagia,
tongue weakness
• Episodic disorder
Neurological diseases

• Congenital • Vascular
• Trauma • Metabolic/Toxic/Drugs
• Tumor • Genetic
• Infection/inflammation • Cryptogenic
• Degeneration • Idiopathic
• Demyelination
What is movement disorder?

• The term : movement disorders: originally refer
to basal ganglia or extrapyramidal diseases

• slowness or poverty of movement
(bradykinesia or hypokinesia (e.g.
parkinsonian disorders)

• abnormal involuntary movements
(hyperkinesias) such as tremor, dystonia,
athetosis, chorea, ballism, tics, myoclonus,
restless legs syndrome, stereotypies,
akathisias, and other dyskinesias
What is movement disorder?

• Some may similar movement disorder
• abnormalities in muscle tone (e.g.rigidity,
spasticity, and stiff man syndrome),

• incoordination (cerebellar ataxia)
• apraxia
• seizure
Anatomy of movement control
Direct and Indirect pathway of movement control
Step approach
3 question should be asked
1.Is it hypokinetic or hyperkinetic
movement disorder?
2.What is the pattern of movement
disorder?
3.What is the classification of such
movement disorder?
Movement disorder

Hypokinetic Hyperkinetic

Pattern of movement
Hyperkinetic disorder
rigid syndrome
Classify by anatomy,
Hypokinetic distribution, cause,
rigid syndrome age
Hyperkinetic
movement disorder
• Rhythmic, sustained, intermittent,
speed, suppressibility, complex
movement

• Tremor, Chorea, athetosis, dystonia,
myoclonus, ballism, tic
Chorea = dance
irregular, nonrhythmic, unsustained
involuntary movement that flows from
one part of the body to another

“motor impersistence”
Dancing lady
Dystonia
syndrome of sustained muscle
contractions, frequently causing twisting,
repetitive movements, or abnormal
postures

“sustained contractions, consistent directional
or patterned character (predictable), and
exacerbation during voluntary movements”
“sensory trick”
Generalised dystonia
Myoclonus
sudden, brief, jerky, and shock-like
involuntary movements involving face,
trunk, and extremities

“positive myoclonus”
“negative myoclonus”
Generalised myoclonus
Tremor
a rhythmic oscillation of a body part
produced by alternating or synchronous
contraction of opposing muscles

other movement clinical symptoms can be act
like tremor: dystonic tremor, myoclonic tremor
Tremor
Tics
repetitive, stereotyped, involuntary,
sudden, inopportune, non-propositional,
and irresistible movement

“unpleasant feeling
“not absolutely clear as patients can exert some control
on the movement”
“can be simple or complex”
He have had tic since he
was 10 y.o.
Ballism=dacing
involuntary, flinging motions of the extremities,
the movement are often violent and have wide
amplitude of motion, continuous and random,
can involve proximal or distal
Athetosis = without fixed
position
involuntary, convoluted, writhing, slow
movements of the arms, fingers and legs
“Pseudoathetosis” in a patient with severe axonal
polyneuropathy
Common movement
disorder
Tremor
Step approach- MDS consensus

1.Inspection the tremor
2.Specific examination for assessment of
signs related to tremor
3.Syndrome classification of tremor
Terminology for tremor and the
hierarchical relation of the terms as
indicated by the numbers
Inspection

• Location
• Frequency • Head: chin, face, tongue,
• Low (<4 Hz) palate

• Medium (4-7 Hz) • Upper extremity: shoulder,
elbow, wrist, fingers

• High (>7 Hz) • Trunk

• Lower extremity: hip,
knee, ankle joint, toes
Specific examination for
assessment of:
• Akinesia/bradykinesia
• Muscle tone (including Froment’s sign for the upper
and lower extremity and coactivation sign for
psychogenic tremor)

