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BRAIN HEALTH

SPECIAL INTEREST GROUP

CONVENOR

Dr. Au Yeung Tung Wai


MEMBERS

Dr. Chui Pui Yuk, Catherine


Dr. Kng Poey Lyn, Carolyn


Dr. Kwan Yiu Keung


Dr. Lau Sze Ting


Dr. Lee Shun Wah, Jenny


Dr. Lo Kwun Man


Dr. Tam Kui Fu, Stanley


Dr. Wong Chit Wai



PREFACE

It has been three years since the publication of Practical manual of dementia
and cognitive disorders in older adults - diagnosis and assessment by the
Hong Kong Geriatrics Society, Brain Health Special Interest Group. With
rapid advances in knowledge about dementia, the Group felt an imperative
to produce an updated second edition. We were uncertain whether naming
it as the second edition would be appropriate as observant readers may
have noticed that the title of this manual has been changed to Practical
manual of neurocognitive disorders in older adults. With the issuance of
DSM-5 in 2013, the reason for using the term neurocognitive disorder to
replace dementia and cognitive disorders is obvious. Nevertheless, we
have not deliberately refrained from using the term dementia. Therefore
it will continuously appear in many parts of this manual. Despite inherent
negative implications of the term, its generic and literal meaning has its own
merit and significance for communication among health professionals and
academia in the field of dementia.

Apart from adding the most current criteria like DSM-5 and NIA-AA criteria,
we have updated many other criteria for various types of dementia. However,
we still retain some classical criteria like the Hachinski ischaemic score for
vascular dementia and the Petersen criteria for mild cognitive impairment.
Not only because of their historical significance in the advancement of
dementia knowledge, but also as fundamental milestones to facilitate
readers understanding of the conceptual evolution of certain types of
neurocognitive disorders.

In this Edition, we have added a section about Late stage dementia,


which constitutes considerable proportion of patients under the care of most
geriatricians. This section covers both end-of-life care and advance care
planning in dementia. We have also added a section on Management of
neurocognitive disorders. We deliberately omitted this in the first edition,
which focused on diagnosis and assessment. We have to emphasize that
non-pharmacological treatment is always an effective and adjunctive
management strategy across various stages of dementia. The section First
time assessment of neurocognitive disorders may be of particular interest
to the trainees and junior fellows. With all these newly added sections, the
manual has increased from three sections in the first edition to six in this
PREFACE ACKNOWLEDGEMENT

second edition. It is no longer possible or necessary to keep it pocket- We would like to acknowledge Dr Wang Ki, consultant radiologist of Prince
sized as doctors already have too many belongings to keep in their white of Wales Hospital, for his writing on chapter Neuroimaging in dementia, and
coats, including commonly a smart phone. An additional App version may Mr. Charles Chiu, Chairman of Guardianship Board, HKSAR, for his writing
be what we should aim for in the future editions. For the present edition, we on chapter Enduring power of attorney, guardianship order and advance
believe it will serve as a useful desk reference in your office or clinic. directive, and Ms. Menda Chau, occupational therapist of Wong Chuk Hang
Hospital, for contributing to Chapter Non-pharmacological management of
Apart from thanking the members of Brain Health Special Interest Group, behavioural and psychological symptoms of dementia.
who have contributed to information gathering, peer discussion, editing
and writing, I must give special thanks to two invited authors. Dr Wang
Ki, consultant radiologist of Prince of Wales Hospital, who has written the
chapter Neuroimaging in dementia and Mr. Charles Chiu, Chairman of
Guardianship Board, HKSAR, who contributed to the chapter Enduring
power of attorney, guardianship order and advance directive.

We hope you enjoy reading this manual.

Au Yeung Tung Wai


Editor and
Convenor, Brain Health Special Interest Group

June 2014
CONTENT

Introduction 10 1.5 Dementia with Lewy bodies (DLB) and Parkinsons 52


disease dementia (PDD)
1.5.1 DSM-5 criteria for neurocognitive disorder with Lewy bodies 52
Section 1: Diagnostic criteria of neurocognitive disorders 12
2013
1.1 All cause dementia 13 1.5.2 DSM-5 criteria for neurocognitive disorder due to Parkinsons 54
disease 2013
1.1.1 DSM-5 criteria for major neurocognitive disorder 2013 13
1.5.3 PDD diagnostic criteria 2007 55
1.1.2 NIA-AA criteria for all cause dementia 2011 17
1.5.4 DLB diagnostic criteria 2005 57
1.2 Alzheimers disease and dementia due to Alzheimers 18
disease (AD) 1.6 Frontotemporal dementia (FTD) 62
1.2.1 DSM-5 criteria for neurocognitive disorder due to AD 2013 18 1.6.1 DSM-5 criteria for frontotemporal neurocognitive disorder 62
2013
1.2.2 DSM-IV criteria for AD 1994 20
1.6.2 International consensus criteria for behavioral variant FTD 64
1.2.3 NIA-AA criteria for dementia due to AD 2011 21
2011
1.3 Vascular dementia and vascular cognitive impairment 28 1.6.3 Classification of primary progressive aphasia and its variants 68
2011
1.3.1 DSM-5 criteria for vascular neurocognitive disorder 2013 28
1.6.4 Frontotemporal lobar degeneration: a consensus on clinical 72
1.3.2 DSM-IV criteria for vascular dementia 1994 30
diagnostic criteria 1998
1.3.3 AHA-ASA criteria for vascular cognitive impairment 2011 31
1.3.4 NINDS-AIREN criteria for vascular dementia 1993 34 1.7 Delirium 80
1.3.5 Hachinski ischaemic score 1975 36 1.7.1 DSM-5 criteria for delirium 2013 80
1.7.2 Confusion Assessment Method 1990 82
1.4 Mild cognitive impairment (MCI) 40
1.4.1 DSM-5 criteria for mild neurocognitive disorder 2013 41
Section 2: Assessment scales in neurocognitive disorders 84
1.4.2 DSM-5 criteria for mild neurocognitive disorder due to AD 42
2013 2.1 Abbreviated mental test (AMT) 85
1.4.3 DSM-5 criteria for mild vascular neurocognitive disorder 2013 43 2.2 AD8 88
1.4.4 NIA-AA criteria for MCI due to AD 2011 45 2.3 Clock drawing test (CDT) 91
1.4.5 AHA-ASA criteria for vascular MCI 2011 47 2.4 Mini-mental state examination (MMSE) 96
1.4.6 International Working Group on MCI criteria 2004 49 2.5 Montreal cognitive assessment (MoCA) 100
1.4.7 Petersen criteria for mild cognitive impairment 1999 50 2.6 Mattis Dementia Rating Scale (MDRS) 112
CONTENT

2.7 Clinical dementia rating (CDR) 118 Section 6: First time assessment of neurocognitive 178
2.8 Functional assessment staging (FAST) 121 disorder in older adults
2.9 Global deterioration scale (GDS) 126 6.1 Practical guide for first time assessment 179
2.10 Geriatric depression scale (GDS) 128
2.11 Cornell Scale for Depression in Dementia (CSDD) 130
2.12 Disability Assessment for Dementia (DAD) 134
2.13 Neuropsychiatric inventory (NPI) 139
2.14 Zarit burden interview (ZBI) 140

Section 3: Investigation 142

3.1 Neuroimaging 143


3.2 Biomarkers 152

Section 4: Management 156

4.1 Non-pharmacological management of behavioural and 157


psychological symptoms of dementia (BPSD)
4.2 Treatment guideline and practical tips in using 162
cholinesterase inhibitors and NMDA receptor
antagonist

Section 5: Late stage dementia 168

5.1 End of life management in late stage dementia 169


5.2 Enduring power of attorney, guardianship order and 175
advance directive
INTRODUCTION

The incidence and prevalence of dementia in older adults are increasing of neurocognitive disorders. The importance of non-pharmacological
rapidly. Not only does the disease carry significant impact on morbidity management can never be over-emphasized. Section 5 discusses late stage
and mortality, but it also creates heavy burdens on caregivers and health dementia including both end-of-life management and medico-legal facts of
expenditure. As clinicians, we increasingly encounter older patients who advance planning. The last section, section 6, is a proposed schema for the
present with cognitive complaints or those who are incidentally discovered first time assessment of neurocognitive disorder in a memory clinic setting.
to have cognitive impairment. The diagnosis of dementia relies solely on
clinical approach. To date, no high technology can replace the diagnostic We wish this manual will serve as a convenient desk reference for geriatricians,
accuracy of a well-trained clinician. A handy reference will definitely facilitate psychiatrists, neurologists and trainees of these specialties; or for any
an accurate diagnosis and a proper assessment in a demented older adult. clinician who encounter older demented persons in their clinical practice.
It is always advisable not to make a diagnosis of dementia causally in Lastly, we hope that the inclusion of Chinese versions of cognitive tests,
acute settings as many secondary causes can complicate our assessment. which are culture and language dependent, may benefit older patients and
Therefore, we advise you to use this manual primarily in clinic, extended care medical practitioners in mainland China, or in any other Chinese speaking
or long-term care settings but with wisdom and caution in acute settings. community worldwide.

This manual consists of six sections. Section 1 contains the diagnostic


criteria of the common types of dementia. Reciting thoroughly the diagnostic
criteria is not a prerequisite for diagnosing or classifying dementia. Instead,
a conceptual understanding of the criteria will definitely help in making a
correct diagnosis and classification. Section 2 contains the assessment
scales for neurocognitive disorders. These scales are very useful in staging
the severity of the illness, and in gauging the functional impairment and
psychiatric disturbance of our patients. Not only do these aspects affect our
treatment plan, but they form the common language we use to communicate
with our colleagues in clinical practice and in the academic field. As these
tests are culturally specific, locally validated versions are included as far as
available. However, because of copyright restriction, we cannot present them
all in this manual. We have tried to add practical notes about the applications
and interpretations of the various diagnostic criteria and assessment scales,
hoping to clarify some of the common pitfalls and misconceptions. And in
some parts we even tried to compare the different criteria. These opinions
are generated by our Special Interest Group members after peer discussion
and we understand that they will by no means be agreeable among all
authorities. Section 3 is about investigations of neurocognitive disorders. To
avoid ending up with a never-ending list, we chose to focus on two areas,
namely neuroimaging and biomarkers. Section 4 is about the management

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SECTION 1
Diagnostic criteria of neurocognitive disorders

1.1 All-cause dementia


1.1.1 DSM-5 criteria for major neurocognitive disorder 2013

Major Neurocognitive Disorder


Diagnostic Criteria
A. Evidence of significant cognitive decline from a previous level of
performance in one or more cognitive domains (complex attention,
executive function, learning and memory, language, perceptual-motor,

SECTION 1 or social cognition) based on:


1. Concern of the individual, a knowledgeable informant, or the clinician
that there has been a significant decline in cognitive function; and
Diagnostic criteria of 2. A substantial impairment in cognitive performance, preferably

neurocognitive disorders documented by standardized neuropsychological testing or, in its


absence, another quantified clinical assessment.
B. The cognitive deficits interfere with independence in everyday activities
(i.e., at a minimum, requiring assistance with complex instrumental
activities of daily living such as paying bills or managing medications).
C. The cognitive deficits do not occur exclusively in the context of a
delirium.
D. The cognitive deficits are not better explained by another mental disorder
(e.g. major depressive disorder, schizophrenia).

Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

13
SECTION 1
Diagnostic criteria of neurocognitive disorders

Practical notes on DSM-5 Perceptual-motor


DSM-5 has extensively revised the diagnostic criteria and approach in Social cognition
neurocognitive disorders (NCDs), which referred to in DSM-IV as Dementia,
Delirium, Amnestic, and Other Cognitive Disorders. Examples of symptoms or observations regarding impairments in everyday
activities, and examples of assessments are also listed in the DSM-5. They
The choice of a new term NCD for dementia is based on the rationale that are useful in guiding the clinical approach and may help in selecting fast and
this disorder is not restricted to older person. It is often a preferred term for practical tests in memory clinics.
conditions affecting younger individuals, such as NCD due to HIV infection.
The prefix neuro represents the structural brain while cognitive alone Aetiological Subtypes
has been understood as psychological mind. The addition of neuro will After determination of major or mild NCD, it follows by subtyping into various
define the disorder belongs to structural brain diseases and not a mind aetiological groups which include:
disorder.
1. NCD due to Alzheimers disease* (P.18)
DSM-5 adopts a multi-step approach in the classification of neurocognitive 2. Vascular NCD* (P.28)
disorders.
3. NCD with Lewy bodies* (P.52)
Major or Mild NCD 4. NCD due to Parkinsons disease* (P.54)
First is to exclude delirium and then decide whether it is a major or 5. Frontotemporal NCD* (P.62)
mild neurocognitive disorder. A single domain being affected (cognitive 6. NCD due to traumatic brain injury
impairment being concerned AND documented by neuropsychological
testing or quantified clinical assessment) is adequate to be classified as 7. NCD due to HIV infection
NCD. The distinction between major and mild depends on how significantly 8. Substance/medication induced NCD
it interferes with the independence in everyday activities and the degree of
impairment in objective testing. Major NCD has substantial impairment with 9. NCD due to Huntingtons disease
the test performance in the range of 2 or more standard deviations below 10. NCD due to prion disease
appropriate norm (below the 3rd percentile), whereas mild NCD has modest
11. NCD due to another medical condition*
impairment with performance lies in 1 - 2 standard deviations range (between
the 3rd and 16th percentiles). The 6 cognitive domains listed in DSM-5 for 12. NCD due to multiple etiologies*
establishing the presence of NCD are: 13. Unspecified NCD
Complex attention
Executive function Note that all can be classified as major or mild. The aetiological subtyping
is linking up with the world expert panel criteria in each disorder. For
Learning and memory
example, mild NCD due to Alzheimers disease (AD) will be broadly similar
Language to mild cognitive impairment in the Petersen criteria. However, it demands

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Diagnostic criteria of neurocognitive disorders

an additional objective assessment. Also note that major NCD due to AD 1.1.2 NIA-AA criteria for all-cause dementia 2011
requires 2 domains, one of which needs to be memory. Therefore, it still
adheres to the convention. In addition, the DSM-5 criteria due to different
subtypes also adopt probable and possible classification in line with world When cognitive or behavioral (neuropsychiatric) symptoms that:
expert panel criteria. This is another major difference from DSM-IV. 1. Interfere with ability to function at work or at usual activities; and
2. Represent a decline from previous levels of functioning and performing;
The * are more important for older adults and can be referred to the different
and
section of this manual with the page numbers in brackets. As remarked in the
DSM-5 manual, older adults are commonly complicated by comorbidities, 3. Are not explained by delirium or major psychiatric disorder;
which make subtyping difficult.
4. Cognitive impairment is detected and diagnosed through a combination
of (1) history taking from patient and a knowledgeable informant and
Specifiers (2) an objective cognitive assessment, either a bedside mental status
After subtyping, we can assign specifiers, which are similar to behavioural examination or neuropsychological testing.
and psychological symptoms of dementia, and they are listed below: 5. The cognitive or behavioral impairment involves a minimum of two of the
Delusions following domains :
Hallucinations a. Impaired ability to acquired and remember new information
Mood disturbance b. Impaired reasoning, handling of complex tasks and poor judgment
Agitation
c. Impaired visuospatial abilities
Combative behaviours
Sleep disturbance d. Impaired language functions

Apathy e. Change in personality, behaviour, or comportment

This is a good list for history taking and mental examination. It helps to identify
non-cognitive problems in dementia that needs either pharmacological or
non-pharmacological intervention.

DSM is a classification manual for mental disorders and is not intended


to replace the clinical approach for the diagnosis of diseases. Although it
may provide a checklist for the clinical features of the disorder, it itself
is neither sufficient nor necessary to establish a diagnosis. Ultimately, Reference
the diagnosis of NCD or dementia is based on clinical judgment and may 1. Mckhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH, et al. The diagnosis of
dementia due to Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers
include considerations outside the criteria. It is only an assistant in clinical Association workgroups on diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011;7:263-9.
practice and a common language for communication among clinicians and
researchers.
16 17
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.2 Alzheimer disease and dementia due to


Alzheimer disease c. No evidence of mixed etiology (i.e., absence of other
1.2.1 DSM-5 criteria for neurocognitive disease due to neurodegenerative or cerebrovascular disease, or another
Alzheimers disease 2013 neurological, mental, or systemic disease or condition likely
contributing to cognitive decline).
For mild neurocognitive disorder:
Major or Mild Neurocognitive Disorder Due to
Alzheimers Disease Probable Alzheimers disease is diagnosed if there is evidence of
a causative Alzheimers disease genetic mutation form either genetic
Diagnostic Criteria testing or family history.
A. The criteria are met for major or mild neurocognitive disorder.
Possible Alzheimers disease is diagnosed if there is no evidence of
B. There is insidious onset and gradual progression of impairment in one or a casusetive Alzheimers disease genetic mutation from either genetic
more cognitive domains (for major neurocognitive disorder, at least two testing or family history, and all three of the following are present:
domains must be impaired).
1. Clear evidence of decline in memory and learning.
C. Criteria are met for either probable or possible Alzheimers disease as
2. Steadily progressive, gradual decline in cognition, without extended
follows:
plateaus.
For major neurocognitive disorder:
3. No evidence of mixed etiology (i.e., absence of other neurodegenerative
Probable Alzheimers disease is diagnosed if either of the following or cerebrovascular disease, or another neurological or systemic
is present; otherwise, possible Alzheimers disease should be disease or condition likely contributing to cognitive decline).
diagnosed.
D. The disturbance is not better explained by cerebrovascular disease,
1. Evidence if a causative Alzheimers disease genetic mutation from another neurodegenerative disease, the effects of a substance, or
family history or genetic testing. another mental, neurological, or systemic disorder.
2. All three of the following are present:
a. Clear evidence of decline in memory and learning and at least
one other cognitive domain (based on detailed history or serial
neuropsychological testing).
b. Steadily progressive, gradual decline in cognition, without
extended plateaus.
Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

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Diagnostic criteria of neurocognitive disorders

1.2.2 DSM-IV criteria for dementia of the 1.2.3 NIA-AA criteria for dementia due to
Alzheimers type 1994 Alzheimers Disease 2011

A. The development of multiple cognitive deficits manifested both NIA-AA criteria for probable AD dementia
1. memory impairment 1. Meet criteria for dementia by NIA-AA
2. one (or more) of the following cognitive disturbances : 2. Insidious onset
aphasia 3. Clear-cut history of worsening of cognition by report or observation
apraxia 4. The initial and most prominent cognitive deficits are evident on history
agnosia and examination in one of the following categories :

disturbance in executive functioning a. Amnestic presentation

B. The cognitive deficits in Criteria A1 and A2 each cause significant b. Non-amnestic presentations:
impairment in social or occupational functioning and represent a Language presentation
significant decline from a previous level of functioning
Visuospatial presentation
C. The course is characterised by gradual onset and continuing cognitive
decline Executive dysfunction

D. The cognitive deficits in criteria A1 and A2 are not due to any of the 5. The diagnosis of probable AD dementia should not be applied when
following: there is evidence of :

other central nervous system conditions that cause progressive a. substantial concomitant cerebrovascular disease
deficits in memory and cognition b. core features of Dementia with Lewy bodies
systemic conditions that are known to cause dementia c. prominent features of behavioral variant frontotemporal dementia
substance-induced conditions d. prominent features of semantic variant primary progressive aphasia
E. The deficits do not occur exclusively during the course of a delirium or non-fluent/agrammatic variant primary progressive aphasia

F. The disturbance is not better accounted for by another mental disorder e. another concurrent, active neurological disease, or a non-
neurological medical comorbidity or use of medication that could
have a substantial effect on cognition
Reference
1. American Psychiatric Association: Diagnostic and statistical Manual of Mental Disorders, Fourth Edition, Text
Note : All patients who met criteria for probable AD by 1984 NINCDS-ADRDA
Revision. Washington, D.C., American Psychiatric Association, 2000.
criteria would meet the NIA-AA criteria for probable AD.

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SECTION 1
Diagnostic criteria of neurocognitive disorders

NIA-AA criteria for possible AD dementia NIA-AA AD dementia criteria incorporating biomarkers
Meets the core clinical criteria for AD dementia, but either of : Neuronal injury
Biomarker (CSF tau, FDG-
1. Atypical course Diagnostic probability of PET, structural
a sudden onset of cognitive impairment or demonstrates insufficient category AD etiology A (PET or CSF) MRI)
historical detail or objective cognitive documentation of progressive Probable AD dementia
decline.
Unavailable, Unavailable,
2. Aetiologically mixed presentation Based on clinical
Uninformative conflicting, or conflicting, or
criteria
indeterminate indeterminate
concomitant cerebrovascular disease; or
Unavailable or
features of dementia with Lewy bodies; or Intermediate
indeterminate
Positive
With three level
evidence for another neurological disease or a non-neurological of evidence of AD Unavailable or
Intermediate Positive
medical comorbidity or medication use that could have a substantial pathophysiological indeterminate
process
effect on cognition
High Positive Positive

Note : A diagnosis of possible AD by 1984 NINCDS-ADRDA criteria would not Possible AD dementia
necessarily meet the NIA-AA criteria for possible AD dementia.
Unavailable, Unavailable,
Based on clinical
Uninformative conflicting, or conflicting, or
criteria
Dementia unlikely to be due to AD indeterminate indeterminate

1. Does not meet criteria for AD dementia. With evidence of AD High but does not
pathophysiological rule out second Positive Positive
2. a. Regardless of meeting clinical criteria for probable or possible process etiology
AD dementia, there is sufficient evidence for an alternative diagnosis.
Dementia-unlikely
Lowest Negative Negative
b. Regardless of meeting clinical criteria for possible AD dementia, due to AD
both amyloid beta and neuronal injury biomarkers are negative.
Abbrevations : AD, Alzheimers disease; A, amyloid-beta; PET, positron emission tomography; CSF,
cerebrospinal fluid; FDG, 18fluorodeoxyglucose; MRI, magnetic resonance imaging.