• Postural abnormalities
• Dystonia
• Cerebellar signs
• Pyramidal signs
• Neuropathic signs
• Systemic signs (thyrotoxicosis and so forth)
• Gait and stance (orthostatic tremor)
Syndrome classification of tremor

Syndrome Activity Specific S/S Cause

Physiologic tremor Postural No Physiologic response

Enhance physiologic Hyperthyroid,
Postural, Kinetic Hyperthyroid, drugs
tremor tachycardia

Essential tremor Postural, Kinetic No No

Bradykinesia,
Parkinsonian tremor Rest postural instability, Neuro-degeneration
rigidity
Various cause
Postural, kinetic,
Cerebellar tremor Ataxia affected cerebellar
intention
pathway
Syndrome
classification of
tremor

Tremor description
(activated by, location,
frequency)
+
Specific s/s
Characteristics of Essential Tremor and Parkinsonian Tremor
Essential tremor
Core criteria for identifying ET

• Bilateral action tremor of the hands and
forearms

• Absence of other neurological signs, with the
exception of the cogwheel phenomenon

• May have isolated head tremor with no
abnormal posture
Essential tremor

Secondary criteria for identifying ET

• Long duration (>3 years)

• Family history: reported in > 50% of the
patients

• Beneficial response to ethanol
Essential Tremor
Achimedes spiral
Achimedes spiral
Treatment ET

• First line
• Propranolol start at 10 mg x 3 => 240-320 mg/d
• Primidone
• Second line
• Gabapentin, topiramate, clozapine, long acting
benzodiazepine (clonazepam)
Holme’s tremor
• “midbrain tremor” “rubral tremor”
“thalamic tremor”

• predominately proximal limb (<4.5 Hz)
during postural in nature

• Upper brain stem, thalamus,
cerebellum, interrupting pathways in the
midbrain tegmentum
Wing Beating Tremor in Wilson’s disease
Dystonic tremor
Parkinson’s
disease
James Parkinson,
London
(1755 – 1824)

An Essay on the Shaking
He identified 6 cases, 3 of whom he personally
Palsy(1817)
examined; 3 he observed on the streets of
London
Shaking Palsy(Paralysis agitans)
J Neuropsychiatry Clin Neurosci 2002;14:223–36
Parkinsonism
• clinical syndrome of bradykinesia, resting tremor,
cogwheel rigidity, and postural instability
Parkinson’s disease
• clinical syndrome of asymmetrical parkinsonism,
usually with rest tremor, in association with the
specific pathological findings of depigmentation of
the SN as a result of loss of melanin-laden
dopaminergic neurons containing eosinophilic
cytoplasmic inclusions(Lewy bodies)
Epidemiology
• Community based series
• prevalence 360 per 100,000 and an
incidence of 18 per 100,000 per year
• PD is an age-related disease
• gradually increase after age 50 years, and
disease before age 30 years is rare
• Female: Male=1:1
de Lau and Breteler. Lancet Neurol 2006; 5: 525-535
Pathophysiology
• Structural change
• Loss of pigmented neurons in the SNc and
other pigmented neuron
• Histopathology: Lewy bodies
• Neurotransmitter change
• Depletion of dopamine containing cells in
the substantia nigra leads to decreased
dopamine n the striatal
Pathology
• Gross: loss of pigmented cell in
substantial nigra(SN) and other
pigmented nuclei(locus
ceruleus(LC), dorsal motor nucleus
of the vagus)

http://www.babraham.ac.uk/images/research/SAS/emson/fig1.jpg
http://www.uhmc.sunysb.edu/pathology/neuropath
Pathology

Normal substantia nigra

Surviving neuron contains a Lewy body
Extensive loss of pigmented neurons
Group of Parkinsonism
TYPICAL
• Primary or idiopathic parkinsonism
OR
• Parkinson’s disease
CLASSIC
• Secondary parkinsonism