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Diagnostic criteria of neurocognitive disorders

NIA-AA preclinical stage of AD 8. Proposed staging framework for preclinical AD :


1. It is intended for research purpose and has no clinical utility. Stage 1. Asymptomatic cerebral amyloidosis
2. It bases on the concept that AD pathophysiological process (AD-P) high PET amyloid tracer retention
emerges long before the clinical phase of AD, and thus AD is regarded low CSF A42
as a continuum from preclinical stages to MCI, and to dementia.
Stage 2. Amyloidosis + neurodegeneration
3. There is no clinical diagnostic criterion for this stage.
neuronal dysfunction on FDG-PET/functional MRI
4. It rests on the laboratory evidence of AD-P in an asymptomatic individual.
high CSF total tau/phosphorylated tau
5. Laboratory tests involve Alzheimers biomarkers are paramount for the
detection of this stage. cortical thinning/hippocampal atrophy in structural MRI

6. Two major categories of Alzheimers biomarkers: Stage 3. Amyloidosis + neurodegeneration + subtle cognitive decline

a. Biomarkers of amyloid beta (A) accumulation evidence of subtle change from baseline level of cognition

which are reductions in CSF A42 and increase amyloid retention poor performance on more challenging cognitive tests
on PET. does not yet meet criteria for mild cognitive impairment
is regarded as initial event in AD-P.
b. Biomarkers of neuronal degeneration or injury
which are elevated CSF tau (both total and phosphorylated tau);
decreased fluorodeoxyglucose 18F (FDG) uptake on PET with
temporoparietal pattern of hypometabolism; atrophy on structural
MRI in a characteristic pattern involving the medial, basal, and
lateral temporal lobes, and medial and lateral parietal cortices.
presence of these biomarkers in addition to biomarkers of A
accumulation may increase the likelihood of clinical decline to
MCI within several years.
References
7. It is postulated that evidence of subtle cognitive decline detected by 1. Jack CR Jr, Albert MS, Knopman DS, McKhann GM, Sperling RA, Carrillo MC, et al. Introduction to the
more sensitive cognitive measures, together with biomarkers evidence of recommendations from the National Institute on Aging-Alzheimers Association workgroups on diagnostic guidelines
for Alzheimers disease. Alzheimers Dement 2011;7;257-62.
A accumulation and neuronal injury, indicate the last stage of preclinical 2. Mckhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH, et al. The diagnosis of dementia due
AD and are approaching MCI. to Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers Association workgroups
on diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011;7:263-9.
3. Sperling RA, Aisen PS, Beckett LA, Bennett DA, Craft S, Fagan AM, et al. Toward defining the preclinical stages of
Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers Association workgroups on
diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011;7:280-92.

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Diagnostic criteria of neurocognitive disorders

Practical notes on Alzheimers Disease and Dementia The original criteria rest on the notion that AD is a clinical-pathological entity.
However, with the emerging concept that AD pathophysiological change
Making the diagnosis of dementia due to Alzheimers disease occurs decades before the clinical overt symptoms, AD is regarded as a
It is a clinical diagnosis by exclusion of some potential causes. There is no continuous spectrum from preclinical/presymptomatic, to pre-dementia/mild
single reliable diagnostic investigation or imaging to rule in the disease. It cognitive impairment (MCI), and finally to clinically overt dementia. This allows
relies on the history from patients and caregivers to establish the diagnosis. the new criteria and guideline to define three stages of disease progression
Therefore, it is always advisable to take history from reliable caregivers. It is over time (preclinical AD, MCI due to AD and dementia due to AD). The
not recommended to assess the patient in acute settings with presence of new criteria and guideline regarding AD dementia and MCI due to AD are
confounding medical and environmental factors. intended to guide diagnosis in the clinical setting. On the other hand, the
recommendation of preclinical AD is intended purely for research purpose.
Alzheimers Disease vs. Alzheimers Dementia and NIN-AA criteria
Biomarkers are parameters that can be measured in the body to reliably
It is worthwhile to note that a number of elderly people suffer from Alzheimers
indicate the pathophysiological process of a disease. Five mostly widely
disease (AD) but not Alzheimers dementia yet. The latter signifies that the
studied biomarkers of AD are incorporated into the new diagnostic criteria.
patient has to exhibit features of dementia while the former signifies presence
They are divided into two categories : (1) the biomarkers of amyloid beta (A)
of Alzheimers pathology such as amyloid plaques or tau proteins in the brain
accumulation, which are PET evidence of A deposition and low CSF A42,
only. Early AD may be picked up by the advances in technology, e.g., the
and (2) the biomarkers of neuronal degeneration or injury, which are elevated
ratio of biomarkers such as low amyloid beta and high phosphorylated tau
CSF tau (both total and phosphorylated tau), decreased 18fluorodeoxyglucose
level in the cerebrospinal fluid, or new neuroimaging techniques. This may
(FDG) uptake on PET in temporoparietal cortex; and disproportionate
allow detection of preclinical stage of AD, in the hope to prevent patients from
atrophy on structural magnetic resonance imaging in medial, basal, and
evolving into the stage of clinical Alzheimers dementia by earlier treatment.
lateral temporal lobe, and medial and lateral parietal cortices. Integration of
NIA-AA has developed criteria based on this conceptual framework about
biomarkers into the new guideline is mainly for research purpose, in which the
the pathophysiological evolution of AD. It encompasses preclinical stages of
use of biomarkers is paramount in the detection of pre-clinical AD, whereas,
AD (solely for research purpose), mild cognitive impairment due to AD and
it is complimentary in the diagnosis of MCI and dementia due to AD, as they
finally AD dementia.
are based primarily on clinical ground.
With advances in the understanding and detection of AD pathophysiology,
DSM-5 vs. NIA-AA criteria in AD
a new criteria and guideline for the diagnosis of AD was published by the
National Institute on Aging-Alzheimers Association (NIA-AA) in 2011 after Both criteria incorporate quantifiable cognitive test as an integral part of the
revising the 1984 criteria for clinical diagnosis of AD by the National Institute diagnosis. NIA-AA does not specifically demand a reference value but DSM-5
of Neurological and Communicative Disorders and Stroke (NINCDS) and the differentiate mild and major NCD by different cut-offs. NIA-AA criteria start
Alzheimers Disease and Related Disorders Association (ADRDA). with meeting the criteria for dementia while DSM-5 starts with meeting the
criteria for major or mild NCD. Both contain probable and possible AD. NIA-
The new criteria and guideline has two notable differences from the original AA criteria contain the preclinical stage of AD which incorporated biomarkers
NINCDS-ADRDA criteria, namely (1) three stages of disease are defined, and for research application, whereas DSM-5 does not cover the preclinical stage
the (2) incorporation of biomarker tests. of AD. MCI due to AD in NIA-AA is equivalent to DSM-5 mild NCD due to AD.

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Diagnostic criteria of neurocognitive disorders

1.3 Vascular dementia and vascular cognitive


impairment 3. Both clinical and genetic (e.g., cerebral autosomal dominant arteriopathy
1.3.1 DSM-5 criteria for vascular neurocognitive disorder with subcortical infarcts and leukoencephalopathy) evidence of
2013 cerebrovascular disease is present.
Possible vascular neurocognitive disorder is diagnosed if the clinical
criteria are met but neuroimaging is not available and the temporal relationship
Major or Mild Vascular Neurocognitive Disorder
of the neurocognitive syndrome with one or more cerebrovascular events is
Diagnostic Criteria not established.
A. The criteria are met for major or mild neurocognitive disorder.
B. The clinical features are consistent with a vascular etiology, suggested
by either of the following:
1. Onset of the cognitive deficits is temporally related to one or more
cerebrovascular events.
2. Evidence of decline is prominent in complex attention (including
processing speed) and frontal-executive function.
C. There is evidence of the presence of cerebrovascular disease from
history, physical examination, and/or neuroimaging considered sufficient
to account for the neurocognitive deficits.
D. The symptoms are not better explained by another brain disease or
systemic disorder.
Probable vascular neurocognitive disorder is diagnosed if one of the
following is present; otherwise possible vascular neurocognitive disorder
should be diagnosed:
1. Clinical criteria are supported by neuroimaging evidence of significant
parenchymal injury attributed to cerebrovascular disease (neuroimaging-
supported).
2. The neurocognitive syndrome is temporally related to one or more
Reference
documented cerebrovascular events.
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

28 29
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.3.2 DSM-IV criteria for vascular dementia 1994 1.3.3 American Heart Association (AHA) / American
Stroke Association (ASA) criteria for vascular
cognitive impairment 2011
A. The development of multiple cognitive deficits manifested by both:
1. Memory impairment (impaired ability to learn new information or to
recall previously learned information) Diagnostic Criteria for Vascular Cognitive Impairment
2. One or more of the following cognitive disturbances: 1. The term vascular cognitive impairment (VCI) characterizes all forms
of cognitive deficits from vascular dementia (VaD) to mild cognitive
aphasia impairment (MCI) of vascular origin.
apraxia 2. These criteria cannot be used for subjects who have an active diagnosis
agnosia of drug or alcohol abuse/dependence. Subjects must be free of any type
of substance for at least 3 months.
disturbance in executive functioning
3. These criteria cannot be used for subjects with delirium.
B. The cognitive deficits in criteria A1 and A2 each cause significant
impairment in social or occupational functioning and represent a
Probable VaD
significant decline from a previous level of functioning.
1. Meet criteria for dementia
C. Focal neurological signs and symptoms (e.g., exaggeration of deep
It should be based on a decline in cognitive function from a prior
tendon reflexes, extensor plantar response, pseudobulbar palsy,
baseline and a deficit in performance in 2 cogntive domains that are
gait abnormalities, weakness of an extremity) or laboratory evidence
of sufficient severity to affect the subjects activities of daily living.
indicative of cerebrovascular disease (e.g., multiple infarctions involving
cortex and underlying white matter) that are judged to be etiologically It must be based on cognitive testing, and a minimum of 4 cognitive
related to the disturbance. domains should be assessed : executive/attention, memory,
language, and visuospatial functions.
D. The deficits do not occur exclusively during the course of a delirium. The deficits in activities of daily living are independent of the motor/
sensory sequelae of the vascular event.
2. There is cognitive impairment and imaging evidence of cerebrovascular
disease and
there is a clear temporal relationship between a vascular event and
the onset of cognitive deficits, or
there is a clear relationship in the severity and pattern of cognitive
Reference impairment and the presence of diffuse, subcortical
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text
Revision. Washington, D.C., American Psychiatric Association, 2000. cerebrovascular disease pathology.

30 31
SECTION 1
Diagnostic criteria of neurocognitive disorders

Practical notes on AHA/ASA criteria for vascular cognitive


3. There is no history of gradually progressive cognitive deficits before impairment
or after the stroke that suggests the presence of a nonvascular Vascular cognitive impairment (VCI) is the preferred term to multi-infarct
neurodegenerative disorder. dementia (MID) or vascular dementia (VaD).
VCI is proposed for all forms of cognitive disorder associated with
Possible VaD cerebrovascular disease (e.g., frank stroke, vascular brain injury, or
1. Meet criteria for dementia subclinical vascular disease), regardless of the underlying pathogenesis
(e.g., cardioembolic, atherosclerotic, ischemic, haemorrhagic, or
2. There is cognitive impairment and imaging evidence of cerebrovascular genetic).
disease but
VCI represents a spectrum of cognitive severity which encompasses
There is no clear relationship (temporal, severity, or cognitive pattern) mild cognitive deficits, vascular mild cognitive impairment (VaMCI), and
between the vascular disease and onset of cognitive impairment. the most severe form, vascular dementia (VaD).
There is insufficient information for the diagnosis of VaD (e.g., clinical VCI can coexist with multiple cerebral and systemic disorders that can
symptoms suggest the presence of vascular disease, but no CT/MRI affect cognition in the elderly, especially Alzheimers disease. Therefore,
studies are available). the term probable is used to characterize the most pure form of VCI
Severity of aphasia precludes proper cognitive assessment. and the term possible when the certainty of the diagnosis is diminished
or the vascular syndrome is associated with another disease process
There is evidence of other neurodegenerative disease or conditions
that can cause cognitive deficits.
in addition to cerebrovascular disease that may affect cognition.
Diagnostic criteria for VCI is based on:
a. Demonstration of the presence of a cognitive disorder.
b. History of clinical stroke or presence of vascular disease by
neuroimaging that suggest a link between the cognitive disorder and
vascular disease.

Reference
1. Gorelick PB, Scuteri A, Black SE, DeCarli C, Greenberg SM, Iadecola C, et al. Vascular contributions to
cognitive impairment and dementia: a statement for healthcare professionals from the American Heart
Association/American Stroke Association. Stroke 2011;42(9):2672 - 713.

32 33
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.3.4 NINDS-AIREN criteria for probable vascular


dementia 1993
Supporting Features:
Subtle onset and variable course of cognitive deficits
Dementia
Early presence of a gait disturbance
Cognitive decline from a previously higher level of functioning
History of unsteadiness or frequent, unprovoked falls
Impairment of memory and of two or more cognitive domains,
preferable established by clinical examination and documented by Early urinary incontinence
neuropsychological testing Pseudo bulbar palsy
Severe enough to interfere with activities of daily living not due to physical Personality and mood changes
effects of stroke alone
Absence of delirium, psychosis, severe aphasia, or major sensorimotor
impairment precluding neuropsychological testing. And absence of any
other disorders capable of producing dementia syndrome

Cerebrovascular disease
Focal signs consistent with stroke, and
Neuroimaging evidence of relevant CVD including multiple large vessel
infarcts or a single strategically placed infarct (angular gyrus, thalamus,
basal forebrain, or PCA or ACA territories), as well as multiple basal
ganglia and white matter lacunes, or extensive periventricular white
matter lesions.

A relationship between the above two disorders


Onset of dementia within 3 months following a recognized stroke
Abrupt deterioration or fluctuating, stepwise progression of cognitive
deficits.

Reference
1. Romn GC, Tatemichi TK, Erkinjuntti T, Cummings JL, Masdeu JC, Garcia JA, et al. Vascular dementia:
diagnostic criteria for research studies: Report of the NINDS-AIREN International Workshop*. Neurology
1993;43(2):250-60.

34 35
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.3.5 Hachinski ischaemic score 1975 Practical notes on vascular dementia


Vascular dementia is defined as cognitive impairment caused by
Abrupt onset 2 cerebrovascular disease. There are 2 main types: stroke like dementia
and small vessel disease-related dementia.
Stepwise deterioration 1
Stroke like vascular dementia is characterized by an abrupt onset and
Fluctuating course 2 stepwise progression. The clinical syndrome is related to the region of
Nocturnal confusion 1 the brain affected by the particular cerebral artery.
Relative preservation of personality 1 Small vessel disease-related dementia has a more gradual deterioration.
The gait is also affected.
Depression 1
ADL functioning should be affected by the impaired cognitive function
Somatic complaints 1
but not due to the physical disability from the stroke. Clinically it may be
Emotional incontinence 1 difficult to differentiate whether the impaired ADL functioning is caused
by impaired cognition alone.
History of hypertension 1
DSM-IV and NINCD-AIREN diagnostic criteria are both widely used to
History of strokes 2
make a diagnosis. NINCD-AIREN has a stricter definition mandatory
Evidence of associated atherosclerosis 1 neuroimaging evidence, onset of dementia within 3 months of stroke,
Focal neurological symptoms 2 abrupt onset or stepwise progression.

Focal neurological signs 2

7 or above will be classified as vascular dementia

References
1. Hackinski VC, Iliff LD, Zihka E, Du Boulay GH, McAllisterVL, Marshall J, et al. Cerebral blood flow in dementia.
Arch Neurol 1975;32(9):632-7.
2. Mayer-Gross W, Slater E, Roth M: Clinical Psychiatry, 3rd ed. Baillier, Tindall & Carssell, London, 1969.

36 37
SECTION 1
Diagnostic criteria of neurocognitive disorders

Differentiation between VaD and AD Practical notes on mixed dementia


The Hachinski Ischemic Score (HIS) has been used clinically to Sometimes it would be difficult to differentiate whether the dementia is
differentiate vascular dementia from other degenerative dementia caused by neuro-degeneration or cerebrovascular disease. A diagnosis
syndromes. A score of 7 is consistent with vascular dementia and a of mixed dementia is often made at this juncture. It is an overlap between
score of 4 is consistent with degenerative dementias. AD and VaD. Currently there are no specific established criteria.
A low HIS is less likely to indicate vascular dementia because ischemic It can be defined as cognitive decline sufficient to impair independent
lesions severe enough to produce a dementia would be expected to be functioning in daily life resulting from the co-occurrence of Alzheimers
severe enough to cause focal neurological signs and symptoms thus disease (AD) brain pathology and cerebrovascular brain pathology,
results in a higher score. documented either by clinical criteria or neuroimaging findings
HIS has been out of fashion in the field of vascular dementia in recent It is most commonly found in the elderly as both neuro-degeneration and
years, but it is still important to understand its content not only because cerebrovascular disease often co-exist in this population.
it is a historical landmark in vascular dementia, but also because it is a
helpful guidance in the diagnosis of vascular dementia. All of its items
are clinical and can be readily applied when we are taking the history and
doing the physical examination on our patients.
Apart from somatic complaints, all other elements are included in the
diagnostic criteria of vascular dementia developed some years later. In
fact, if somatic complaints are viewed as depressive symptoms, they are
also more commonly found in patients suffering from vascular dementia.

Reference
1. Langa KM, Foster NL, Larson EB. Mixed dementia: emerging concepts and therapeutic implications. JAMA
2004;292(23):2901-8.

38 39
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4 Mild cognitive impairment (MCI) 1.4.1 DSM-5 criteria for mild neurocognitive disorder 2013
In this part, diagnostic criteria of MCI or its equivalent are extracted from
the original full spectrum criteria from different sources, including DSM-5,
Mild Neurocognitive Disorder
NIA-AA and AHA-ASA. Therefore, reference to other sections in this manual
is required for the complete criteria of that particular disease. The classical Diagnostic Criteria
Petersen criteria have been retained to give a complete picture about the A. Evidence of modest cognitive decline from a previous level of
concept of MCI in evolution. performance in one or more cognitive domains (complex attention,
executive function, learning and memory, language, perceptual-motor,
or social cognition) based on:
1. Concern of the individual, a knowledgeable informant, or the clinician
that there has been a mild decline in cognitive function; and
2. A modest impairment in cognitive performance, preferably
documented by standardized neuropsychological testing or, in its
absence, another quantified clinical assessment.
B. The cognitive deficits do not interfere with capacity for independence
in everyday activities (i.e., complex instrumental activities of daily living
such as paying bills or managing medications are preserved, but greater
effort, compensatory strategies, or accommodation may be required.
C. The cognitive deficits do not occur exclusively in the context of a
delirium.
D. The cognitive deficits are not better explained by another mental disorder
(e.g., major depressive disorder, schizophrenia).

Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

40 41
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4.2 DSM-5 criteria for mild neurocognitive disorder 1.4.3 DSM-5 criteria for mild vascular neurocognitive
due to AD 2013 disorder 2013

Mild Neurocognitive Disorder Due to Alzheimers Disease Mild Vascular Neurocognitive Disorder
Diagnostic Criteria Diagnostic Criteria
A. The criteria are met for mild neurocognitive disorder. A. The criteria are met for mild neurocognitive disorder.
B. There is insidious onset and gradual progression of impairment in one or B. The clinical features are consistent with a vascular etiology, suggested
more cognitive domains (for major neurocognitive disorder, at least two by either of the following:
domains must be impaired).
1. Onset of the cognitive deficits is temporally related to one or more
C. Criteria are met for either probable or possible Alzheimers disease as cerebrovascular events.
follows:
2. Evidence of decline is prominent in complex attention (including
Probable Alzheimers disease is diagnosed if there is evidence of processing speed) and frontal-executive function.
a causative Alzheimers disease genetic mutation from either genetic
C. There is evidence of the presence of cerebrovascular disease form
testing or family history.
history, physical examination, and/or neuroimaging considered sufficient
Possible Alzheimers disease is diagnosed if there is no evidence of to account for the neurocognitive deficits.
a causative Alzheimers disease genetic mutation from either genetic
D. The symptoms are not better explained by another brain disease or
testing or family history, and all three of the following are present:
systemic disorder.
1. Clear evidence of decline in memory and learning.
Probable vascular neurocognitive disorder is diagnosed if one of the
2. Steadily progressive, gradual decline in cognition, without extended following is present; otherwise possible vascular neurocognitive disorder
plateaus. should be diagnosed:
3. No evidence of mixed etiology (i.e., absence of other neurodegenerative 1. Clinical criteria are supported by neuroimaging evidence of significant
or cerebrovascular disease, or another neurological or systemic parenchymal injury attributed to cerebrovascular disease (neuroimaging-
disease or condition likely contributing to cognitive decline). supported).
D. The disturbance is not better explained by cerebrovascular disease, 2. The neurocognitive syndrome is temporally related to one or more
another neurodegenerative disease, the effects of a substance, or documented cerebrovascular events.
another mental, neurological, or systemic disorder.
3. Both clinical and genetic (e.g., cerebral autosomal dominant arteriopathy
Reference
with subcortical infarcts and leukoencephalopathy) evidence of
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013. cerebrovascular disease is present.