• hydrocephalus, vascular parkinsonism, encephalitis

• Parkinson plus syndrome

• Progressive supranuclear palsy(PSP), corticobasal
degeneration(CBD), multiple system atrophy(MSA)

• Hereditary parkinsonism ATYPICAL
• Wilson’s disease, Dopa-responseive dystonia,
Huntington’s disease(HD)
United Kingdom Parkinson's Disease
Society(UKPDS) Brain Bank Diagnostic
Criteria for PD

• Step 1: Diagnosis of Parkinsonism
• Step 2: Features tending to exclude
Parkinson’s disease as the cause of
Parkinsonism
• Step 3: Features that support a diagnosis of
Parkinson’s disease (three or more required
for diagnosis of definite Parkinson’s disease)

Diagnostic
accuracy to 82% Hughes AJ, Daniel SE, Kilford L, Lees AJ. JNNP 1992 Mar;55(3):181-4
Step 1: Diagnosis of
Parkinsonism
• Bradykinesia and at least one of the
following:
• Muscular rigidity
• 4–6 Hz resting tremor
• Postural instability not caused by primary
visual, vestibular, cerebellar or
proprioceptive dysfunction
Pill rolling tremor
Finger tapping
Bradykinesia
Micrographia
Micrographia
Step 2: Features tending to exclude Parkinson’s
disease as the cause of Parkinsonism

• History of repeated strokes • Supranuclear gaze palsy
with stepwise progression of
parkinsonian features
• Cerebellar signs

• Early severe autonomic involvement
• History of repeated head
injury • Early severe dementia with
disturbances of memory, language
• History of definite encephalitis and praxis

• Neuroleptic treatment at • Babinski's sign
onset of symptoms
• Presence of a cerebral tumour or
• >1 affected relatives communicating hydrocephalus on
computed tomography scan
• Sustained remission
• Negative response to large doses of
• Strictly unilateral features after levodopa (if malabsorption
excluded)
3 years
• MPTP exposure
Step 3: Features that support a diagnosis
of PD (three or more required)
• Unilateral onset

• Rest tremor present

• Progressive disorder

• Persistent asymmetry affecting the side of onset most

• Excellent (70–100%) response to levodopa

• Severe levodopa-induced chorea

• Levodopa response for 5 years

• Clinical course of 10 years
Non-motor symptoms

• Loss of sense of smell, constipation
• REM behavior disorder (a sleep
disorder)

• Mood disorders
• Orthostatic hypotension (low blood
pressure when standing up)
Diagnostic studies

• MRI/CT brain: using for exclude
other cause of parkinsonism
• In PD, the MRI brain usually reveals
normal
PD disease progression-
treatment response
Modality of treatment

• Symptoms based treatment
• Pharmacologic vs Non-pharmacologic
• Motor vs Non-motor symptoms
• Neuro-protection
• Prevention
Motor symptoms of Parkinson’s
disease

Dopamine Acetylcholine
Symptomatic based
treatment
• Enhance dopaminergic transmission
• L-dopa, dopamine agonist, drug that
decrease dopamine destruction

• Drug manipulating other
neurotransmitter

• Anti-cholinergic drug
Dose of the preparations of Sinemet and Madopar

Levodopa + DDI

Madopar (levodopa+benserazide)
Sinemet (levodopa+carbidopa)

Madopar HBS
Sinemet CR
As the disease progress,
the Therapeutic window narrow
symptoms and side effects occur as the levodopa therapeutic window diminishes

Dyskinesia threshold

Efficacy threshold

•  Smooth, extend •  Diminished duration •  Short, unpredictable
response response
•  Increased incidence
•  Absent or infrequent of dykinesia •  on time is
dyskinesia associared with
dyskinesia 39
Parkinson Plus
Syndrome
Parkinson-plus
syndrome
• Multiple system atrophy
• cerebellar sign + ve, autonomic dysfunction
• Progressive supranuclear palsy
• vertical gaze palsy
• Corticobasal degeneration
• limb apraxia
• Dementia with lewy bodies
• Vivid visual hallucination with dementia
PSP described by Richardson
Dystonia
Dystonia classification
• Age of onset
• early-onset: age < 26 year
• late-onset: age > 26 year
• Distribution
• focal (single body reion)
• segmental (contiguous region)
• multifocal (eg. hemidystonia)
• Generalized
Dystonia classification-by etiology
Primary dystonia