42 43
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4.4 NIA-AA criteria for MCI due to AD 2011


Possible vascular neurocognitive disorder is diagnosed if the clinical
criteria are met but neuroimaging is not available and the temporal relationship 1. Cognitive concern reflecting a change in cognition reported by patient or
of the neurocognitive syndrome with one or more cerebrovascular events is informant or clinician.
not established.
2. Objective evidence of impairment in one or more cognitive domains,
typically including memory.
3. Preservation of independent in functional abilities.
4. Not demented.
5. Etiology of MCI consistent with AD pathophysiological process :
rule out vascular, traumatic, medical causes of cognitive decline,
where possible.
provide evidence of longitudinal decline in cognition, when feasible.
report history consistent with AD genetic factors, where relevant

Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

44 45
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4.5 AHA-ASA criteria for vascular MCI 2011


MCI criteria incorporating biomarker
1. Vascular MCI (VaMCI) includes the 4 subtypes proposed for the
Biomarker Neuronal injury classification of MCI: amnestic, amnestic plus other domains, non-
Diagnostic probability of (tau, FDG, amnestic single domain, and non-amnestic multiple domain.
category AD etiology A (PET or CSF) sMRI)
2. The classification of VaMCI must be based on cognitive testing, and a
Conflicting/ Conflicting/ minimum of 4 cognitive domains should be assessed: executive/attention,
MCI - core clinic
Uninformative indeterminant/ indeterminant/
criteria memory, language, and visuospatial functions. The classification should
untested untested
be based on an assumption of decline in cognitive function from a prior
MCI due to AD - Positive Untested baseline and impairment in at least 1 cognitive domain.
intermediate Intermediate
likelihood Untested Positive 3. Instrumental activities of daily living could be normal or mildly impaired,
independent of the presence of motor/sensory symptoms.
MCI due to AD -
Highest Positive Positive
high likelihood

MCI - unlikely due


Lowest Negative Negative
Probable VaMCI
to AD
1. There is cognitive impairment and imaging evidence of cerebrovascular
Abbreviations: AD, Alzheimers disease; A, amyloid beta peptide; PET, positron emission tomography; disease and
CSF, cerebrospinal fluid; FDG, fluorodeoxyglucose; sMRI, structural magnetic resonance imaging.
there is a clear temporal relationship between a vascular event and
onset of cognitive deficits, or
there is a clear relationship in the severity and pattern of cognitive
impairment and the presence of diffuse, subcortial cerebrovascular
disease pathology.
2. There is no history of gradually progressive cognitive deficits before
or after the stroke that suggests the presence of a non-vascular
neurodegenerative disorder.

Reference
1. Albert MS, DeKosky ST, Dickson D, Dubois B, Feldman HH, Fox NC, et al. The diagnosis of mild cognitive
impairment due to Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers
Association workgroups on diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011;7:270-9.

46 47
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4.6 International Working Group on MCI criteria 2004


Possible VaMCI
There is cognitive impairment and imaging evidence of cerebrovascular Presence of a cognitive complaint from either the subject and/ or a family
disease but member

1. There is no clear relationship (temporal, severity, or cognitive pattern) Absence of dementia


between the vascular disease and onset of cognitive deficits. Change from normal functioning
2. There is insufficient information for the diagnosis of VaMCI (e.g., clinical Decline in any area of cognitive functioning
symptoms suggest the presence of vascular disease, but no CT/MRI
Preserved overall general functioning but possibly with increasing
studies are available).
diffculty in the performance of activities of daily living
3. Severity of aphasia precludes proper cognitive assessment.
Sub-classifed to amnestic or non-amnestic; single domain or multiple
4. There is evidence of other neurodegenerative disease or conditions in domain
addition to cerebrovascular disease that may affect cognition.

Cognitive Complaint

Not Normal for Age


No Dementia Cognitive Decline
Essentially Normal Functional Activities

MCI

Yes Memory Impaired? No

Amnestic MCI Nonamnestic MCI

Memory Single Nonmemory


Yes No Yes No
Impairment Only? Cognitive Domain Impaired?

Amnestic MCI Amnestic MCI Nonamnestic MCI Nonamnestic MCI


Single Domain Multiple Domains Single Domain Multiple Domains

Reference Reference
1. Gorelick PB, Scuteri A, Black SE, DeCarli C, Greenberg SM, Iadecola C, et al. Vascular contributions to 1. Winblad B, Palmer K, Kivipelto M, Jelic V, Fratiglion L, Wahlund L-O, et al. Mild cognitive impairment-beyond
cognitive impairment and dementia: a statement for healthcare professionals from the American Heart controversies, towards a consensus: report of the International Working Group on Mild Cognitive Impairment.
Association/American Stroke Association. Stroke 2011;42(9):2672 - 2713. J Intern Med 2004;256(3):240-6.

48 49
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.4.7 Petersen criteria for mild cognitive impairment 1999 Practical notes on MCI
The concept of MCI has been developed since 1999 with the classical
Presence of a subjective memory complaint Petersen criteria for MCI. The diagnosis is made when there is a cognitive
impairment for their age and education, and that the deficit does not interfere
Preserved general intellectual functioning significantly with their daily activities. It is often considered to be the boundary
Demonstration of a memory impairment by cognitive testing or transitional stage between normal aging and dementia, although some
may remain stable and some may even revert back to normal (e.g., those due
Intact ability to perform activities of daily living
to depression or medical conditions). The annual conversion rate of amnestic
Absence of dementia MCI to Alzheimers dementia is approximately 5-10%. The 2004 International
Working Group on MCI criteria included the component of increasing
difficulty in performing everyday tasks without the loss of autonomy, like
changes in the areas of dressing, use of telephone, money and household
appliances. In addition, it also included cognitive deficits other than memory
and sub-classified MCI into amnestic or non-amnestic, and single or multiple
domain types. This direction has been followed on in the updated criteria
from DSM-5, NIA-AA and AHA-ASA, wherein MCI is broadly defined rather
than specifically tied up with only memory deficit or AD.

Reference
1. Petersen RC, Smith GE, Waring SC, Ivnik RJ, Tangalos EG, Kokmen E. Mild cognitive impairment: clinical
characterization and outcome. Arch Neurol 1999;56(3):303-8.

50 51
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.5 Dementia with Lewy bodies (DLB) and


Parkinsons disease dementia (PDD) D. The disturbance is not better explained by cerebrovascular disease,
1.5.1 DSM-5 criteria for neurocognitive disorder another neurodegenerative disease, the effects of a substance, or
with Lewy bodies 2013 another mental, neurological, or systemic disorder.

Major or Mild Neurocognitive Disorder With Lewy Bodies


Diagnostic Criteria
A. The Criteria are met for major or mild neurocognitive disorder.
B. The disorder has an insidious onset and gradual progression.
C. The disorder meets a combination of core diagnostic features
and suggestive diagnostic features for either probable or possible
neurocognitive disorder with Lewy bodies.
For probable major or mild neurocognitive disorder with Lewy
bodies, the individual has two core features, or one suggestive feature
with one or more core features.
For possible major or mild neurocognitive disorder with Lewy
bodies, the individual has only one core feature, or one or more
suggestive features.
1. Core diagnostic features:
a. Fluctuating cognition with pronounced variations in attention and
alertness.
b. Recurrent visual hallucinations that are well formed and detailed.
c. Spontaneous features of parkinsonism, with onset subsequent to
the development of cognitive decline.
2. Suggestive diagnostic features:
a. Meets criteria for rapid eye movement sleep behavior disorder. Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
b. Severe neuroleptic sensitivity. Arlington, VA, American Psychiatric Association, 2013.

52 53
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.5.2 DSM-5 criteria for neurocognitive disorder due to 1.5.3 Parkinsons disease dementia criteria 2007
Parkinsons disease 2013

1. The core features must be present:


Major or Mild Neurocognitive Disorder Due to Parkinsons Prior diagnosis of Parkinsons disease
Disease
A dementia syndrome causing a decline in function severe enough to
Diagnostic Criteria impair the patient in daily activities
A. The criteria are met for major or mild neurocognitive disorder.
B. The disturbance occurs in the setting of established Parkinsons disease. 2. Associated clinical features:
C. There is insidious onset and gradual progression of impairment. At least 2 cognitive domains:

D. The neurocognitive disorder is not attributable to another medical Impaired attention - which may fluctuate over time
condition and is not better explained by another mental disorder. Impaired executive function - Impairment in complex thought
Major or mild neurocognitive disorder probably due to Parkinsons processes such as in initiating an action, planning, or organization
disease should be diagnosed if 1 and 2 are both met. Major or mild Impaired visuo-spatial ability - Marked deficits in the processing
neurocognitive disorder possibly due to Parkinsons disease should be of visual-spatial material
diagnosed if 1 or 2 is met:
Impaired free recall memory - which improved with cueing
1. There is no evidence of mixed etiology (i.e., absence of other
neurodegenerative or cerebrovascular disease or another neurological, At least one behavioral symptoms
mental, or systemic disease or condition likely contributing to cognitive Apathy - Decreased spontaneity, motivation, effortful behavior
to cognitive decline).
Changes in personality and mood - Can include depression and
2. The Parkinsons disease clearly precedes the onset of the neurocognitive anxiety
disorder.
Hallucinations - Usually complex and visual
Delusions - Usually paranoid delusions, such as infidelity or
perceived unknown guests in the home
Excessive daytime sleepiness

Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

54 55
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.5.4 Revised criteria for the clinical diagnosis of


3. Features which do not exclude PDD, but make the dementia with Lewy bodies (DLB) 2005
diagnosis uncertain:
Existence of an abnormality which causes cognitive impairment Central feature (essential for a diagnosis of possible or
although not determined to cause dementia probably DLB)
If the duration of time between the onset of motor and cognitive Dementia defined as progressive cognitive decline of sufficient magnitude
symptoms is not known to interfere with normal social or occupational function
Prominent or persistent memory impairment may not necessarily occur
4. None of the following features present:
in the early stages but is usually evident with progression
Cognitive or behavioral symptoms which occur only in existing
Deficits on tests of attention, executive function, and visuospatial ability
conditions, such as systemic diseases, drug intoxication, or major
may be especially prominent
depression
Symptoms compatible with vascular dementia Core features (two core features for probable DLB, one for
possible DLB)
Fluctuating attention and concentration
Recurrent well-formed visual hallucinations
Spontaneous feature of parkinsonism

Suggestive features ( 1 suggestive features and 1 core


features for probable DLB, 1 suggestive features and
without core feature for possible DLB)
REM sleep behaviour disorder
Severe neuroleptic sensitivity
Low dopamine transporter uptake in basal ganglia demonstrated by
SPECT or PET imaging
Reference
1. Emre M, Aarsland D, Brown R, Burn DJ, Duykaerts C, Mizuno Y, et al. Clinical diagnostic criteria for dementia
associated with Parkinsons disease. Movement Disorders 2007;22(12):1689-707.

56 57
SECTION 1
Diagnostic criteria of neurocognitive disorders

Supportive features Temporal sequence of symptoms


Repeated falls and syncope DLB should be diagnosed when dementia occurs concurrently or within
one year of parkinsonism (if it is present)
Transient, unexplained loss of consciousness
Severe autonomic dysfunction
Hallucinations in other modalities
Systematized delusions
Depression
Relative preservation of medial temporal lobe structure on CT/MRI scan
Generalized low uptake on SPECT/PET perfusion scan with reduced
occipital activity
Abnormal (low uptake) MIBG myocardial scintigraphy
Prominent slow wave activity on EEG with temporal lobe transient sharp
waves

A diagnosis of DLB is less likely


In the presence of cerebrovascular disease evident as focal neurologic
signs or on brain imaging
In the presence of any other physical illness or brain disorder sufficient
to account in part or in total for the clinical picture
If parkinsonism only appears for the first time at a stage of severe
dementia

Reference
1. McKeith IG, Dickson DW, Lowe J, Emre M, OBrien JT, Feldman H, et al. Diagnosis and management of
dementia with Lewy bodies: third report of DLB Consortium. Neurology 2005;65(12):1863-72.

58 59
SECTION 1
Diagnostic criteria of neurocognitive disorders

Practical notes on PDD and DLB DLB and PDD


Dementia with Lewy bodies (DLB) DLB and PDD share many common pathological and clinical features.
Whether they are different entities or part of the same Lewy body disease
McKeith diagnostic criteria have been the most widely accepted
is still debatable.
diagnostic criteria for DLB. The dementia features are different from that
of Alzheimers disease, with less memory loss and more impairment on In terms of neuropsychiatric profiles, DLB/ PDD patients have more
visuospatial constructional tasks, psychomotor slowing, judgment and visuoperceptual, visucontructional and attentional deficits, while AD
problem solving. patients have more prominent memory impairment. Hence, MMSE may
not be sensitive enough for screening DLB/ PDD while the Montreal
The patients have fluctuation in alertness, cognition or function over hours
Cognitive Assessment (MoCA) has adequate psychometric properties
or days, which may resemble delirium. They have visual hallucinations
and may serve as a more appropriate screening instrument.
with detailed, formed images of people or animals. About 50-80% of
them have parkinsonism features. The temporal sequence of symptoms is most crucial in the differentiation
between DLB and PDD. In DLB, dementia occurs before, at the same
Parkinsons disease dementia (PDD) time, or shortly after the onset of parkinsonism. If parkinsonism is present
for more than one year prior to the onset of dementia, the 1-year rule
About 18-40% of patients with Parkinsons disease have dementia. A could apply and the case can be classified as PDD.
diagnosis of Parkinsons disease must be made years before dementia
was noted. In general, Parkinsons disease should exist at least 1 year Other helpful features includes: DLB has faster clinical decline, decreased
prior to the onset of dementia (the 1-year rule). This is to distinguish L-dopa responsiveness, less prominent tremors and more cognitive
PDD from DLB which may present with both dementia and parkinsonism fluctuation.
within a short period of time. The DSM-5 criteria for NCD with Lewy bodies and NCD due to Parkinsons
Risk factors for developing dementia include more advanced Parkinsons disease are in general in line with the expert panel criteria.
disease, co-existing depression and the absence of prominent tremor.

References
1. Hoops S, Nazem S, Siderowf D, Duda JE, Xie SX, Stern MB, et al. Validity of the MoCA and MMSE in the detection
of MCI and dementia in Parkinson disease. Neurology 2009;73(21):173845.
2. Emre M, Aarsland D, Brown R, Burn DJ, Duyckaerts C, Mizuno Y, et al. Clinical diagnostic criteria for dementia
associated with Parkinsons disease. Movement disorders 2007;22(12):1689-707.
3. McKeith IG, Dickson DW, Lowe J, Emre M, OBrien JT, Feldman H, et al. Diagnosis and management of dementia
with Lewy bodies: third report of DLB Consortium. Neurology 2005;65(12):1863-72.

60 61
SECTION 1
Diagnostic criteria of neurocognitive disorders

1.6 Frontotemporal dementia (FTD)


1.6.1 DSM-5 criteria for frontotemporal neurocognitive Probable frontotemporal neurocognitive disorder is diagnosed if either of
disorder 2013 the following is present; otherwise, possible frontotemporal neurocognitive
distorder should be diagnosed:
1. Evidence of a causative frontotemporal neurocognitive disorder genetic
Major or Mild Frontotemporal Neurocognitive Disorder mutation, from either family history or genetic testing.
Diagnostic Criteria 2. Evidence of disproportionate frontal and/or temporal lobe involvement
A. The criteria are met for major or mild neurocognitive disorder. from neuroimaging.

B. The disturbance has insidious onset and gradual progression. Possible frontotemporal neurocognitive disorder is diagnosed if there
is no evidence of a genetic mutation, and neuroimaging has not been
C. Either (1) or (2): preformed.
1. Behavioral variant:
a. Three or more of the following behavioral symptoms:
i. Behavioral disinhibition.
ii. Apathy or inertia.
iii. Loss of sympathy or empathy.
iv. Perseverative, stereotyped or compulsive/ritualistic behavior.
v. Hyperorality and dietary changes.
b. Prominent decline in social cognition and /or executive abilities.
2. Language variant:
a. Prominent decline in language ability, in the form of speech
production, word finding, object naming, grammar, or word
comprehension.
D. Relative sparing of learning and memory and perceptual-motor function.
E. The disturbance is not better explained by cerebrovascular disease,
Reference
another neurodegenerative disease, the effects of a substance, or
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
another mental, neurological, or systemic disorder. Arlington, VA, American Psychiatric Association, 2013.

62 63
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Diagnostic criteria of neurocognitive disorders

1.6.2 The International Behavioural Variant FTD Criteria


Consortium (FTDC) - Diagnostic criteria for D. Early preservative, stereotyped or compulsive/ritualistic behaviour (one
behavioural variant FTD 2011 of the following symptoms must be present) :
D.1. Simple repetitive movements
I. Neurodegenerative disease D.2. Complex, compulsive or ritualistic behaviours
The following symptoms must be present to meet criteria for behavioural
E. Hyperorality and dietary changes (one of the following symptoms must
variant FTD (bvFTD)
be present):
A. Shows progressive deterioration of behaviour and/or cognition by
E.1. Altered food preferences
observation or history
E.2. Binge eating, increased consumption of alcohol or cigarettes
II. Possible bvFTD E.3. Oral exploration or consumption of inedible objects
Three of the following behavioural/cognitive symptoms (A-F) must be present F. Neuropsychological profile: executive/generation deficits with relative
to meet criteria. sparing of memory and visuospatial functions (all of the following
A. Early (first 3 years) behavioural disinhibition (one of the following symptoms symptoms must be present) :
must be present) : F.1 Deficits in executive tasks
A.1. Socially inappropriate behaviour F.2. Relative sparing of episodic memory
A.2. Loss of manners or decorum F.3. Relative sparing of visuospatial skills
A.3. Impulsive, rash or careless actions
B. Early (first 3 years) apathy or inertia (one of the following symptoms
must be present) :
B.1. Apathy
B.2. Inertia
C. Early (first 3 years) loss of sympathy or empathy (one of the following
symptoms must be present) :
C.1. Diminished response to other peoples needs and feelings
C.2. Diminished social interest, interrelatedness or personal
warmth

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Diagnostic criteria of neurocognitive disorders

III. Probable bvFTD V. Exclusionary criteria for bvFTD


All of the following symptoms must be present to meet criteria. Criteria A and B must be answered negatively for any bvFTD diagnosis.
Criterion C can be positive for possible bvFTD but must be negative for
A. Meets criteria for possible bvFTD
probable bvFTD.
B. Exhibits significant functional decline (by caregiver report or as evidenced
A. Pattern of deficits is better accounted for by other non-degenerative
by Clinical Dementia Rating Scale or Functional Activities Questionnaire
nervous system or medical disorders
scores)
B. Behavioral disturbance is better accounted for by a psychiatric diagnosis
C. Imaging results consistent with bvFTD (one of the following must be
present): C. Biomarkers strongly indicative of Alzheimers disease or other
neurodegenerative process
C.1. Frontal and/or anterior temporal atrophy on MRI or CT
C.2. Frontal and/or anterior temporal hypoperfusion or
hypometabolism on PET or SPECT

IV. bvFTD with definite frontotemporal lobar degeneration


(FTLD) pathology
Criterion A and either criterion B or C must be present to meet criteria.
A. Meets criteria for possible or probable bvFTD
B. Histopathological evidence of FTLD on biopsy or at post-mortem
C. Presence of a known pathogenic mutation

Reference
1. Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, et al. Sensitivity of revised
diagnostic criteria for the behavioral variant of frontotemproal dementia. Brain 2011;134(9):2456-77.

66 67
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Diagnostic criteria of neurocognitive disorders

1.6.3 International Consensus Criteria - Classification of


primary progressive aphasia and its variants 2011 2. Diagnostic features for the nonfluent/agrammatic variant PPA
I. Clinical diagnosis of nonfluent/agrammatic variant PPA
1. Inclusion and exclusion criteria for the diagnosis of primary
progressive aphasia (PPA) : At least one of the following core features must be present:

Inclusion: criteria 1-3 must be answered positively 1. Agrammatism in language production

1. Most prominent clinical feature is difficulty with language 2. Effortful, halting speech with inconsistent speech sound errors and
distortions (apraxia of speech)
2. These deficits are the principal cause of impaired daily living activities
At least 2 of 3 of the following other features must be present:
3. Aphasia should be the most prominent deficit at symptoms onset
and for the initial phases of the disease 1. Impaired comprehension of syntactically complex sentences

Exclusion: criteria 1-4 must be answered negatively for a PPA diagnosis 2. Spared single-word comprehension

1. Pattern of deficits is better accounted for by other nondegenerative 3. Spared object knowledge
nervous system or medical disorders II. Imaging-supported nonfluent/agrammatic variant diagnosis
2. Cognitive disturbance is better accounted for by a psychiatric Both of the following criteria must be present:
diagnosis
1. Clinical diagnosis of nonfluent/agrammatic variant PPA
3.
Prominent initial episodic memory, visual memory, and
2. Imaging must show one or more of the following results:
visuoperceptual impairments
a. Predominant left posterior fronto-insular atrophy on MRI or
4. Prominent, initial behavioural disturbance
b. Predominant left posterior fronto-insular hypoperfusion or
hypometabolism on SPECT or PET
III. Nonfluent/agrammatic variant PPA with definite pathology
Clinical diagnosis (criterion 1 below) and either criterion 2 or 3 must be
present:
1. Clinical diagnosis of nonfluent/agrammatic variant PPA
2. Histopathologic evidence of a specific neurodegenerative pathology
3. Presence of a known pathogenic mutation

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3. Diagnostic criteria for the semantic variant PPA 4. Diagnostic criteria for logopenic variant PPA
I. Clinical diagnosis of semantic variant PPA I. Clinical diagnosis of logopenic variant PPA
Both of the following core features must be present: Both of the following core features must be present:
1. Impaired confrontation naming 1. Impaired single-word retrieval in spontaneous speech and naming
2. Impaired single-word comprehension 2. Impaired repetition of sentences and phrases
At least 3 of the following other diagnostic features must be present: At least 3 of the following other features must be present:

1. Impaired object knowledge, particularly for low-frequency or low- 1. Speech (phonologic) errors in spontaneous speech and naming
familiarity items 2. Spared single-word comprehension and object knowledge
2. Surface dyslexia or dysgraphia 3. Spared motor speech
3. Spared repetition 4. Absence of frank agrammatism
4. Spared speech production (grammar and motor speech) II. Imaging-supported logopenic variant diagnosis
II. Imaging-supported semantic variant PPA diagnosis Both criteria must be present:

Both of the following criteria must be present: 1. Clinical diagnosis of logopenic variant PPA

1. Clinical diagnosis of semantic variant PPA 2. Imaging must show at least one of the following results:
a. Predominant left posterior perisylvian or parietal atrophy on MRI
2. Imaging must show one or more of the following results:
b. Predominant left posterior perisyvian or parietal hypoperfusion of
a. Predominant anterior temporal lobe atrophy
hypometabolism on SPECT or PET
b. Predominant anterior temporal hypoperfusion or hypometabolism
III. Logopenic variant PPA with definite pathology
on SPECT or PET
Clinical diagnosis (criterion 1 below) and either criterion 2 or 3 must be
III. Semantic variant PPA with definite pathology present:
Clinical diagnosis (criterion 1 below) and either criterion 2 or 3 must be 1. Clinical diagnosis of logopenic variant PPA
present:
2. Histopathologic evidence of a specific neurodegenerative pathology
1. Clinical diagnosis of semantic variant PPA
3. Presence of a known pathogenic mutation
2. Histopathologic evidence of a specific neurodegenerative pathology
Reference
3. Presence of known pathogenic mutation 1. Gorno-Tempini ML, Hills AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, et al. Classification of primary
progressive aphasia and its variants. Neurology 2011;76(11):1006-14.

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Diagnostic criteria of neurocognitive disorders

1.6.4 Consensus criteria for frontotemporal dementia


(FTD) by Neary et al 1998 Supportive diagnostic features
A. Behavioural disorder
Clinical profile summarizes the neurobehavioural profile necessary to
fulfil criteria for diagnosis Decline in personal hygiene and grooming

Cores diagnostic features must be all present for diagnosis Mental rigidity and inflexibility

Supportive diagnostic features are not necessary conditions for diagnosis Distractibility and impersistence
but they add substantial weight to the clinical diagnosis Hyperorality and dietary changes
Supportive features are common to all clinical syndromes of Perseverative and stereotyped behaviour
frontotemporal lobar degeneration (FTD), progressive nonfluent aphasia
Utilization behaviour
and semantic dementia. These features support but not necessary for
diagnosis B. Speech and language
Diagnostic exclusion features must be absent Altered speech output
Relative diagnostic exclusion features are against but do not firmly Aspontaneity and economy of speech
exclude diagnosis Press of speech

Clinical profile Stereotypy of speech

Character change and disordered social conduct are the dominant features Echolalia
initially and throughout the disease course. Instrumental functions of Perseveration
perception, spatial skills, praxis, and memory are intact or relatively well
Mutism
preserved.
C. Physical signs
Core diagnostic features Primitives reflexes
Insidious onset and gradual progression Incontinence
Early decline in social interpersonal conduct Akinesia, rigidity, and tremor
Early impairment in regulation of personal conduct Low and liable blood pressure
Early emotional blunting
Early loss of insight

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Diagnostic criteria of neurocognitive disorders

D. Investigations Relative diagnostic exclusion features


Neuropsychology: significant impairment on frontal lobe tests in the Typical history of chronic alcoholism
absence of severe amnesia, aphasia, or perceptuospatial disorder
Sustained hypertension
Electroencephalography: normal on conventional EEG despite
History of vascular disease
clinical evident dementia
Brain imaging (structural and/or functional): predominant frontal and/
or anterior temporal abnormality

Supportive features
Onset before 65 years: positive family history of similar disorder in first-
degree relatives
Bulbar palsy, muscular weakness and wasting, fasciculations

Diagnostic exclusion features


A. Historical and clinical
Abrupt onset with ictal events Head trauma related to onset
Early, severe dementia Spatial disorientation
Myoclonus Corticospinal weakness
Cerebellar ataxia Choreoathetosis
Logoclonic, festinant speech with loss of train of thought
B. Investigations
Brain imaging: predominant postcentral structural or functional
deficits; multifocal lesions on CT or MRI
Laboratory tests indicating brain involvement of metabolic or
Reference
inflammatory disorder
1. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S. Frontotemporal lobar degeneration: a
consensus on clinical diagnostic criteria. Neurology 1998;51(6):1546-54.

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Diagnostic criteria of neurocognitive disorders

Practical notes on Frontotemporal dementia Practical guide on frontal lobe assessment


Two broad presentations of frontotemporal dementia (FTD) are recognized:
1. History
behavioral variant FTD (bvFTD) and primary progressive aphasia (PPA).
The 1998 consensus criteria by Neary and colleagues are the most widely Personality changes
adopted diagnostic guideline for FTD. After years of knowledge accumulation,
Hyperorality
a revised guideline for bvFTD and a new classification system for PPA and its
three main clinical variants, were published in 2011. Distractibility
Poor motivation
Revised diagnostic criteria for the behavioural variant of frontotemporal
dementia 2011 Inability to adapt to new situations
Poor problem solving skills
The revised guideline for bvFTD was developed by the International
Behavioral Variant FTD Criteria Consortium (FTDC). It simplified the 1998
criteria and focused on features that best distinguish bvFTD from psychiatric 2. Frontal Release Reflexes
disorders, Alzheimers disease and other dementing conditions. Hence, in Grasp: Stroke patients palm from radial to ulnar side. Patient will
the new criteria, there is no longer need to distinction between core and
grasp your hand.
supportive features, and does not need to take account of a number of
restrictive exclusion features. These improve the sensitivity of the new Pout: Stroke the filtrum or tap a spatula placed over lips. The lips
criteria compared with the 1998 criteria. Another advance in the new criteria will pout or make a contraction resembling a snout.
is the diagnostic hierarchy in which distinction between possible, probable
or definite bvFTD with frontotemporal lobar degeneration (FTLD) pathology Palmomental: Stroke patients thenar eminence and the patients mentalis
depends on the level of diagnostic certainty. muscle will contract.

Classification of primary progressive aphasia and its variants 2011


A new classification system for PPA and its 3 main variants (non-fluent/
agrammatic, semantic, and logopenic) was formulated by an international
group of PPA investigators, with the objective of unifying the heterogeneous
clinical criteria adopted across centres. With the new classification system,
patients should first meet basic PPA criteria. Then, they are divided into
clinical variants based on specific speech and language features. They
can be further specified as imaging-supported if the expected pattern
of atrophy is found and with definite pathology if pathologic or genetic
data are available. It is worth noting that PPA has clinical and pathological
overlap of FTLD spectrum of disorder and Alzheimers disease pathology.
Furthermore, there is no direct correlation between PPA clinical phenotypes
and pathologic or genetic findings.

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3. Simple Bedside Tests Tips for clinicians


Abstract thinking: 1. It is not practical to do all the frontal lobe tests during the busy clinic time.
The above bedside tests may serve to immediately test the suspicion
Proverb interpretation
generated during history taking.
People in glass houses should not throw stones at others
2. MMSE, while commonly used, is an insensitive tool for screening frontal
Similarities test lobe dysfunction.
What are the similarities between apple and banana / table 3. MoCA includes several tasks sensitive to frontal lobe dysfunction:
and chair?
mini trail-making test for visuospatial / executive function
Cognitive estimates:
tests of attention,
What is the height of an average woman?
verbal fluency
What is now the population of Hong Kong
similarities test for abstraction.
Verbal fluency:
Tell me the names of as many animals as you can think of, as
quickly as possible.
Lurias three-step test (Motor sequencing):
Tell the patient that you are going to show them a series of
hand movements. Demonstrate fist, edge and palm five times
on your leg without verbal prompts. Ask the patient to repeat
the sequence.
Go / No go test:
Ask the patient to make a response to one signal (Go) and not
to respond to another signal (No go). The most basic is to ask
the patient to tap their knee when examiner says, Go and to
make no response when the examiner says, Stop.
Stroop Test:
Ask the patient to state the colour in which words are printed
rather than words themselves, e.g., RED may be printed in
blue, the patient should say BLUE; GREEN may be printed References
1. Kipps CM, Hodges JR.Cognitive assessment for clinicians. J Neurol Neurosug Psychiatry 2005;76(Suppl I): i22-30.
in red, the patient should say RED.
2. Simple psychiatry. www.simplypsychiatry.co.uk/

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Diagnostic criteria of neurocognitive disorders

1.7 Delirium
1.7.1 DSM-5 criteria for delirium 2013 Specify cause:
Substance intoxication delirium
Substance withdrawal delirium
A. A disturbance in attention (i.e., reduced ability to direct, focus,
Medication-induced delirium
sustain, and shift attention) and awareness (reduced orientation to the Delirium due to another medical condition
environment). Delirium due to multiple aetiologies
B. The disturbance develops over a short period of time (usually hours to a
Specify onset and duration:
few days), represents a change from baseline attention and awareness,
Acute: Lasting a few hours or days
and tends to fluctuate in severity during the course of a day.
Persistent: Lasting weeks or months
C. An additional disturbance in cognition (e.g., memory deficit,
disorientation, language, visuospatial ability, or perception). Specify type:
Hyperactive
D. The disturbances in Criteria A and C are not better explained by another Hypoactive
preexisting established, or evolving neurocognitive disorder and do not Mixed level of activity
occur in the context of a severely reduced level of arousal, such as
coma.
E. There is evidence from history, physical examination, or laboratory
findings that the disturbance is a direct physiological consequence of
another medical condition, substance intoxication or withdrawal (i.e.,
due to a drug of abuse or to medications), or exposure to a toxin, or is
due to multiple etiologies.

Reference
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
Arlington, VA, American Psychiatric Association, 2013.

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1.7.2 Confusion Assessment Method 1990 Practical notes on DSM-5 criteria and the Confusion
Assessment Method (CAM) for delirium
The presence of features (1), (2), and either (3 or 4). Delirium is common in older adults with or without underlying dementia,
and is an emergency condition. In DSM-5, the exclusion of delirium is a
1. Acute onset and fluctuating course prerequisite for the diagnosis of any form of NCD. DSM-5 has timely added
2. Inattention in the clinically relevant aetiological and descriptive specifiers to establish
a comprehensive framework for the diagnosis of delirium. This can assist
3. Disorganized thinking
clinicians in the management of older delirious patients. Medication-induced
4. Altered level of consciousness delirium, delirium due to another medical condition and delirium due to
multiple aetiologies are common underlying causes of delirium in old age. It
is worth noting that persistent delirium, on contrary to the conventional acute
and short-lived delirium, can last for months. In reality, persistent delirium
frequently occurs in older adults, yet this and the hypoactive form of delirium
remain highly under-recognized in clinical practice. If the DSM-5 diagnostic
criteria for delirium are being observed closely, these deficits hopefully can
be alleviated in the future. Despite the comprehensiveness of the DSM-5
delirium diagnostic criteria, the classical CAM items are still a practical guide
in the detection of delirium for their inherent beauty of simplicity.

Reference
1. Inouye SK, Van Dyck CH, Alessi CA, Balkin S, Siegal AP, Horwitz RI. Clarifying confusion: the confusion assessment
method: A new method for detection of delirium. Annals of internal medicine 1990;113(12):941-8.

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Assessment scales in neurocognitive disorders

2.1 Abbreviated mental test (AMT)


Abbreviated Mental Test (Hong Kong version)
1. Age (+/- 5 years)
2. Time (nearest hour, or a, p, n)
3. Address for recall at the end of the test: 42 Shanghai Street
4. Year (+/- 1 year)
5. Place name

SECTION 2 6. Recognition of two persons (doctor, nurse)


7. Date of birth (day and month)

Assessment scales in 8. Date of mid-Autumn festival


9. Name of present Governor or Chinese leader
neurocognitive disorders 10. Count 20 - 1 backwards

* Each correct response scores 1 mark; no half mark is given


The best cut-off score for Hong Kong geriatric patient is 6, i.e. below 6 is
considered abnormal.

Reference
1. Chu LW, Pei LKW, Ho MH, Chan PT. Validation of the abbreviated Mental Test (Hong Kong version in the elderly
medical patient. HKMJ 1995;1:207-11.

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Abbreviated Mental Test (Original Hodkinsons Version) Practical notes on the Abbreviated Mental Test (AMT)
Test item Scoring instructions* The AMT is a screening test, and not a test to monitor progress or drug
response. Although a lower score is supposed to reflect lower cognitive
Age Score for exact age only function, the test was not designed for this purpose and hence should
not be interpreted as such.
Date of birth Score for correct date and month
(year not required) The AMT can tell us whether the individual is at risk of cognitive
impairment, but not its severity.
Year Score for current year only

Time of day Score if correct to the nearest hour

Place Score if exact address or name of hospital


given (in hospital is insufficient)

Monarch Score for current monarch only

Year of first world war Score for year of start or finish


(both not necessary)

Counting backwards Score if no mistakes or subject corrects


from 20 to 1 himself or herself spontaneously

Recognition of two people Score if roles of two people correctly


recognised - for example, doctor and nurse

Recall of three point address Score if registered correctly near beginning of


such as 42 West Street test and on recall at end of test

*Each correct response scores 1 mark; no half marks given.


Score of less than 7 suggests subject may be confused.

Reference
1. Hodkinson HM. Evaluation of a mental test score for assessment of mental impairment in the elderly. Age Aging
1972;1(4):223-8.

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2.2 AD8 AD-8

Remember. Yes, a change indicates that YES, NO, N/A,


there has been a change in the last several A change No change Dont know ()
years caused by cognitive (thinking and
memory) problems.
1.
1. Problems with judgement (e.g., problems
making decisions, bad finical decisions,
problems with thinking) 2.

2. Less interest in hobbies/activities


3.
3. Repeats the same things over and over
(questions, stories, or statements) 4.

4. Trouble learning how to use a tool, appliance, ()
or gadget (e.g., VCR, computer, microwave,
remote control) 5.

5. Forgets correct month or year 6.



6. Trouble handling complicated financial affairs
(e.g., balancing checkbook, income taxes, 7.
paying bills)

7. Trouble remembering appointments 8.

1
8. Daily problems with thinking and/or memory AD8
0

TOTAL AD8 SCORE 2009

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Assessment scales in neurocognitive disorders

Practical notes on the AD8 2.3 Clock drawing test (CDT)


The AD8 is a short and simple informant based screening tool to detect The clock drawing test is a simple and popular screening tool for dementia.
cognitive impairment with good sensitivity (> 84%), specificity (> 80%), It takes two minutes and is a useful adjunct to the MMSE for a quick survey
positive predictive value ( > 85%), negative predictive value ( > 70%), and of global cognitive functions.
area under the curve (AUC) (0.908).
There are many variations but the usual instructions to the patient are to
The AD8 contains 8 items that test for memory, orientation, judgment, and draw the clock face in a pre-drawn circle marking the hours and with the
function. hands to indicate a particular time ( e.g.10 minutes past 11)
Each item requires the responder to indicate : Yes, A change, or No, No
change, or Dont know. The final score is a sum of items marked Yes, A Practical notes for CDT
change. 1. Cognitive domains tested in CDT reflects frontal and temporo-parietal
Cut-off points are: 0-1 for normal cognition, 2 or greater for impairment in functioning, such as
cognition. Auditory comprehension
Scores in the impaired range indicate a need for further assessment. Scores Language
in the normal range suggest that a dementing disorder is unlikely, but a
very early disease process cannot be ruled out. Memory

It is preferable to administer the AD8 to an informant. If an informant is not Abstract thinking


available, it can be administered to the person to be screened. Conceptualisation of time
A Chinese version has been validated with good reliability (sensitivity 93.9%, Visuo-spatial organization
specificity 76.0%, AUC 0.93)
Inhibition of distracting stimuli
Goal directed action planning

References
1. Galvin JE, Roe CM, Powlishta KK, Coats MA, Muich SJ, Grant E, et al. The AD8 A brief informant interview to detect
dementia. Neurology 2005;65(4):559-64.
2. Li T, Wang HL, Yang YH, Galvin JE, Morris JC, Yu X. The reliability and validity of Chinese version of AD8. Zhonghua
nei ke za zhi [Chinese Journal of Internal Medicine] 2012;51(10):777-80.

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Assessment scales in neurocognitive disorders

2. Scoring systems 3. Qualitative interpretation


Numerous methods exist but none are proven superior in the screening for Simply observing the patient relays useful information to the physician
dementia, with similar sensitivity and specificity of around 86% and 87%
Provides an educational impact on families
respectively. The positive and negative predictive values ranged from 93-97%
and 70-74% respectively, assuming a 20% clinic prevalence of dementia. Interpretation of common errors (ref 3) may assist understanding of
the patients cognitive deficits within the context of the presenting
For reference, a simple and a local version are described briefly.
symptoms.
Simple method - Scoring system adopted from Mini-cog test
(sensitivity 85% , specificity 86% ) (ref 1). FIGURE 1. Representative illustration of common types of clock-drawing errors.

1 = correct if clock looks grossly normal with placement of correct Graphical Diffculties Stimulus Bound Conceptual Deficits Spatial/Planning Perseveration
Response Deficits
time
0 = incorrect
Hong Kong version as screening test in the elderly with low
educational levels (sensitivity 83%, specificity 79% ) (ref 2). It is a
2-part composite CDT with
Adapted from reference 3.
a. clock drawing scored with 0-10 points (Figure 2). Cut off points
3/4. 1. Graphical errors - hands and numbers imprecise and clumsy, but still appear
recognizable as a clock.
b. reading and setting the time on a toy clock.
Impaired fine motor control and planning from disrupted frontostriatal circuits in, e.g.,
vascular dementia
2. Stimulus-bound response errors - drawing is dominated by a single stimulus, usually
related to the time-setting instruction.
E.g., instructed to Set time to 11:10, patient fail to recode the 10 min into setting the
minute hand at 2.
3. Conceptual deficits - misrepresentations of the clock and time (e.g., does not look
like a clock, hands do not communicate a time, hands are absent, time written on
the clock). Impaired access to knowledge store of factual and conceptual knowledge,
i.e. semantic memory of meaning/features of a clock, a function of lateral temporal
lobes which is affected earlier in Alzheimers disease (AD) than vascular dementia (VaD),
Parkinsons disease dementia (PDD) or frontotemporal dementia (FTD).
4. Spatial and/or planning deficits - planning difficulties with irregular spacing between
numbers, left hemi-neglect, numbers written outside clock-face or counter-clockwise
direction. Impaired parietal lobe function, greater in PDD, DLB, VaD, AD than in FTD.
5. Perseveration - recurrence of activity without an appropriate stimulus, e.g., drawing
more than 2 hands, repeating same numbers, or writing more beyond 12. These errors
are common in AD and may be localized to the prefrontal area of the frontal lobe.