• AD: early-onset limb dystonia (DYT1), Mixed dystonias
(DYT6, DYT13), Late-onset craniocervical dystonia (DYT7)

• Idiopathic (cervical dystonia, writer cramp, generalised
dystonia etc)

Secondary dystonia

• Dystonia-plus: Dopa-responsive dystonia(DRD), rapid onset
dystonia parkinsonism (RDP), Myoclonus-dystonia(M-D)

• Heredodegerative dystonias: AD (HD, SCA,3, DRPLA), AR
(Wilson’s disease, MLD)

• Acquired cause: drug induced, basal ganglia lesions
• From other degenerative disorder (PD, PSP etc.)
Classification of dystonia
by distribution
• 5 categories: focal, segmental,
multifoacl, hemi-, generalized

• Focal dystonia: 2/3 of dystonic patients
• Focal dystonia: cervical dystonia(most
common), oromandibular dystonia,
blemphalospasm, laryngeal dystonia,
limb dystonia
Cervical
dystonia 2 พี่น้อง cervical dystonia
• patterned, repetitive, clonic
(spasmodic), or tonic
(sustained) muscle
contractions resulting in
abnormal movements and
postures of the the head
and neck

• Symptoms: pain,
headache, abnormal
posture, tremor, orthopedic
or neurological
complications
Blephalospasm+oromandibular
dystonia= Meige’s syndrome
Treatment
• Levodopar should be tried to exclude
DRD

• Anti-cholinergic:
• Clonazepam, baclofen,
benzodiazepine, carbamazepine,
tizanidine

• Botulinum toxin infection
Myoclonus
Classification
• Etiology • Contraction pattern
• physiological, essential, • rhythmic, arrhythmic,
epileptic, symptomatic
oscillaroty
• Anatomical distribution • Clinical neurophysiology
• focal, segmental, testing
multifocal, generalize
• cortical, cortical-
• Provocative factor subcortical, subcortical-
supraspinal, spinal,
• spontaneous, reflex, peripheral
action
Step
• Describe distribution of myoclonus-
focal, generalize

• Anatomical localization: cortical,
subcortical, spinal, peripheral

• Describe type - negative vs positive
• Look for other neurological vs physical
sign

• Identify cause
Negative myoclonus (flapping tremor or asterixis)
in patient with hepatic encephalopathy
Post hypoxic myoclonus - cortical myoclonus
Hemifacial spasm

Most common peripheral myoclonus
Chorea

“the dancing”
Chorea
irregular, nonrhythmic, unsustained involuntary
movement that flows from one part of the body to
another

step to identify chorea
- Most important Identify body part of chorea
- focal, hemibody: structural lesion in the brain
- generalized: diffuse brain lesion (acquire vs
congenital) or Toxic/Metabolic/Drug
Acute right side chorea in acute basal ganglia
infarction
A Parkinson’s disease
patient with motor
dyskinesia (chorea)
during “on” L-dopa

“On”

“Off”
Conclusion
• Movement disorder: hypo-hyperkinetic
• Each type of hyper kinetic - description the movement
disorder pattern

• Identify distribution, associated neurological finding
and possible cause (for work up)

• Very common movement disorder you should know :
Parkinson’s disease, essential tremor, structural lesion
in the brain (mostly cause focal, hemi-body movement
disorder), Generalised movement disorder (look for
metabolic/drug)

• Some may have genetic cause (ask for the family
members)
NNC MU
Thank you for your
kind attention
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