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FIGURE 2. Hong Kong CDT version

Instructions :
6 Abnormal clock-face drawing with inaccurate time
Fill inside a pre-drawn circle of 2.5 diameter the numbers of a clock with arms
denotation (e.g. reversal of numbers, perseveration
indicating the 3 oclock position. beyond twelve, misplaced numbers, drawing only
to one side, omitting most numbers)
Scoring Criteria for Clock Drawing Test (CDT)
Score Criteria Example 7 A recognizable attempt to draw a clock face but
with no clear denotation of time
0 Correct denotation of time with normal spacing

8 Some evidence that a clock face is drawn

1 Slight impairment in spacing of lines or numbers

9 Minimal evidence that a clock face is drawn

2 Noticeable impairment in line spacing

10 No reasonable or understandable attempt of


drawing a clock face (excluding gross visual
3 Incorrect spacing between spokes (or numbers) disturbance, hemiplegia, severe psychotic state,
with subsequent inappropriate denotation of time rendering uncooperativeness)

Adapted from reference 2.


4 Obvious errors in correct time denotation
(e.g. arms grossly misplaced, numbers drawn at
wrong places)
References
1. Krner EA, Lauritzen L , Nilsson FM , Lolk A, Christensen P. Simple scoring of the Clock-Drawing Test for dementia
5 More obvious errors in time denotation screening. Dan Med J 2012;59(1):A4365.

(e.g. one arm omitted, circle the numbers or 2. Lam LCW, Chiu FK, Ng KO, Chan C, Chan WF, Li SW, Wong M. Clock-face drawing, reading and setting tests in the
screening of dementia in Chinese elderly adults. The Journal of Gerontology Series B: Psychological Sciences and
lines of clock face instead of drawing arms) Social Sciences 1998;53(6):353-7.
3. Eknoyan D, Hurley R, Taber K. The clock drawing task: Common errors and functional neuroanatomy. J
Neuropsychiatry Clin Neurosci 2012;24(5):260-5.

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2.4 Mini-mental state examination (MMSE) Performing the test


The references of the mini-mental state examination (MMSE) are shown as The Serial 7: The patient should be instructed by How much is 100
below: minus 7?, then minus 7 again only ONCE more, then just say do it
again until I tell you to stop. If the patient stops prematurely, remind by
1. Folstein MF, Folstein SE, McHugh PR. Mini-mental state. A practical
continue to subtract, but never by prompting them with the previous
method for grading the cognitive state of patients for the clinician. J
number they got, or the 7. It is a test of STM as well as concentration
Psychiat Res 1975;12(3):189-98
and some abstract thinking.
2. Chiu HFK, Lee HK, Chung WS, Kwong PK. Reliability and Validity of
The score for the serial-7: as long as the number given is the previous
the Cantonese version of mini-mental state examination a preliminary
number minus 7, it should be considered correct and 1 point should be
study. J Hong Kong Coll Psychiatr 1994;4 (9Suppl 2): 25-8
given. It does not matter if the previous answer was wrong. For example,
the numbers given were 93, 90 and 83. The 93 and 83 should both
Practical notes on the Mini-Mental State Examination: be given points.
Limitations The 3 step command: Note that the original Folstein version uses put it
It is designed for screening but is also used for monitoring progress in on the floor, while our Hong Kong version uses put it on the table. As
clinical practice nowadays. most people are right-handed, they will automatically pick up the paper
with their dominant hand, whether they understood the command or not
The cut-off values are education level sensitive. In the Cantonese version, (as long as they heard pick up the paper). Some patients merely touch
the cut-off for normal is 19 or above, for those with no formal education, the paper with their right hand, then withdraw their hand and thus leave
but note that the highest education level is > 2 years of education the paper lying on the table. Should we give 2 marks then? Scoring this
(cut-off at 22). A score of 23 might be falsely normal for someone with kind of performance can be difficult, and only when we actually see the
secondary or higher education. performance, we can have a clear idea of the patients understanding
The test is deficient in detecting frontal lobe or executive functions It can or cognitive function. That is why it is often more useful if the clinician
be falsely normal for frontal lobe dysfunction e.g. FTD patients. performs the MMSE him/ herself. If in doubt of a score provided by
others, it is always advisable to repeat it yourself.
Any impairment in communications, for example dysphasia, dialect
barrier, vision and hearing impairment, may result in falsely low scores. Prompting: There should be no prompting. Neutral but kind words such
as its ok or thats fine can be used for encouragement. Instructions
Beware of memorized answers: the test is so often used in elderly can be repeated once only, but only if the patient has not started to
services nowadays (e.g. in social centres) that many older people have response. For the 3-item recall, if the patient repeated one item but
been able to memorize the 3 short term memory items. Although it is asked for prompting for the next 2, record 1 point for registration, then
off-label, if in doubt, one can consider substitution by another 3 similar repeat all 3 items at 1 second per word until all 3 could be remembered,
yet different items (a fruit, an IADL item, and a transport vehicle). This for the delay recall.
is for practical/ clinical use only and is not recommended for formal
studies.

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Cautions
Do not make a diagnosis of dementia based on the MMSE score alone.
Remember the MMSE is only a screening test, and dementia is a very
serious, irreversible and devastating disease. The analogy is that we do
not make a diagnosis of cancer just based on a lung shadow on the
CXR. It could be a wrong score, it could be mild cognitive impairment or
depression, or even a noisy environment during the test.
Try not to make a diagnosis of dementia based on the MMSE performed
during or immediately after a hospitalization or acute illness. The
performance could be affected by physical discomfort, low mood,
medication effects, strange environment, and lost of contact with the
real, usual world.
The usual decline rate on the MMSE for AD patients in observational
studies (without treatment) in the West is 3-4 points per year. In Hong
Kong, however, the decline was noted to be slower, about 2 points
per year. This difference should be taken into consideration when drug
response is being considered.

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2.5 Montreal cognitive assessment (MoCA) VISUOSPATIAL / EXECUTIVE Point


Read list of digits Subject has to repeat them in the [ ] 2 1 8 5 4
NAME : Education : Date of birth :
(1 digit /sec). forward order
Sex : DATE :
Subject has to repeat them in the [ ] 7 4 2
backward order _____ / 2
VISUOSPATIAL / EXECUTIVE Point
Read list of letters. The subject must tap with his hand at each letter A.
No point if 2 errors
E EA A Copy Copy
[ ] FBACMNAAJKLBAFAKDEAAAJAMOFAAB _____ / 1
End End
cube cube

5 5 Serial 7 substraction [ ] 93 [ ] 86 [ ] 79 [ ] 72 [ ] 65
B 2B 2 starting at 100 4 or 5 correct subtractions : 3pt , 2 or 3 correct; 2pts, 1 correct; 1pt, 0 correct 0 pt _____ / 3
1 1
Begin Begin
LANGUAGE
D D 4 4
3 3 Repeat : I only know that John is the one to help today. [ ]
_____ / 2
C C The cat always hid under the couch when dogs were in the room. [ ]
[ ] [ ]
Fluency/ Name maximum number of words in one minute that begin with the letter F
[ ] (N 11 words) _____ / 1
Draw CLOCK (Ten past eleven) (3 points)

ABSTRACTION
Similarity between e.g. banana - orange = fruit [ ] train - bicycle [ ] watch ruler _____ / 2

DELAYED RECALL
Point for
Has to recall words FACE VELVET CHURCH DAISY RED
_____ / 5 UNCUED
Cotour [ ] Number [ ] Hands [ ] WITH NO CUE [ ] [ ] [ ] [ ] [ ] recall only

NAMING Category Cue


Optional
Multiple choice cue _____ / 5

ORIENTATION
[ ] Date [ ] Month [ ] Year [ ] Day [ ] Place [ ] City _____ / 6

z.Nasredding MD Version 7.1 www.mocatest.org Normal 26/30 TOTAL _____ / 30


[ ] [ ] [ ] _____ / 3 Add 1 point if 12 yr edu
Administered by: ____________________________
MEMORY FACE VELVET CHURCH DAISY RED

Read list of words,


subject must repeat 1st trial
them. Do 2 trials,
event if 1st trial is
successful. Do a NO Refernce
2nd trial
recall after 5 minutes. POINT 1. Nasredding ZS, Philips NA, Bdirian V, Charbonneau S, Whitehead V, Collin I, et al. The Montreal Cognitive
Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc 2005;53(4):695-9.

100 101
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[ ] 2 1 8 5 4

[ ] 7 4 2 _____ / 2

1
2
10 10
[ ] 5 2 1 3 7 4 1 1 8 0 6 2 1 5 1 7 4 5 1 1 1 4 1 7 0 5 1 1 2 _____ / 1
10 10 2 2

2

2 3

9

3
99
100 7 [ ] 93 [ ] 86 [ ] 79 [ ] 72 [ ] 65
4 34
1 9
_____ / 3
3
453232110
8
8 1 4
8 7
6 5 4
8 7 56 5 [ ]
7 6 _____ / 2
5
[ ] [ ] [ ]
7 6
3 [ ] N11 _____ / 1


= [ ] [ ] _____ / 2



[ ] [ ] [ ] _____ / 5
[ ] [ ] [ ] [ ] [ ]

_____ / 5


[ ] [ ] [ ] [ ] [ ] [ ] _____ / 6

61
[ ] [ ] [ ] _____ / 3
22

_____ / 30




Nasreddine MD
Hong Kong version 08 June 2010

5 Translated by Wong A and Mok V
www.mocatest.org

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3.






1. 1

1


1
1 1

2 3 10

1

1 2 3 4 5 6 7 8 9

10 10
0

2.
4.




1 1 (1) (2) (3)




0

104 105
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Assessment scales in neurocognitive disorders

5.

1
1

7s







301

23

1007
79285
78716492

3

6. 7.





[]
[]



1

1247

106 107
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Assessment scales in neurocognitive disorders

8. 10.



[] [60 ( )
]


1
601


9.
( )









1


=

=


=
=

108 109
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11. Practical notes on the MoCA


The MoCA is one of the newest tests in the field of cognitive assessment.
It was originally designed for the screening of mild cognitive impairment
(MCI) but now also has a cut-off for possible dementia in addition to
[] possible MCI.
It is a pencil-and-paper test and more so than the MMSE (only drawing
the intersecting pentagons), and thus may be more difficult for those

individuals with lower education. However, that also makes it a more
1 appropriate test for those with higher education, eliminating the ceiling
0 effect in the MMSE.

The MoCA places more emphasis on frontal lobe functions such as


inhibition, planning and thus will be more useful in picking up frontal lobe
1 deficits e.g. in FTD patients.
3022
The cut-off is education adjusted. For those with < 6 years of education,
1 mark should be added to the total. For individuals with higher
education, it is always advisable to take a detail history to compare the
current with premorbid cognitive performances based on the patients
prior professional or scholastic attainment.

Nasreddine MD
Hong Kong version 08 June 2010
Translated by Wong A and Mok V
www.mocatest.org

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2.6 Mattis Dementia Rating Scale (MDRS) Initiation/Perseveration

Mattis Dementia Rating Scale Item Task Points

Attention Complex Verbal


Naming U.S. States 20
Initiation/Perseveration
Item Task Points Simple Verbal
Naming Colors found in the room 8
Digit Span Recalling digits forward and backward 8 Initiation/Perseveration

Two Successive Commands Following two-part verbal commands 2 Consonant Perseveration Say,gee-key-bee four times. 1

Single Command Following one-part verbal commands 4 Vowel Perseveration Say,boh-bah-bee four times. 1

Imitation Imitating motoric gestures 4 Double Alternating


Palm up/palm down - five repetitions 1
Movements 1
Visual scanning and target number
Counting Distraction 1 6
identification Double Alternating
Clenched/extended - five repetitions 1
Movements 2
Visual scanning and target number
Counting Distraction 2 5
identification Alternate Tapping Tap left/tap right - 10 repetitions 1

Verbal Recognition- Reproduction ramparts - five consecutive


Reading a list of words aloud 4 Graphomotor Design 1 1
Presentation alternating

Visual Matching Identifying visual designs 4 Graphomotor Design 2 Reproduction of semi circle 1

37 (total) Graphomotor Design 3 Reproduction of cross 1


Reproduction of at least five consecutive
Graphomotor Design 4 1
alternating cross and semi circle pairs
37 (total)

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Construction Memory
Item Task Points Item Task Points
Construction Design 1 Reproduction of vertical lines 1 Orientation Orientation to time, day, date and situation 9
Construction Design 2 Reproduction of diamond in box 1 Verbal Recall - Reading Recall of sentence presented orally 4
Construction Design 3 Reproduction of square and diamond 1 Verbal Recall - Recall of sentence that was generated by the
3
Sentence Invitiation client
Construction Design 4 Reproduction of diamond 1
Verbal Recognition Verbal forced-choice recognition memory 5
Construction Design 5 Reproduction of square 1
Visual Memory Visual forced-choice recognition memory 4
Construction Design 6 Reproduction of recognizable name/signature 1
25 (total)
6 (total)

Conceptualization
Item Task Points
Identities and Oddities Identifying similar and dissimilar 16
Identifying how objects are alike; abstract
Similarities 8
concept formation
Priming Inductive
Generating similar objects 3
Reasoning
Differences Identifying dissimilar verbal objects 3
Similarities - Multiple Identifying how objects are alike in a
8
Choice multiple-choice format
Verbal Recall - Sentence
Generate sentence using man and car 1
Initiation
39 (total)

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Practical Notes on Mattis Dementia Rating Scales The Chinese version of the Mattis Dementia Rating Scale (CDRS) has been
validated by The Chinese University of Hong Kong with cut-off scores to
Dementia Rating Scale (DRS) is an instrument developed to detect and
discriminate normal from AD patients. The optimal cut-off points for CDRS
characterize cognitive impairment. It consists of 36 tasks, measuring 5
subscales and total scores with sensitivities and specificities as follows:
cognitive domains:
Attention Sensitivity/
Cut-off scores
specificity (%)
Initiation/ Perseveration
Adjusted CDRS total* 141 85.0/91.6
Construction
CDRS total ( total =144) 112 80.0/91.6
Conceptualization Attention ( total = 37) 29 22.5/98.8
Memory Initiation/Perseveration ( total =37) 26 85.0/94.0
Construction (total= 6) 3 33.0/88.0
It has been validated for staging demented patients, such that it differentiates
normal controls from mildly demented patients and the mildly from moderately Conceptualization ( total=39) 28/29 65.0/79.5
demented Alzhemiers disease (AD) patients. Memory (total = 25) 18 82.5/92.7

The pattern of scores or the subtest score in the DRS has been found to * Adjusted CDRS total is the CDRS total score adjusted for age and educational level, which is calculated by raw CDRS
total + .421(age)-1.091(education)
be useful in differentiating AD patients from patients with other types of
dementia (e.g., Parkinsons disease dementia, dementia with Lewy bodies,
Huntingtons disease, and progressive supranuclear palsy).

It takes about 20-40 minutes to administer depending on the level of the


patients impairment.

Test performance is affected by age and education. Age and education


conversion scores were obtained from the Mayo Older American Normative
Studies. The newer version (DRS-2) has expanded age and education
corrected normative data from community dwelling population but are not
to applicable to non-Caucasians and individuals with less than 8 years of
References
education. 1. Mattis S. Dementia rating scale. In Bellak L, Karasu TB (eds). Geriatric psychiatry: A handbook for psychiatrists and
primary care physicians. New York: Grune and Stratton:1976:10821.
2. Shay KA, Duke LW, Conboy T, Harrell LE, Callaway R, Folks DG. The clinical validity of the Mattis Dementia Rating
Scale in staging Alzheimers dementia. Journal of Geriatric Psychiatry and Neurology 1991;4:18-25.
3. Chan AS, Choi A, Chiu H, Lam L. Clinical validity of the Chinese version of Mattis Dementia Rating Scale in
differentiating dementia of Alzheimers type in Hong Kong. Journal of the international Neuropsyhological Society
2003;9:45-55.

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2.7 Clinical dementia rating (CDR)

Impairment

None Questionable Mild Moderate Severe


0 0.5 1 2 3

No memory loss or slight inconsistent Consistent slight forgetfulness; partial Moderate memory loss; more Severe memory loss; only Severe memory loss; only
Memory forgetfulness recollection of events; benign marked for recent events; defect highly learned material retained; fragments remain
forgetfulness interferes with everyday activities new material rapidly lost

Fully oriente Fully oriented except for slight Moderate difficulty with time Severe difficult with time Oriented to person only
difficulty with time relationships relationships; oriented for place at relationships; usually
Orientation examination; may have geographic disoriented to time, often to
disorientation elsewhere place

Solves everyday problems & handles Slight impairment in solving problems, Moderate difficulty in handling Severely impaired in handling Unable to make judgments
Judgment
business & financial affairs well; judgment similarities, and differences problems, similanities, and problems, similarities, and or solve problems
& Problem good in relation to past performance differences; social judgment usually differences; soicial juddgment
Solving maintained usually impaired

Independent function at usual level in job, Slight impairment in these activities Unable to function independently No pretense of independent function outside home
shopping, volunteer and social groups at these activities although may
Community still be engaged in some; appears Appears well enough to be Appears too ill to be taken
Affairs normal to casual inspection taken to functions outside a to functions outside a family
family home home

Life at home, hobbies, and intellectual Life at home, hobbies, and intellectual Mild but definite impairment Only simply chores preserved; No significant function in
interests well maintained interests slightly impaired of function at home; more very restricted interests, poorly home
Home and
difficultchores abandoned; more maintained
Hobbies complicated hobbies and interests
abandoned

Fully capable of self-care Needs prompting Requires assistance in Requires much help with
Personal dressing, hygiene, keeping of personal care; frequent
care personal effects incontinence

* Score in each domain are combined to obtain a global CDR score range from 0 (normal) through 3 (severe dementia), which is based on the Washington University CDR - assignment algorithm
(https://www.alz.washington.edu/cdrnacc.html).
References
1. Morris JC. The Clinical Dementia Rating (CDR) : current version and scoring rules. Neurology 1993;43(11):2412-14.

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Practical notes on the CDR (Clinical Dementia Rating scale) 2.8 Functional assessment staging (FAST)
The CDR is often used in drug trials for staging of dementia and requires Stage 1
input from both the patient and the caregiver. It is very comprehensive
and captures deficits in orientation, ADL, IADL and social domains, in No cognitive decline. No subjective complaints of memory deficit. No memory
addition to that in memory. deficit evident on clinical interviews.

A thorough CDR assessment may take up to 45 minutes, which is not


Stage 2 (Forgetfulness)
practical in most clinic situations. However, the structure and items in
the CDR serves as a very good backbone for history taking in dementia, Very mild cognitive decline.
especially for trainees and colleagues new to the field. Subjective complaints of memory deficit, most frequently in the following
Score in each domain are combined to obtain a global CDR score area:
range from 0 (normal) through 3 (severe dementia), which based on forgetting where one has placed familiar objects;
the washington University CDR-assignment aigorithm (https://www.alz.
washington.edu/cdrnacc.html). forgetting names on formerly knew well.

Free training for the CDR is available on the web at No objective evidence of memory deficit on clinical interview. No
http://alzheimer.wustl.edu/cdr/default.htm. objective deficits in employment or social situations. Appropriate
concern regarding symptoms.

Reference
1. Morris JC. The Clinical Dementia Rating (CDR) : current version and scoring rules. Neurology 1993;43(11):2412-14.

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Stage 3 (Early Confusional) Stage 4 (Late Confusional)


Mild cognitive decline. Earliest clear-cut deficits. Moderate cognitive decline. Clear-cut deficit on careful clinical interview.
Manifestations in more than one of the following areas: Deficit manifest in following areas:
A. patient may have gotten lost when traveling to an unfamiliar location; A. decreased knowledge of current and recent events;
B. co-workers become aware of patients relatively low performance; B. may exhibit some deficit in memory of ones personal history;
C. word and name finding deficit becomes evident to intimates; C. concentration deficit elicited on serial subtractions;
D. patient may read a passage of a book and retain relatively little D. decreased ability to travel, handle finances, etc.
material;
Frequently no deficit in the following areas:
E. patient may demonstrate decreased facility in remembering names
upon introduction to new people; A. orientation to time and person;
F. patient may have lost or misplaced an object of value; B. recognition of familiar persons and faces;
G. concentration deficit may be evident on clinical testing. C. ability to travel to familiar locations.
Objective evidence of memory deficit obtained only with an intensive Inability to perform complex tasks. Denial is dominant defense mechanism.
interview. Denial begins to become manifest in patient. Mild to moderate Flattening of affect and withdrawal from challenging situations occur.
anxiety accompanies symptoms.

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Stage 5 (Early Dementia) B. obsessive symptoms, e.g., person may continually repeat simple
cleaning activities;
Moderately severe cognitive decline.
C. anxiety agitation, and even previously nonexistent violent behavior may
Patient can no longer survive without some assistance. Patient is unable
occur;
during interview to recall a major relevant aspect of their current lives, e.g.,
an address or telephone number of many years, the names of close family D. cognitive abulla, i.e., loss of willpower because an individual cannot
members (such as grandchildren), the name of the high school or college carry a thought long enough to determine a purposeful course of action.
from which they graduated. Frequently some disorientation to time (date,
day of week, season, etc.) or to place. An educated person may have Stage 7 (Late Dementia)
difficulty counting back from 40 by 4s or from 20 by 2s. Persons at this stage
retain knowledge of many major facts regarding themselves and others. They Very severe cognitive decline. All verbal abilities are lost.
invariably know their own names and generally know their spouses and Frequently there is no speech at all - only grunting. Incontinent of urine,
childrens names. They require no assistance with toileting and eating, but requires assistance toileting and feeding. Lose basic psychomotor skills,
may have some difficulty choosing the proper clothing to wear. e.g., ability to walk, sitting and head control. The brain appears to no longer
be able to tell the body what to do. Generalized and cortical neurologic signs
Stage 6 (Middle Dementia) and symptoms are frequently present.

Severe cognitive decline.


May occasionally forget the name of the spouse upon whom they are
entirely dependent for survival. Will be largely unaware of all recent events
and experiences in their lives. Retain some knowledge of their past lives
but this is very sketchy. Generally unaware of their surroundings, the year,
the season, etc. May have difficulty counting from 10, both backward and
sometimes forward. Will require some assistance with activities of daily living,
e.g., may become incontinent, will require travel assistance but occasionally
will display ability to familiar locations. Diurnal rhythm frequently disturbed.
Almost always recall their own name. Frequently continue to be able to
distinguish familiar from unfamiliar persons in their environment.
Personality and emotional changes occur. These are quite variable and
include:
A. delusional behavior, e.g., patients may accuse their spouse of being an
impostor, may talk to imaginary figures in the environment, or to their
own reflection in the mirror;
References
1. Reisberg B. Functional assessment staging (FAST). Psychopharmacol Bull 1988;24(4):653-9.

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2.9 Global deterioration scale (GDS)

GDS follows the same staging system as in FAST with no sub-staging


in stage 6 and 7. Both were developed by Reisberg in the 80s when
Alzheimers Disease was at the centre stage in the field of dementia
research.
GDS and FAST
The staging system is useful in 2 aspects:
It describes longitudinally the progressive functional deterioration
in Alzheimers disease from onset to end-stage. What will happen
to me/ the patient? is a common question from the caregivers or
the patients themselves. FAST can provide information to answer
this question. This information will assist in advanced directives and
other social and financial planning.
Secondly, it helps our diagnostic accuracy. If the history of illness
shows prominent deviation from the progression as described in the
GDS/ FAST, we had better review our diagnosis. For example, the
early manifestation of motor symptoms and urinary incontinence will
make the diagnosis of AD very unlikely.
The FAST is not that difficult to remember as long as you know the child
development stages: it is fascinating that FAST is the reverse.
Remember the FAST and GDS only apply to AD and not all dementia.

Reference
1. Reisberg B, Ferria SH, de Leon MJ, Crook T. The global deterioration scale for assessment of primary degenerative
dementia. Am J Psychiatry 1982;139:1136-9.

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2.10 Geriatric depression scale (GDS)



Choose the best answer for how you have felt over the past week:
1. Are you basically satisfied with your life? YES / NO

2. Have you dropped many of your activities and interests? YES / NO 1.


2.
3. Do you feel that your life is empty? YES / NO
3.
4. Do you often get bored? YES / NO
4.
5. Are you in good spirits most of the time? YES / NO
5.
6. Are you afraid that something bad is going to happen to you? YES / NO
6.
7. Do you feel happy most of the time? YES / NO
7.
8. Do you often feel helpless? YES / NO
8.
9. Do you prefer to stay at home, rather than going out and doing new
9. /
things? YES / NO
10.
10. Do you feel you have more problems with memory than most? YES / NO
11.
11. Do you think it is wonderful to be alive now? YES / NO
12.
12. Do you feel pretty worthless the way you are now? YES / NO
13.
13. Do you feel full of energy? YES / NO
14.
14. Do you feel that your situation is hopeless? YES / NO 15.
15. Do you think that most people are better off than you are? YES / NO

*Answers in bold indicate depression. Score 1 point for each bolded answer. 2, 3, 4, 6, 8, 9, 10, 12, 14, 15
A total score of 0 to 5 is normal and a total score greater than 5 suggests depression. 1, 5, 7, 11, 13

8
Source : www.cuhk.edu.hk/med/shhcgg/healthyageing/dl/GDS.pdf

Reference Reference
1. Sheikh JI, Yesavage JA. Geriatric Depression Scale (GDS) : recent evidence and development of a shorter version. 1. Lee HCB, Chiu HFK, Kwok WY, Leung CM. Chinese Elderly and the GDS short form: a preliminary study. Clinical
Clin Gerontol 1986;5: 165-73. Gerontologist: The Journal of Aging and Mental Health 1993;14(2):37-42.

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2.11 Cornell Scale for Depression in Dementia D. CYCLIC FUNCTIONS


(CSDD)
12. DIURNAL VARIATION OF MOOD
A. MOOD RELATED SIGNS Symptoms worse in the morning

13. DIFFICULTY FALLING ASLEEP


1. ANXIENTY
Later than usual for this individual
Anxious expression, ruminations, worrying

2. SADNESS 14. MULTIPLE AWAKENINGS DURING SLEEP


Sad expression, sad voice, tearfulness
15. EARLY MORNING AWAKENINGS
3. LACK OF REACTIVITY TO PLEASANT EVENTS Earlier than usual for this individual

4. IRRITABILITY E. IDEATIONAL DISTURBANCE


Easily annoyed, short tempered
16. SUICIDE
B. BEHAVIORAL DISTURBANCE Feels life is not worth living, has suicidal wishes, or make sucicide attempt

17. SELF-DEPRECIATION
5. AGITATION
Self-blame, poor self esteem, feelings of failure
Restiessness, handwringing, hairpulling
18. PESSIMISM
6. RETARDATION
Anticipation of the worst
Slow movements, slow speech, slow reactions
19. MOOD CONGRUENT DELUSIONS
7. MULTIPLE PHYSICAL COMPLAINTS
delusions of poverty, illness, or loss
(Score 0 if Gi symptoms only)

8. LOSS OF INTEREST Each question is rated for severity with 0 point = absent, 1 points = mild or intermittent,
Less involved in usual activities (score only if change occurred acutely i.e.: in less than 1
2 points = severe, or A = unable to evaluate.
month)
Rating should be based on symptoms and signs occurring during the week prior to
C. PHYSICAL SIGNS interview.
9. APPETITE LOSS No score should be given if symptoms results from physical disability or illness.
Eating less than usual A total score >10 indicates probably major depressive episode and a total score >18
indicates definite major depression episode.
10. WEIGHT LOSS
(score 2 if greater than 5 lbs. in 1 month)

11. LACK OF ENERGY


Fatigues easily, unable to sustain activities (score only if change occurred acutely i.e.: in less
than 1 month)

Reference
1. Alexopoulos GS, Abrams RC, Young RC, Shamoian CA. Cornell Scale for Depression in Dementia. Biol Psychiatry
1988;23(3):271-84.

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Practical notes on the CSDD (Cornell Scale for Depression


in Dementia) and GDS
The CSDD is specifically designed to detect the signs and symptoms
of depression in the demented by using both patient-provided and
caregiver-provided information. It has the advantage of not relying solely
on the patients recall or opinion, which may not always be accurate in
dementia.
The CSDD is a semi-structured interview and not a questionnaire.
Therefore it also relies on observation of the patient in the clinic (e.g.
fidgeting or looking sad) rather than just the yes/no answer given.
In contrast to the GDS which is a screening test, the CSDD score do
indicate different levels of severity.
In contrast to the GDS, the CSDD also addresses biological symptoms
(e.g. appetite, sleep, diurnal mood variation, and diurnal mood variations)
and behavioural symptoms (e.g. agitation, motor retardation and multiple
somatic complaints) that could be observed by the caregiver or family
member. The GDS addresses subjective feelings which might not be
presented accurately by dementia patients.
It uses simple language such as are you feeling sad or no good,
rather than exploratory questions such as do you feel there is no hope
in your life. The latter might be too abstract for dementia patients.

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2.12 Disability Assessment for Dementia (DAD)

HYGIENE MEAL PREPARATION

Undertake to wash himself/ herself or to take a bath or a shower. Undertake to prepare a light meal or snack for himself/ herself.

Undertake to brush his/ her teeth or care for his/ her dentures. Adequately plan a light meal or snack (ingredients, cookware).

Decide to care for his/ her hair (wash and comb). Prepare or cook a light meal or a snack safely.

Prepare the water. towels, and soap for washing, taking a bath or a shower. TELEPHONING

Wash and dry completely all parts of his/ her body safely. Attempt to telephone someone at a suitable time.

Brush his/ her teeth or care his/ her dentures appropriately. Find and dial a telephone number correctly.

Care for his/ her hair (wash and comb). Carry out an appropriate telephone conversation.

DRESSING Write and convey a telephone message adequately.

Undertake to dress himself/ herself. GOING ON AN OUTING

Choose appropriate clothing (with regard to the occassion, neatness, the weather and Undertake to go out (walk, visit, shop) at an appropriate time.
color combination).
Adequately organize an outing with respect to transportation, keys, destination,
Dress himself/ herself in the appropriate order (undergarments, pants/ dress, shoes). weather, necessary money, shopping list.

Dress himself/ herself completely. Go out and reach a familiar destination without getting lost.

Undress himself/ herself completely. Safely take the adequate mode of transportation (car, bus, taxi).

CONTINENCE Return from the store with the appropriate items.

Decide to use the toilet at appropriate times FINANCE AND CORRESPONDENCE

Use the toilet without accidents Show an interest in his/ her personal affairs such as his/ her finances and written
correspondence.
EATING
Organize his/ her finance to pay his/ her bills (cheques, bankbook, bills).
Decide that he/ she needs to eat.
Adequately organize his/ her corresspondence with respect to stationery, address,
Choose appropriate utensils and seasonings when eating. stamps.

Eat his/ her meals at a normal pace and with appropriate manners. Handle adequately his/ her money (make change).

134 135
SECTION 2
Assessment scales in neurocognitive disorders

Activities of Daily Living (ADL) and Instrumental Activities of


Daily Living (IADL) assessment in Dementia
MEDICATIONS The Disability Assessment for Dementia (DAD), the Barthel Index (BI) and the
Decide to take his/ her medications at the correct time. Katz Index of Independence of ADLs scales
In usual clinic settings a scale for evaluation of ADLs (activites of daily
Take his/ her medications as prescribed (according to the right dosage).
living e.g. transfer, toileting, bathing, dressing, eating, continence, etc)
LEISURE AND HOUSEWORK or IADLs (instrumental activities of daily living e.g. cooking, shopping,
telephoning, going out, etc.) is not an absolute necessity.
Show an interest in leisure activity(ies)
There is a tendency to focus on cognitive status (e.g. MMSE) for
Take an interest in household chores that he/ she used to perform in the past.
treatment/ disease monitoring in dementia. However, functional
Plan and organize adequately household chores that he/ she used to perform in the limitations should never be left out, because they are often more related
past. to caregiver burden. For example, a patient with low MMSE but can
Complete household chores adequately that he/ she used to perform in the past. bathe and do toileting herself is much easier to take care of than another
who has better memory but cannot do any of these without assistance
Stay safely at home by himself/ herself when needed. or prompting.
The indication of most if not all current dementia drugs are to delay
Each item can be scored: 1 point = Yes, 0 point = No, or N/A = non-applicable.
cognitive decline and maintain ADL independence. Knowing which
A total score is obtained by adding the score for each question and converting into a
ADL items to observe and how to monitor changes in these ADLs will
percentage. For example, a score of 33 out of 40 (maximum score) is equivalent to 83%
and a score of 33 out of 38 (exclude 2 items that indicates N/A) is equivalent to 87%. be important for treatment monitoring and communicating with the
Higher scores represent less disability in activities of daily living while lower scores caregiver.
indicate more dysfunction These ADL scales or questionnaires provide an idea of what ADL
questions to ask caregivers, and the DAD especially provides us with
a structured set of questions which we can go through to monitor
treatment effect, decline or deficits.
The basic difference between these 3 scales:
The DAD was designed to assess the disability in BOTH activities
of ADLs and IADLs in dementia, whereas both the BI and the Katz
were designed to measure basic ADLs only, and are not specific for
dementia.

Reference
The BI measures independency in 10 basic ADLs, and the Katz
1. Gelinas I, Gauthier L, McIntyre M, Gauthier S. Development of a functional measure for persons with Alzheimers measures independency in 6 basic ADLs.
disease: the disability assessment for dementia. Am J Occup Ther 1999;53:471-81.

136 137
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Assessment scales in neurocognitive disorders

The BI has a score ranging from 1-100, hence is more sensitive to 2.13 Neuropsychiatric inventory (NPI)
changes and is used more often in rehabilitation settings. The Katz
The NPI is one of the most frequently used tools in assessing behavioural
ranges only from 0-6, is less sensitive to changes, but is very simple
psychcological symptoms of dementia (BPSD). It addresses 12 symptoms
to use, and therefore more often used to assess stable nursing home
which varied from psychotic symptoms (hallucinations, delusions), mood
residents.
(anxiety, agitation/ aggression, depression, euphoria, apathy), to behavioural
The DAD measures not only whether the patient is able to perform an symptoms (irritability, disinhibition, aberrant motor behaviour, sleep and
activity, but also his/ her initiative to start an activity, without being nighttime behaviour, and eating problems).
prompted. For example, the DAD asks whether the patient decides to
The full NPI takes about 30 - 45 minutes to complete and is often used in
take a bath or wash her hair on her own, while the BI or Katz only ask
drug studies. In clinical practices however there is often not enough time for
whether she can bathe herself with or without assistance. The lack of
this. Neverthelss, the list of BPSDs in the NPI are very useful reminders for
initiation is a hallmark of dementia. In someone with dementia, being
clinicians during history taking in dementia care. As BPSDs are often highly
able to decide to take a bath without being reminded to is very different
related to caregiver stress, noting the type, severity and frequency of BPSDs,
from doing so only with prompting or coercing.
and the follow-up of their progress after treatment is important in relieving
The DAD addresses other problems common in dementia, e.g. caregiver burden, allowing the patient to be cared at home longer, or causing
judgement. It asks whether the patient can choose appropriate clothing less conflicts in institutions.
for the occasion or weather, in addition to being able to dress properly.
The BI and Katz only assess whether the patient can dress herself or
not. Judgment is an important area in dementia only addressed by the
DAD.
The DAD, being a scale for dementia patients, also addresses memory.
For example, it asks whether the patient can take down or convey
a telephone message. Memory is not assessed by the BI or Katz. In
fact none of the basic ADLs assessed by the BI or Katz required much
memory.

Reference
1. Cummings JL. The Neuropsychiatric Inventory: assessing psychopathology in dementia patients. Neurology
1997;48(Suppl 6): S10-6.

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Assessment scales in neurocognitive disorders

2.14 Zarit Burden Interview (ZBI) Practical notes on Zarit Burden Interview
Chinese Version of the Zarit Burden Interview The Zarit Burden Interview (ZBI) is a popular caregiver self-report measure
for use in clinical or community settings. It has been specially designed to
reflect the stresses experienced by the caregivers of demented patients.

It originated as a 29-item questionnaire but the revised version containing

22-item is more commonly used. This instrument covers areas of concern
1.
mostly mentioned by caregivers, including caregivers health, psychological
4 3 2 1 0
well-being, social life, finance, and the relationship between the caregiver
2. 4 3 2 1 0
and patient. Each item is a question which the caregiver is asked to endorse
3.
4 3 2 1 0 using a 5-point scale. Response options range from 0 (never) to 4 (nearly

always). It is scored by adding the numbered response of the individual
4. 4 3 2 1 0
items. The total score of the 22 items ranges from 0 to 88 with higher scores
5. 4 3 2 1 0
representing a higher level of caregiver burden. It is suggested that a score
6. 4 3 2 1 0 below 21 indicates little or no burden, 21-40 for mild to moderated burden,
7. 4 3 2 1 0 41-60 for moderate to severe burden, and 61-88 for severe burden. The
8. 4 3 2 1 0 Chinese version of ZBI was validated in Hong Kong in 2005. Overall, ZBI has
9. 4 3 2 1 0
been shown to be a valid and reliable instrument for measuring the burden
10.
of caregiver in studies.
4 3 2 1 0
11. 4 3 2 1 0
12. 4 3 2 1 0
13. 4 3 2 1 0
14.
4 3 2 1 0

15. 4 3 2 1 0
16. 4 3 2 1 0
17. 4 3 2 1 0
18. 4 3 2 1 0
19. . 4 3 2 1 0
20. 4 3 2 1 0 References
1. Zarit SH, Reever KE, Bach-Peterson J. Relatives of the impaired elderly: correlated of feelings of burden. Gerontologist
21. 4 3 2 1 0 1980;20(6):649-55.
22. 4 3 2 1 0 2. Rankin ED, Haut MW, Keefover RW, Franzen MD. The establishment of clinical cutoffs in measuring caregiver burden
in dementia. Gerontologist 1994;34(6):828-32.
/ 88 3. Chan TSF; Lam LCM; Chiu HFK (2005). Validation of the Chinese Version of the Zarit Burden Interview. Hong Kong
Journal Psychiatry 2005;15(1):9-13.

140 141
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Investigation

3.1 Neuroimaging
Indications for Neuroimaging in Dementia
When clinical diagnosis is in doubt, e.g., Alzheimers disease vs dementia
with Lewy bodies, depression vs pseudo-dementia
When organic disease is suspected, e.g., recent onset of signs and
symptoms, history of head injury, acute delirium
When result of neuroimaging affects clinical management

SECTION 3
Investigation

143
SECTION 3
Investigation

Baseline Investigation
Brain region Significance
CT brain (non-contrast) is enough to exclude most of the organic causes,
e.g., small vessel disease, hydrocephalus, infarctions and hemorrhage, Frontal lobes atrophy FTLD, FTD, non-specific atrophy
subdural collection and brain tumour AD (symmetrical),
Temporal lobes atrophy
Coronal and sagittal images should be requested to adequately assess FTD, PPA, CBD (asymmetrical)
pattern of atrophy, e.g. coronal images for hippocampus, sagittal images AD (symmetrical)
for brainstem Hippocampal atrophy
usually not significant in early FTD
MRI brain (non-contrast) is better than CT brain in terms of lesion AD and non-specific age related atrophy
Parietal lobes atrophy
characterization, and MRI is automatically performed in multi-planar less prominent in FTD
format.
Usually not prominent unless in moderate
Serial comparison is essential to find out the dominant lobar atrophy Occipital lobes atrophy or severe stage of dementia, if occurs in
in various dementias or minimal progression in age-related cerebral early stage, DLB is suspected
atrophy
Brainstem atrophy PSP, MSA

Cerebellum Alcoholism, MSA-C

Periventricular regions Small vessel disease (SVD) vs NPH

Corpus callosum
PPA, CBD
(Normal 5mm thick at post. Genus)

Basal ganglia More than 5 infarcts in SVD

Abbrevations : FTLD, frontotemporal lobar degeneration; FTD, frontotemporal dementia;


AD, Alzheimers disease; PPA, primary progressive aphasia; CBD, corticobasal
degeneration; DLB, dementia with Lewy bodies; PSP, progressive supranuclear palsy;
MSA, multiple system atrophy; MSA-C, MSA with cerebellar features; SVD, small vessel
disease; NPH, normal pressure hydrocephalus.

144 145
SECTION 3
Investigation

Specific Dementia Syndromes Corticobasal degeneration (CBD)


Alzheimer Disease (AD) Asymmetry is the rule, cortical atrophy contralateral to the side of limb
dyspraxia
Hippocampal atrophy is more prominent in early AD, and severity
compatible with temporal atrophy in all stages of AD Atrophy starts at peri-central sulcus and spread downwards to peri-
Sylvian fissure in later stage
(Fronto-) Parietal atrophy may also be present
may associate with corpus callosum atrophy
Symmetry is the rule. When asymmetrical, consider atypical AD such
as posterior cortical atrophy and frontal AD, further imaging such as brainstem involvement usually in late stage
perfusion SPECT is helpful perfusion SPECT shows characteristic corticobasal hypo-perfusion

Frontotemporal Dementia (FTD) Progressive Supranulcear Palsy (PSP)


Hippocampal atrophy is not dominant in early to moderate FTD when Asymmetry is the rule
compared with dominant temporal lobe atrophy, but eventually will
become significant in moderate to severe stage of FTD brainstem involvement usually in earlier stage than in CBD and PPA,
most prominent at pons with widening of pre-pontine space and slender
Frontal or temporal variant of FTD may show dominant lobar atrophy anterior border of pons
respectively
may associate with frontal and corpus callosum atrophy
Usually asymmetrical atrophy can be used to differentiate FTD from AD
cerebellar atrophy not prominent
Primary Progressive Aphasia definitive diagnosis may requires SPECT or PET showing disproportional
Asymmetry is the rule, usually on left side (dominant hemisphere) for brainstem hypoactivity
speech
Multiple System Atrophy (MSA)
Atrophy starts at peri-Sylvian fissure and spread upwards to peri-central
sulcus in later stages (PPA, CBD and FTD as 3-in-1 syndrome) Progressive atrophy of brainstem and cerebellum, especially prominent
at midbrain
Usually associate with corpus callosum atrophy
MRI shows characteristic Hot cross bun sign and Humming bird sign
Further sub-typing may require perfusion/metabolism brain scan in midbrain atrophy
(HMPAO or ECD SPECT and FDG PET)
Supratentorial atrophy not compatibly progressive as brainstem atrophy
MR tractography showing respective fascicular atrophy in subtypes (for on serial scans
research purpose)

146 147
SECTION 3
Investigation

Dementia of Lewy Bodies (DLB) Depression with Pseudo-dementia


Atrophy pattern usually not characteristic on imaging, can be similar to Usually no obvious atrophic pattern in early stage
AD or age- related cerebral atrophy
Usually normal MR spectroscopy with borderline low NAA while
MR spectroscopy shows markedly depressed NAA but normal unremarkable myoinositol level
myoinositol level
Usually non-specific perfusion abnormality on brain SPECT
Definitive diagnosis may require perfusion SPECT or FDG PET with
predominant patieto-occipital hypo-activity
Frontal lobe involved in advanced stage of DLB

Vascular Dementia (VD)


Usually caused by small vessel disease (SVD) with diffuse confluent
periventricular hypodensities or hyperintensities on T2WI and FLAIR MRI
Lacunar or sizable infarcts are common
May associate with global cerebral atrophy
Ventriculomegaly proportional to cerebral atrophy
Cerebral Amyloid Angiopathy shown on Susceptibility Weighted Imaging
(SWI)

Normal Pressure Hydrocephalus (NPH)


Ventriculomegaly disproportional to cerebral atrophy
Dominant periventricular hypodensities particularly around the horns of
lateral ventricles
More suspicious if infarct is absent
Need to have compatible clinical signs and symptoms before Indium-
DTPA cisternography is requested for characteristic ventricular reflux
activity at 4 hour and disproportionally higher activity in ventricles than in
cerebral sulci at 24-48 delay scan on SPECT/CT

148 149
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Investigation

Additional Neuroimaging Cisternography


MR Spectroscopy (MRS) Intrathecal injection of Indium-DTPA, requires in-patient care for post-
injection period and delay scanning
Recommend 1.5T MRS with 2 x 2 x 2 cm3 voxel on posterior cingulate
gyrus processed by LCModel (Version 6.3-1B) Indicated for clinically compatible Normal Pressure Hydrocephalus (NPH)
To differentiate pseudo-dementia from dementia Equivocal result may need CSF tapping or infusion test to exclude NPH
To differentiate age-related cerebral atrophy from pathological MCI with
high risk of progression to dementia in subsequent 2 years (moderate to
Amyloid PET
severe low NAA and raised MI) Pattern of amyloid deposit is more specific than amount of amyloid
loading
Combine with atrophy pattern, MRS may differentiate FTD (mildly raised
MI in FTD) from AD (moderate raised MI in AD) Only able to confirm or exclude AD, sometimes DLB, but not others

MR Tractography Dopaminergic Imaging


Dedicated processing of diffusion tensor imaging (DTI) to draw the fiber DaTscan and CIT scan and Dopa PET scan are the most commonly used
tracts
Useful in differentiating AD (normal) from DLB (decrease uptake) when
For research purpose in sub-classification of PPA clinically difficult
Therapeutic implication for dopamine therapy in Parkinson disease,
Brain Perfusion SPECT (Single Photon Computerized Tomography) atypical parkinsonism or DLB
Use either HMPAO or ECD as brain perfusion agent Not helpful in differentiating between Parkinsons disease and atypical
Radiation dose similar to a bone scan which is acceptable in elderly parkinsonism
(> 60y)
Talairach analysis is preferred with a 3D brain perfusion map display
References
Supplementary to conventional imaging if diagnosis in doubt after CT or 1. Kantarci K, Petersen RC, Boeve BF, Knopman DS, Tang-Wai DF, OBrien, et al. 1H MR spectroscopy in common
MRI dementias. Neurology 2004;63(8):1393-8.
2. Galantucci S, Tartaglia MC, Wilson SM, Henry ML, Filippi M, Agosta F, et al. White matter damage in primary
progressive aphasias: a diffusion tensor tractography study. Brain 2011;134(10):3011-29.
FDG Positron Emission Tomography (PET) 3. Warren JD, Fletcher PD, Golden HL. The paradox of syndromic diversity in Alzheimer disease. Nature Reviews
Neurology 2012;8(8):451-64.
Better resolution to SPECT especially at brainstem and basal ganglia 4. Tokumaru AM, Saito Y, Murayama S, Kaztomi K, Sakiyama Y, Toyoda M, et al. Imaging-pathologic correlation in
corticobasal degeneration. AJNR 2009;30:1884-92.
Radiation dose to brain is high, not considered in young patients 5. Koyama M, Yagishita A, Nakata Y, Hayashi M, Bandoh M, Mizutani T. Imaging of corticobasal degeneration syndrome.
Neuroradiology 2007;49:905-12.
Concomitant CT brain performed 6. Cummings JL, Henchcliffe C, Schaier S, Simuni T, Waxman A, Kemp P. The role of dopaminergic imaging in patients
with symptoms of dopaminergic system neurodegeneration. Brain 2011;134(11):3146-66.

150 151
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Investigation

3.2 Biomarkers Changes of CSF Biomarkers in Alzheimers disease


Biomarkers can help diagnosis, monitoring the disease progression, Reduced A42
detecting early onset of the disease, monitoring the effect of therapeutic Increased total tau
intervention, and also avoiding false diagnosis of the disease.
Increased phosphorylated tau
Pathophysiology of Alzheimers disease (AD):
The major neuropathological hallmarks of AD are the presence of It is essential that the CSF assay is standardized in diagnostic procedures,
extracellular amyloid plaques that are aggregates of amyloid beta (A) (A40 sample processing, and testing. As a result, the diagnostic conclusions
and A42), and intracellular neurofibrillary tangles (NFT) which is composed drawn from these results are therefore comparable.
of hyperphosphorylated tau protein. Neuroimaging techniques provide
information about the structure and physiology of brain while the cerebrospinal
fluid (CSF) biomarkers represent the most direct and convenient means as
it is in direct contact with the extracellular space of the brain and can reflect
biochemical changes that occur inside the brain.

Category Markers Advantages Limitations

Imaging FDG-PET, 1. Non-invasive 1. Expensive


PIB-PET, MRI 2. Provides structural 2. Requires expertise
and functional details 3. The sensitivity and
of brain immediately specificity to AD is
3. Can detect AD not satisfactory
progression

CSF A42, t-tau, 1. Can correlate AD 1. Invasive


p-tau, directly 2. Irreproducible
2. Highly sensitive and diagnosis due to
specific sample storage and
3. Can detect AD transportation
progression

Abbrevations: FDG-PET, 18-fluorodeoxyglucose positron emission tomography; PIB-


PET, Pittsburgh Compound-B (2-[4-(methylamino)phenyl]-6-hydroxybenzothiazole)
positron emission tomography; A, amyloid beta; t-tau, total tau; p-tau, phosphorylated
tau.

152 153
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Investigation

Use of CSF Biomarkers in B. Mild Cognitive Impairment (MCI) - Mainly for research purpose
A. AD dementia - To improve the diagnostic accuracy. 1. To establish the underlying aetiology for recruiting patients for new drug
trial
The accuracy of clinical diagnostic criteria for AD dementia is poor, such that
the clinical diagnosis is correct only 63% to 90% of the time. The accuracy is 2. To determine the likelihood of progression to AD dementia within a
even lower in early stages of the disease. The development and application of defined period
revised diagnostic criteria that include biomarkers will substantially improve
the diagnostic accuracy for AD dementia and other dementias. MCI criteria incorporate biomarkers to suggest the probability of AD aetiology

Clinical Category Biomarker A Neuronal Injury


AD dementia criteria incorporate biomarkers to suggest the probability of AD aetiology
probability of AD (PET or CSF) (CSF tau, FDG-
Clinical Biomarker A Neuronal Injury aetiology PET, structural
Category probability of AD (PET or CSF) (CSF tau, FDG-PET, MRI)
aetiology structural MRI) MCI - core clinical Uninformative Unavailable, Unavailable,
Probable AD High Positive Positive criteria conflicting or conflicting or
indeterminate indeterminate
Intermediate Positive Unavailable,
conflicting or MCI - due to AD Intermediate Positive Untested
indeterminate Untested Positive
Intermediate Unavailable, Positive High Positive Positive
conflicting or
indeterminate MCI - unlikely due Lowest Negative Negative
to AD
Uninformative Unavailable, Unavailable,
conflicting or conflicting or
Adapted from reference 3.
indeterminate indeterminate
Possible AD High but does not Positive Positive
rule of second
etiology
Dementia unlikely Lowest Negative Negative References
due to AD 1. Anoop A, Singh PK, Jacob RS, Maji SK. CSF biomarkers for Alzheimers disease diagnosis. International Journal of
Alzheimers Disease 2010; Article ID 606802, 12 pages. doi:10.4061/2010/606802.
Adapted from reference 2. 2. McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH, et al. The diagnosis of dementia due
to Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers Association workgroups
on diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011:7:263-9.
3. Albert MS, DeKosky ST, Dickson D, Dubois B, Feldman HH, Fox NC, et al. The diagnosis of mild cognitive impairment
due to Alzheimers disease: recommendations from the National Institute on Aging-Alzheimers. Association
workgroups on diagnostic guidelines for Alzheimers disease. Alzheimers Dement 2011;7: 2709.
4. Vanderstiichele H, Bibi M, Engelborghs S, Le Bastard N, Lewczuk P, Molinuevo JL. Standardization of preanalytical
aspects of cerebrospinal fluid biomarker testing for Alzheimers disease diagnosis: a consensus paper from the
Alzheimers Biomarkers Standardization Initiative. Alzheimers Dement 2012;8:6573.

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Management

4.1 Non-pharmacological management of


behavioural and psychological symptoms of
dementia (BPSD)
The International Psychogeriatric Association (IPA) Consensus Conference
in 1999 produced a statement on the definition of BPSD which refers to
the non-cognitive symptoms of disturbed perception, thought content,
mood or behaviour that frequently occur in patients with dementia. Those
symptoms are called neuropsychiatric symptoms which include behavioral
and psychological aspects:
a. Specific behavioural symptoms: Usually identified on the basis of

SECTION 4
observation of the person with dementia, e.g., wandering, agitation,
disinhibition, catastrophic reactions, complaining and negativism.
b. Specific psychological symptoms: Usually and mainly assessed
Management through interviews with persons with dementia and their relatives,
e.g., delusion, hallucination, misidentification, depression, apathy
and anxiety

Assessment / Intervention:
In the third edition of the Occupational Therapy Clinical Guideline for People
with Dementia (2011), some assessment tools for BPSD are recommended:
1. Chinese version of the Cohen-Mansfield Agitation Inventory (CMAI)
The CMAI was developed for use in assessing different agitated
behaviours in individuals with dementia in institutions or in the community.
The agitated behaviours include wandering, aggression, inappropriate
vocalization, sexual disinhibition and negativism. It takes 10-15 minutes
to administer.
2. Chinese version of the Neuropsychiatry Inventory (CNPI)
The CNPI is a brief interview assessing 10 domains: delusions,
hallucinations, dysphonia, anxiety, agitation/aggression, euphoria,
disinhibition, irritability/liability, apathy, and aberrant motor behaviour.
It may help to distinguish among different causes of dementia, record
severity and frequency separately. It takes 10 -15 minutes to administer.
157
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Management

3. Clifton Assessment Procedures of the Elderly (CAPE) Apart from the formal assessment tools, the TECH approach developed by
Sharon Kratiuk-wall (1996) is also recommended. It provides a systematic
The CAPE is used to evaluate the presence and severity of impairment
and structured way to determine the triggering factors of BPSD by asking 4
in mental and behavioural functioning. It comprises of two components:
key questions. Those questions are broken down into four headings: Task,
the Cognitive Assessment Scale (CAS) and the Behaviour Rating Scale
Environment, Communication and Health status, as follows:
(BRS). The CAS is consisted of 12 questions covering information/
orientation subtests. The BRS covers physical disability, apathy, Task Environmental Communication Health Status
communication difficulties and social disturbance. Higher scores
indicate a greater severity of behaviour problems. It takes 15-30 minutes Too many Provide safety Is there a need to: Is the behavior
to administer. choices? (limit and security? related to
ability to process (Physical and Focus on
information) emotional) the feelings Cognitive
underlying a deficit?
Task too Too much / too persons words?
complex? little stimulation? Aging e.g.
Reduce hearing,
Too many steps? Too much / too complexity of the sight, change
little space? communication? of sleeping
Difficulty in pattern?
recognition or Appear Place emphasis
familiarity? (e.g. unfamiliar? on non-verbal Medication?
object) communication?
Appear Fatigue?
Is the task reminiscent of Be calmer,
reminiscent of past unpleasant quieter, less Hunger or thirst?
past pleasant experience? demanding Constipation?
/ unpleasant
experiences? Optimum for Minimize Pain or
function? e.g. distraction? discomfort?
temperature,
lighting, seating
etc.
Structure
to allow
participation?
Provide cues for
activity?

Under each key question, not only the possible causes considered to be
triggers to the persons behaviour are analyzed, but a framework to tackle
the BPSD is also provided.
For both professional group therapy and individual therapy, it is generally
agreed that non-pharmacological interventions (NPIs) should be considered
158 159
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Management

as the first-line management of most BPSD. Although a number of NPIs Montessori materials consist of learning games suited to a patients abilities
(see table) are now available for persons with dementia, it should be noted and interests, examples are memory games, household utensils, books
that there are several areas of overlap between these therapies and, in and so on. The aim of the Montessori Method for dementia is to provide
fact, each approach is rarely used in isolation. Client-centered and tailored opportunities for the patient to enjoy life by engaging in daily roles, routines
therapy guided by the patients background, interests, cultural, religious and and activities of daily living that are created and presented based on his/her
life factors to the person with dementia are the most important concept in needs, strengths, skills, abilities and interests, and delivered in a supportive
treating ones BPSDs. environment purposefully and meaningfully. The rationale is that undesirable
behaviour would be significantly decreased when the patients unmeet needs
Categories for Non-Pharmacological Interventions for BPSD are met.
National and international guidelines often recommend NPIs as the first
Sensory Enhancement / Cognitive / emotion- line of treatment for BPSD. However, there is evidence that antipsychotic
Behavior Therapy
Relaxation oriented interventions
medications can also be used effectively when the BPSD poses significant
e.g. Massage and e.g. Reminiscence e.g. Differential risks of harm to the patient or others, or when the aggressive symptoms
touch Therapy reinforcement are persistent, recurrent or severe enough to cause significant suffering and
Music therapy Validation Therapy Stimulus control distress.
Snoezelen Habit training
Multisensory
Art therapy
Aroma therapy

Client-centered Training and


Structured Activities
approach Development
References

e.g. Recreational e.g. Montessori e.g. Staff education 1. Lebowitz BD. Behavioral and Psychological Symptoms of Dementia: A Clinical and Research Update. International
Psychogeriatrics 2000;12(S1):19-21.
activities Methods for Staff support 2. Occupational Therapy Clinical Guideline for People with Dementia (Third Edition, April 2011), Working Group on
Outdoor walks dementia (MMD)TM
Training programs Psychogeriatrics of Coordinating Committee in Occupational Therapy, Hong Kong Hospital Authority.
Physical activities for family 3. Choy CNP, Lam LCW, Chan WC, Li SW, Chiu HFK. Agitation in Chinese elderly: validation of the Chinese version of
caregivers the Cohen-Mansfield Agitation Inventory. International Psychogeriatrics 2001;13(3):325-35.
4. Leung VPY, Lam LC, Chiu HF, Cummins JL, Chen QL. Validation study of the Chinese version of the neuropsychiatry
inventory (CNPI). International Journal of Geriatric Psychiatry 2001;16(8):789-93.
Environmental Social contact:
Cognitive therapy 5. Pattie AH, Gilleard CJ. Clifton assessment procedure for the elderly manual. Windsor: NFER Nelson, 1979.
Modifications Real or Simulated
6. Krattiuk-Wall S, Quirke S, Heal C, Shanley C. The TECH Approach to Dementia Care: a resource kit for people with
challenging behaviours in a residential care setting: task, environment, communication, health. Concord, N.S.W.:
e.g. Reality orientation e.g. Wandering areas e.g. Individual social Centre for Education and Research on Aging; 1996.
Natural / enhanced contact
7. British Columbia Minstry of Health. Best Practice Guideline for Accommodating and Managing Behavioral and
environments Pet therapy Psychological Symptoms of Dementia in Residential Care. 2011.
Reduced Simulated 8. Ballard CG, OBrien J, James I, Swann A. Dementia: management of behavioural and psychological symptoms.
Nordic Journal of Psychiatry 2003;57(2): 159-60.
stimulation interactions /
family videos 9. Lin LC, Yang MH, Kao CC, Wu SC, Tang SH, Lin JG. Using Acupressure and Montessori-based activities to decrease
Light therapy agitation for residents with dementia: a cross-over trial. Journal of the American Geriatrics Society 2009;57(6):1022-
29.

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Management

4.2 Treatment guideline and practical tips in using Rivastigmine (Exelon, Exelon Patch)
cholinesterase inhibitors and NMDA antagonist Oral rivastigmine is indicated for the treatment of mild to moderate dementia
Although patients and caregivers have high expectations of drug treatment of the Alzheimer type. Initial oral dosing recommendation is 1.5 mg twice a
for dementia, clinicians should always emphasize non-pharmacological day, with a maximum dose of 6mg twice a day (12mg/day). Rivastigmine is
measures to enhance the coping skills for patients and caregivers at all times a potent, selective inhibitor of brain AChE and butylcholinesterase (BuChE).
and through all stages of the disease. Rivastigmine is considered a pseudo-irreversible inhibitor of AChE. While
the precise mechanism of rivastigmines action is unknown, it is postulated
Cognitive enhancers on mild cognitive impairment (MCI) to exert its therapeutic effect by enhancing cholinergic function. This is
accomplished by increasing the concentration of acetylcholine through
It is not uncommon that physicians are asked by the families of MCI patients reversible inhibition of its hydrolysis by cholinesterase. The transdermal
to start these drugs, even though their safety and efficacy in this condition patches 4.6 mg/24 hours and 9.5 mg/ 24 hours are approved for mild to
is uncertain. A systemic review of randomized, placebo-controlled trials moderate AD patients. It has the advantage of causing less gastrointestinal
compared cholinesterase inhibitor or memantine in MCI using outcome side effect, better 24 hours drug profile and is easier to administer to patients.
measurements of cognition, function, behaviour, global status and mortality, Yet, mild dermatitis is not uncommon at the application sites of the patches.
found no significant effects of either on cognition or function. On the contrary, A reliable caregiver has to be committed to remove the old patch before a
higher rates of nausea, diarrhea, vomiting, and headache were observed in new patch is applied every 24 hours.
the treatment group compared with the placebo group.
Galantamine (Reminyl PRC)
Cognitive enhancers on Alzheimers disease dementia
Galantamine is indicated for the treatment of mild to moderate dementia
Cholinesterase inhibitors (ChEIs) are used to enhance the cholinergic function of the Alzheimer type. It enhances central cholinergic function by inhibiting
in Alzheimers disease (AD) patients. ChEIs (donepezil, rivastigmine, and AChE. The dosing recommendation for the immediate-release formulation
galantamine) inhibit acetylcholinesterase (AChE) (specific cholinesterase) at is 4 mg twice daily. The extended-release formulation is given at a dose of
the synapse 8 mg orally once daily. The maintenance dose after dose titration is 16-24
mg/day.
Donepezil (Aricept, Aricept Evess)
Donepezil has shown efficacy in patients with mild to moderate AD, as well Memantine (Ebixa)
as moderate to severe AD. It selectively inhibits AChE and improves the Memantine is the only drug on the market targeted for N -methyl-D-
availability of acetylcholine. Donepezils long half-life provides a long duration aspartate (NMDA) antagonism and has been approved by the US Food and
of drug availability for binding at the receptor sites. Dosing recommendations Drug Administration (FDA) for patients with moderate to severe AD dementia.
for mild to moderate AD are 5-10 mg orally once daily. Aricept Evess is Memantine is also recommended as an option for managing people with
an oral dispersible formula which is easier to administer to patients with moderate AD dementia who are intolerant of or have a contraindication to
swallowing problem. AChE inhibitors. The initial dose for the immediate-release formulation is 5
mg orally once daily, and it can be titrated to a maximum dose of 20 mg/day.

162 163
SECTION 4
Management

Drugs other than cognitive enhancers for Alzheimers disease of the Alzheimer Type) study, a randomized, double-blind trial comparing
dementia the 13.3 mg/24 hours dose patch with the lower 4.6 mg/24 hours dose.
The higher dose patch demonstrated statistically significant improvement in
AD patients who suffer from behavioural and psychological symptoms of
overall cognition and function in severe AD patients at week 24, as assessed
dementia (BPSD) and who fail non-drug treatment may need psychotropic
by measures of cognition and daily function using the Severe Impairment
medications. The BPSD of AD dementia including depression, agitation,
Battery (SIB) and the Alzheimers Disease Cooperative StudyActivities of
aggression, hallucinations, delusions, and sleep disorders, are frequently
Daily LivingSevere Impairment Version (ADCS-ADL-SIV), respectively.
treated with antidepressants, anxiolytics, antiepileptic drugs as mood
stabilizers, and neuroleptics.
In a recent study of once-daily donepezil 23 mg involving 1,467 randomized
When prescribing these drugs, clinicians should assess the individuals patients with moderate to severe AD dementia, a significantly greater
clinical profile and balance the possible benefits against risks of potential cognitive benefit compared with patients taking donepezil 10 mg/day
side effects. Most importantly, a reliable caregiver should be sought to was demonstrated on the SIB after 6 months of treatment. In this study,
monitor the symptoms and facilitate compliance to treatment as necessary. co-primary effectiveness measures were changes in cognition and global
functioning, as assessed using least squares mean changes from baseline
High dose cognitive enhancers for moderate to severe AD on the Severe Impairment Battery (SIB; cognition) and the Clinicians
dementia Interview-Based Impression of Change Plus Caregiver Input scale (CIBIC+;
global function rating) overall change score at week 24. Donepezil 23
In 2012, the US FDA approved a 13.3 mg/24 hours dosage strength of
mg/day was associated with greater benefits in cognition compared with
the rivastigmine transdermal system for patients with mild to moderate AD
donepezil 10 mg/day. Patients with more advanced AD appeared to benefit
dementia who are experiencing a decline in overall function and cognition.
from donepezil 23 mg/day on assessment of global functioning.
The approval of the higher dosage 13.3 mg/24 hours patch was based
on the findings from the 48-week double-blind OPTIMA study. Patients
However, clinicians should be vigilant of side effects in using high dose CEI.
treated with the 13.3 mg/24 hours dose experienced significantly improved
In particular, low body weight patients are commonly intolerant to high dose
overall function compared with those who received the 9.5 mg/24 hour
therapy. Slow and cautious upward titration to the conventional maximum
patch (P < .05), as measured by the Alzheimers Disease Cooperative Study
dose is recommended.
Instrumental Activities of Daily Living (ADCS-IADL) scale at week 48. In the
OPTIMA study , there was also improvement in cognition as measured by
the Alzheimers Disease Assessment Scale-Cognitive (ADAS-Cog) which
was significant at 24 weeks but not at 48 weeks.

Recently, the US FDA also approved an expanded indication for the


rivastigmine transdermal system to include patients with severe disease,
making it the only transdermal therapy that can be used across all stages
of disease. The approval in severe AD dementia was based on the ACTION
(ACTivities of Daily Living and CognitION in Patients with Severe Dementia

164 165
SECTION 4
Management

Practical tips Memantine is recommended as an option for managing people with


moderate AD dementia who are intolerant of or have a contraindication to
Before starting drug therapy, it is always advisable to manage the expectations
AChE inhibitors, or severe Alzheimers disease. The common undesirable
of patient and family by clarifying that it is only a symptomatic treatment and
effects of memantine are dizziness, headache, constipation, somnolence
may retard the disease progression, but is not a cure.
and hypertension. Special precaution should be noted when prescribing this
drug to patients with severe renal failure and patients with epilepsy.
Whenever treatment is initiated, carers views on the patients condition at
baseline should be sought. Not all patients are responders and treatment may
have to be stopped. Treatment should be continued only when it provides a
worthwhile effect on cognitive, global, functional or behavioural symptoms.
Patients on treatment should be reviewed regularly using appropriate
cognitive, global, functional, and behavioural assessment tools.

Initiate treatment at a low dose and titrate upwards within the first 3 months
to the optimal dose. The latter is usually lower than the recommended
dose based on trials done in western populations. If one drug is ineffective,
some clinicians will switch to another class or another drug of the same
class. Despite not being evidence-based, it appears to be a fair approach.
Combination therapy (ChEI together with memantine) is used by some
clinicians but this has not been extensively investigated in clinical trials.

The three AChE inhibitors, donepezil, galantamine, and rivastigmine, are


recommended as options for managing mild to moderate AD dementia. The
family and patient should be warned about GI upset such as anorexia which
are the most common reasons for treatment withdrawal. Patch formulary References
may be worth a trial in such circumstances. Other common undesirable 1. Farlow MR, Grossberg G, Gauthier S, Mend X, Olin JT. The ACTION study: methodology of a trial to evaluate safety
and efficacy of a higher dose rivastigmine transdermal patch in severe Alzheimers disease. Current Medical Research
effects include diarrhoea, muscle cramps, fatigue, and insomnia. Special and Opinion 2010;26(10):2441-47.
precautions apply for patients with sick sinus syndrome, cardiac conduction 2. Farlow MR, Salloway S, Tariot PN, Yardley J, Moline ML, Wang Q, et al. Effectiveness and tolerability of high-dose (23
mg/d) versus standard-dose (10 mg/d) donepezil in moderate to severe Alzheimers disease: a 24-week, randomized,
defects, history of obstructive airway diseases, overactive bladder, double-blind study. Clin Ther 2010;32(7):1234-51.
gastroduodenal ulcer diseases and hepatic impairment. Occurrence of 3. Grossberg,G, Cummings J, Frlich L, Bellelli G, Molinevo JL, Krahnke T, et al. Efficacy of higher dose 13.3 mg/24
resting tremor mimicking Parkinsons disease has been reported after ChEI h rivastigmine patch on instrumental activities of daily living in patients with mild-to-moderate Alzheimers disease.
American Journal of Alzheimers Disease and Other Dementias 2013;28(6):583-91.
therapy. It is important to monitor any body weight loss after treatment. 4. Donepezil, galantamine, rivastigmine and memantine for the treatment of Alzheimers disease. London (UK): National
Whether we should continue treatment with progressive weight loss relies Institute for Health and Clinical Excellence (NICE); 2011 Mar. 84 p. (Technology appraisal guidance; no. 217).
5. Donepezil, galantamine, rivastigmine and memantine for the treatment of Alzheimers disease (Guideline Summary
on clinical judgment. NGC-8659)
6. Tricco AC, Soobiah C, Berliner S, Ho JM, Ng CH, Ashoor HM, et al. Efficacy and safety of cognitive enhancers for
patients with mild cognitive impairment: a systematic review and meta-analysis. CMAJ 2013;185(16):1393-1401.

166 167
SECTION 5
Late stage dementia

5.1 End of life management in late stage dementia


All dementia patients will invariably enter into the final stage. In this stage
patients start to lose their mobility, speech, and finally, the praxis of basic
activities such as walking, eating, and communication by facial expressions.
Anti-dementia drugs become less useful in this stage, as there are no longer
meaningful functions to be salvaged. The aim of management therefore will
switch from cognition and function maintenance, to providing the patient
with as much comfort as possible.

End of Life Care

SECTION 5 Very few early dementia patients would have considered how they would like
to spend the last year of their lives. Often patients and families are engrossed
in the loss of cognition and difficulties in daily activities in the earlier stages,

Late stage dementia without realizing that dementia and its complications lead to upto one-third
of deaths in developed countries. Being unaware and therefore unprepared,
many advanced dementia patients die with much suffering. In order to allow
family caregivers, and certain early patients, to have an informed choice of
end of life decisions, we must familiarize ourselves with some background
knowledge.

169
SECTION 5
Late stage dementia

When does End of Life begin? End of Life Symptoms in Dementia


Prognostication tools used for other diseases such as cancer or respiratory It is difficult to assess for end of life symptoms in dementia because most
failure cannot be applied to advanced dementia patients, because the disease patients would have become non-communicable by then. Symptoms
trajectories are very different. The Gold Standards Framework (UK) [1] used detection and relief therefore largely depends on the sensitivity of the
the following criteria for a life expectancy of approximately 12 months: caregiver and direct observation of behavioural changes in the patient. The
most common symptoms listed in the literature include aspirations, pressure
Triggers to consider that indicate that someone is entering a later stage are: ulcers, dyspnea and pain. [2] We must also remember that over-aggressive
Unable to walk without assistance and medical interventions such as invasive procedures and frequent blood-
Urinary and faecal incontinence, and taking or instrumentation will also cause discomfort or even unnecessary
risks. Restraints, often applied in relation to instrumentation such as tube
No consistently meaningful conversation and
feeding or intravenous fluid / medication administration, will always cause
Unable to do Activities of Daily Living (ADL) discomfort, immobility, and impair the quality of dying, in particular in the
Barthel score <3/20 severely demented who cannot articulate their needs.
Plus any of the following:
Weight loss
Detection of pain in advanced dementia
Urinary tract infection
Severe pressures ulcers - stage three or four Common tools for this purpose include:
Recurrent fever PAINAD (Pain Assessment in Advanced Dementia) [Warden et al. JAMDA
Reduced oral intake 2003]

Aspiration pneumonia NOPPAIN (Non-Communicative Patients Pain Assessment Instrument)


[Snow et al, Dement Geriatr Cogn Disord 2004]
In another cohort study (Mitchell, 2009), it was found that the clinical These tools are based on the observation of patient body language, non-
presentations of pneumonia, febrile illness other than pneumonia, or eating verbal vocalization and facial expressions, and can also be used for dementia
problems (including weight loss, swallowing or chewing problems, refusal to patients other than those at the end of life.
eat or drink, suspected dehydration, and persistently reduced oral intake)
were the most common clinical events that signaled the approach of the
end of life phase in advanced dementia patients. Having any of these events
reduced the one-year survival by approximately half in these patients. [2] The
adjusted 6-month mortality rates after the occurrence of these events were
46.7%, 44.5%, and 38.6%, respectively.
These data should be able to provide a guide to prognostication in clinical
practice.

170 171
SECTION 5
Late stage dementia

Management of pain or end of life symptoms Alternatives to tube feeding


The management of these does not differ from those of cognitively normal Towards the end of life, the aim of feeding is no longer the maintenance of
patients. However, in face of increased irritability, one should not assume a balanced, healthy diet for healthy living. When both life expectancy and
that it is delirium or BPSD. An active search for the cause of pain or physical functioning becomes severely limited, the purpose of eating is to
discomfort should always be done as in good geriatrics practice: relief of bring comfort and enjoyment. This view of feeding is also in line with the
bladder distension, constipation, itchiness or mal-placement of a limb can person-centred care approach in severe dementia. [6] Comfort feeding is a
often improve the quality of dying for these non-communicable patients. A term now adopted to describe careful hand-feeding for end of life patients.
trial of analgesics or related medications can also be attempted if in doubt. Although it is difficult to assess patient satisfaction in advanced dementia, it
is reasonable to believe that personal attention and tasty food provided by a
Feeding Problems in Advanced Dementia kind face should be preferred over a tube and bottle hanging over the bed.

Is tube feeding a good solution? Problem Possible solutions

Feeding problems often herald the last stage of life in dementia. Common Choking, coughing Upright positioning, chin-down head position, smaller spoon,
symptoms are slow eating, choking or coughing on or after eating, noisy thickened fluids, minced or puree texture
breathing or wet voice, refusal to eat, or progressive weight loss. Eating
problems are often due to dysphagia which is a part of the neurodegenerative Wet voice Prompt to swallow or cough, gentle throat stroking, change
head position
process. Refusal to eat may be due to fear of choking with food, which
the patient cannot communicate to caregivers, or agnosia (when the Refuse eating Modify environment, reduce disturbance in surrounding,
patient cannot recognize food as food), or simply due to anxiety caused improve food appearance or taste, favourite foods, favourite
by unfamiliar surroundings or a change of caregivers. Tube feeding is often caregiver
used to manage feeding problems in advanced dementia. However, there
is little evidence to support that tube feeding can improve survival, prevent Poor appetite / Rule out pathology from mouth to anus (oral ulcers, poor
early satiety dental/oral hygiene, constipation), avoid over-sedation, reduce
aspiration pneumonia and prevent pressure ulcers. Yet there are ample
unnecessary medications (pill burden)
evidence that tube feeding is associated with increased use of restraints and
patient discomfort (including that due to force feeding such as abdominal Weight loss Keep finger foods or favourite snacks at hand, frequent small
bloating, and those due to the presence of the nasogastric tube, such high caloric-dense meals (forget about diet restrictions!)
as nasal / esophageal erosions, frequent tube replacement, reflux and
associated pneumonitis / pneumonia). [5]

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SECTION 5
Late stage dementia

Conclusion 5.2 Enduring power of attorney, guardianship order


End of life care and feeding problems in advanced dementia are quality of
and advance directive
life (or death) issues. Medications are often not the mainstay of care, and 5.2.1 Enduring Powers of Attorney (EPA)
as survival time is limited, invasive or over-zealous interventions should be
Like most common law jurisdictions, Hong Kong SAR has put in place the
avoided. Caregiver quality determines the quality of the care.
Enduring Power of Attorney Ordinance, Cap, 501 since 1997. This is an
Therefore, the task of the healthcare professional will have to change from advance instrument by a maker to appoint another person of the makers
curative or preventive therapy to caregiver education and support; and choice, while still mentally capable, to manage those areas of financial
finally, symptom relief for the patient in his or her last months or days. affairs specified therein, in the event that the maker subsequently loses
mental capacity to take charge of finances. Due to extremely low take-up
(only 54 EPA registered as at 30 June 2012), two important consultations
were completed by the Law Reform Commission to improve the system
respectively in April 2007 (Report in March 2008) and in July 2009 (Report
in July 2011). The first mentioned consultation dealt with the cumbersome
dual-witness requirement (i.e. simultaneous witnessing by a lawyer and a
medical doctor) and the second one dealt with expanding the scope of
EPA to include personal care and new supervisory powers to Guardianship
Board. The first law amendment was made effective on 3 July 2012 to relax
dual-witness requirement to one witness following by the other within 28
days. The second law amendment is now being actively processed.
Therefore, those older adults at risk of or in the very early stage of
neurocognitive disorder should be advised and encouraged to make such an
advance instrument at the time while he is still mentally capable to appoint
his surrogate.

References
1. http://www.goldstandardsframework.org.uk/cd-content/uploads/files/General%20Files/Prognostic%20
Indicator%20Guidance%20October%202011.pdf; last accessed on 24 Dec 2013.
2. Mitchell SL, Teno JM, Kiely DK, Shaffer ML, Jones RN, Prigerson HG, et al. The clinical course of advanced dementia.
N Engl J Med 2009;361(16):1529-38.
3. Warden V, Hurley AC, Volicer L. Development and psychometric evaluation of the Pain Assessment in Advanced
Dementia (PAINAD) scale. J Am Med Dir Assoc 2003;4(1):9-15; available at http://www.amda.com/caring/may2004/
painad.htm
4. Snow AL, Weber JB, OMalley KJ, Cody M, Beck C, Bruera E, et al. NOPPAIN: a nursing assistant-administered pain
assessment instrument for use in dementia. Dement Geriatr Cogn Disord 2004;17(3):240-6.
5. Finucane TE, Christmas C, Travis K. Tube feeding in patients with advanced dementia: a review of the evidence.
JAMA 1999;282(14):1365-70.
6. Edvardsson D, Winblad B, Sandman PO. Person-centred care of people with severe Alzheimers disease: current
status and ways forward. Lancet Neurol 2008;7(4):362-367.

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SECTION 5
Late stage dementia

5.2.2 Guardianship Order (GO) 5.2.3 Advance directive (AD)


Hong Kongs adult guardianship system was established in February 1999. In general and in Hong Kong context, advance directive is made by an
The Guardianship Board will, on application, appoint a guardian for a adult while mentally capable to refuse certain specified life-sustaining
patient or a mental handicapped person (who must be over 18) if certain treatments (LST) to be provided to him at a time when he becomes
legal criteria are satisfied. These criteria include whether the patient (i.e. mentally incapacitated due to terminal illness, persistent vegetative state or
subject-person) is established by medical evidence that s/he is a mentally irreversible coma. LST include cardiopulmonary resuscitation and artificial
incapacitated person within the meaning of s.2 of Mental Health Ordinance hydration and nutrition etc. Forms of AD are NOT prescribed by law in Hong
Cap. 136. Also, it must be proved that the subject-person is suffering from Kong. However, the model form proposed by the Law Reform Commission
mental incapacity to an extent or degree that warrants his/her reception into and the form used by the Hospital Authority each sets out that the signing
guardianship. The Board also considers if there are other informal means by the maker should be in the presence of two witnesses, one of whom is a
through which the particular need of the subject-person can be satisfied medical practitioner.
without guardianship. The Board aims to act as a facilitator and empowering
In summary, the EPA and the AD can be made at the time when an adult
authority to protect or promote the best interests of the subject-person. It
is mentally capable whereas the GO is used to promote the interests of a
is important to mention that a death during guardianship will need to be
mentally incapacitated person without any advance instruments. The EPA
subjected to a coroners inquest. Also, all guardianship orders will have to
can only be used to manage finance currently but is now being reviewed
be reviewed before expiration. Currently, the Board deals with around 600
to expand the scope to personal care and medical decisions. On the other
matters a year and predominant users are elderly people.
hand, the AD takes charge of withholding or withdrawal of life-sustaining
The Guardianship Board can grant to a guardian six powers which include treatments of patients in terminal conditions. The Guardianship Board can
consent to treatment (but not refusal or withdrawal of treatment), to decide only grant six limited powers to a guardian but the financial power remains
on finance and residence arrangement. Unlike EPA, the financial power of restricted. Clinicians taking care of demented patients should have some
a guardian is restricted to the use of cash or savings within a monthly limit understanding about the varied functions and purposes of the three legal
according to the latest median monthly earnings (currently $12,500). Therefore, provisions above.
if the assets are sizeable and varied, the family should apply to High Court
under Part II of the Mental Health Ordinance for a committee appointment.
With regard to medical treatment to be provided to a mentally incapacitated
person, a clinician can provide treatment without consent of a legal guardian
provided such treatment is in the persons best interests pursuant to Part IVC
of the Mental Health Ordinance. In Hospital Authority practice, two medical
practitioners are required to sign for the medical procedure. It is good clinical
management to seek the agreement (not consent) of the next of kin at the
same time. Mostly, no guardianship application is needed. Guardianship
application is required only in controversial circumstances e.g. the treatment
risk and benefit balance is not definitive or the patient or family objects to a
treatment of obvious medical benefits.

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SECTION 6
First time assessment of neurocognitive disorder in older adults

6.1 Practical guide for first time assessment


This guide is by no means exhaustive and can be shortened and lengthened
depending on the clinical context. Do not use it as a prototype questionnaire.
Clinical assessment is an art and the flow of communication among the
clinician, patient and care-giver can often change and shift during the
consultation. Most likely, all the following cannot be done in one session
because it will be too long and the patient will become tired. Modify it
according to your own work setting.

SECTION 6 History (collect history from someone who knows the patient well)
Referral source
Main carer
First time assessment of Age and sex

neurocognitive disorder Past or current occupation


Years of education
in older adults Smoking and alcohol habit

Chief complaint
Cognitive deficits
Duration / onset / course / initial symptom (memory first or other cognitive
domain first)
Memory loss
Perceptual problems
Disorientation: time, place, person
Language (naming, speech and communication) difficulty
Executive function
Attention

179
SECTION 6
First time assessment of neurocognitive disorder in older adults

Suggested list of questions: Physical examination


Short term memory became worse? General impression - Apathetic / agitated / sociable
Stocking repeated items in refrigerator? Pulse regular or in AF, bruits, murmur
Forgot meals? Focal neurological sign, muscle tone, muscle power, tendon reflexes
Forgot turn off stove? Frontal lobe assessment (please refer to P.77 for details)
Got lost in neighbourhood? Gait and parkinsonian sign
Cannot recognise persons? Do not omit other systemic examination (you could miss a para-neoplastic
syndrome presenting as dementia)
Functional assessment for staging (suggested items)
Basic ADL - selection of appropriate clothes, continence status Neurocognitive tests
Instrumental ADL - telephone, finance (banking, paying bills), shopping, Depending on the setting and time constraint and resources, find an
cooking, medication management, travelling alone appropriate set of test you find practical and you are familiar with.
Social activities and hobbies Note the affect and attention during the test.

Behvioural and psychological symptoms of dementia (BPSD) Suggested list:


Apathy, initiation, depression, anxiety, irritability, anger, delusion, AD8
hallucination, agitation, aggression, temper outburst, wandering, pacing MMSE
Sleep disturbance, sun-downing, REM sleep behavior disorder MoCA
Clock drawing test
Common examples:
Naming test
Delusion of theft by domestic maid
Motor sequencing test - slap/fist/cut
Visual hallucination - seeing many small people or deceased relatives
Sleep and nighttime behaviour disturbance
Lost interest in past hobbies (e.g. playing Mahjong, watching TV, reading
newspaper)

Carer stress / community services / respite care/ daycare resources

Past health : diabetes mellitus, hypertension, stroke, atrial fibrillation (AF)

Drug history
Family history of neurocognitive disorder

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SECTION 6
First time assessment of neurocognitive disorder in older adults

Investigations
Renal and liver function, complete blood picture
Blood Glucose, lipid profile
Thyroid function
Serum vitamin B12 and folate level
VDRL (optional)
Neuroimaging
ECG - note AF (vascular dementia) , long QT interval (neuroleptic treatment
precaution), bradycardia

Diagnosis - type and stage (use FAST scale if AD suspected)

Management
Social aspects
Prognosis information
Financial arrangement
Advance care planning
Enduring power of attorney
Community and Day care resource

Medical aspects
Vascular risk factors control
Non-pharmacological treatment (do not ignore this)
Pharmacological treatment (it may not be the most useful part)
Follow-up

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ACKNOWLEDGEMENT

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