VASCULAR DEMENTIA
Presenter Dr Pavan kumar
John Mathai
Chaired by Dr P
INTRODUCTION
Vascular dementia - cognitive decline caused
by
ischemic,
hemorrhagic, or
oligemic injury to the brain
as a consequence of
cerebrovascular
or
cardiovascular
disease.
It is part of a spectrum of vascular disease
causing cognitive impairment, which also
includes
1) mild cognitive impairment of vascular
origin and
2) mixed Alzheimer's disease plus
EPIDEMIOLOGY
Epidemiology -affected by variations in
definition of disorder, clinical criteria
used, and
clinical methods applied.
10-50% depending upon geographic location,
patient population, and clinical methods used
Prevalence of 1.2-4.2% of persons aged >65 yrs,
6-12 cases per 1000 per year aged >70 years.
Vascular pathology coexists with other forms
in many, if not most, cases of
dementia.
10 20 % of dementia when only a pure
vascular etiology is considered
Isolated vascular dementia - now considered
the 3rd MC form of dementia after AD and DLB.
(Kaplan & Saddock)
Mixed Alzheimer's disease + Cerebrovascular
disease may be the MC presentation of
dementia.
>
In India AD>VaD
HISTORY
Kraepelin (1896. ) - arteriosclerotic
dementiadirect result of
arteriosclerotic disease in brain.
Hachinski multi-infarct dementia as
dementia related to series of multiple
cerebral infarcts. (Hachinski Ischemic Score in
1975).
subcortical dementia introduced in 1970s in
reference to pts with vascular lesions. Invalid
&rational to change the term from subcortical
dementia to `fronto-subcortical dementia
Later vascular dementia in ICD 10 & DSM IV.
ICD
ICD 8 (1965)
ICD 9 (1975)
ICD 10 (1990)
Organic psychosis
293.0
Psychosis associated
with cerebral
arteriosclerosis
290.4
Arteriosclerotic
dementia
F01 Vascular
dementia
F01.0 Vascular
dementia of acute
onset
F01.1 Multi-infarct
dementia
F01.2 Subcortical
vascular dementia
F01.3 Mixed cortical
and subcortical
vascular dementia
F01.8 Other vascular
dementia
F01.9 Vascular
dementia,
293.1
Psychosis associated
with other
cerebrovascular
disturbances
DSM
DSM I (1952) - `Organic Brain Syndrome' (OBS), chronic
and `more or less' irreversible in contrast to Acute brain
injury.
DSM II- `Psychoses associated with organic brain
syndromes with cerebral arteriosclerosis (293.0) .
DSM III, DSM III R& DSM IV no longer mentioned OBS
and concept of irreversibility, and introduced the term
dementia & defined as `a loss of intellectual abilities of
sufficient severity to interfere with social or occupational
functioning'.
DSM IIIR-Multi-Infarct Dementia,
DSM IV-renamed as Vascular Dementia as course quite
variable and not always stepwise, dropped the
requirement of patchy distribution of deficits, and allowed
evidence to be either laboratorial or physical.
In 1993, the NINDS-AIREN criteria (relationship b/w
dementia & CeV disease) were published, most
widely accepted and used in research studies.
concept of vascular dementia continues to evolve
and undergone considerable revision in last 2 to 3
decades
New concept of vascular cognitive impairment
(VCI) introduced - encompassing all forms of
cognitive impairment related to vascular disease in
brain.
the importance of vascular lesions in Alzheimer's
disease is being increasingly recognized
ETIOLOGY
Main causes- Cerebrovascular diseases and their
risk factors
large artery disease
(artery-to-artery embolism, occlusion
of an extra- or intracranial artery),
cardiac
embolic events,
small vessel disease
(lacunar infarcts, ischaemic whitematter lesions) and
haemodynamic mechanisms
Less frequent causes include hereditary disorders,
arteriopathies (CADASIL),
amyloidopathies(CAA),
haemorrhage (intracranial haemorrhage, SAH),
Risk factors
vascular factors (e.g. HTN,AF,MI, CAD,DM,
generalized atherosclerosis, lipid abnormalities,
smoking),
demographic factors (e.g age, education),
genetic factors (e.g. family history, individual genetic
features), and
stroke-related factors (e.g. type of cerebrovascular
disease, site and size of stroke).
Hypoxic ischaemic events (cardiac arrhythmias, CHF,
MI, seizures, pneumonia) may be an important risk
factor for incident dementia in patients with stroke.
PATHOLOGY
vascular changes in the brain -infarct and embolism,
haemorrhage, hypovolemia background pathology
cognitive impairment depends on
volume of brain infarcts (with a critical threshold),
number of infarcts,
site of infarcts (b/l, in strategic cortical or subcortical, or
affecting white matter),
other ischaemic factors (incomplete ischaemic injury,
delayed neuronal death, functional changes),
atrophic changes (origin, location, extent), and
finally to the additive effects of other pathologies
(Alzheimers, DLB pathology, frontal lobe dementia
pathology).
CLINICAL FEATURES
development of multiple cognitive deficits both memory impairment and
impairment in at least
one other cognitive domain including language,
praxis, gnosis, and executive functioning.
cognitive deficits - decline from previous level of
functioning, interference with personal activities
of daily living, significant impairment in social or
occupational functioning,
symptoms - heterogeneous dependent on type
and location of the vascular lesions and etiology.
Imp to establish a temporal and causal
relationship b/w the brain lesion and the
cognitive impairment.
Cognitive impairment-Memory deficits and
dysexecutive syndrome and information
processing deficits are common.
Typical focal neurological signs (cortical/subcortical)
Behavioral and psychological symptoms
depression, anxiety, emotional lability,
psychomotor retardation..
The ischemic index by Hachinski et al
(1975) widely employed to distinguish
multi-infarct dementia from AD.
A score 7 or above suggests multiinfarct dementia.(4 or less
nonvascular dementia)
Laboratory and radiological
investigations- depending on suspected
aetiology.
Diagnosis based on criteria (ICD 10, ICD
DCR, DSM IV, NINDS-AIREN, ADDTC)
SUB-TYPES
Subtypes based on clinical, radiological &
neuropathological features.
Multi-infarct dementia/ cortical vascular dementia
Small-vessel disease/subcortical vascular
dementia/SIVD
Post-stroke dementia/ strategic infarct dementia.
Specific vascular dementia syndromes(hereditary
vascular)
Other- hypoperfusion, hemorrhagic, and
combined/mixed dementia(AD with CeV disease)
MULTI-INFARCT DEMENTIA
CORTICAL VASCULAR
DEMENTIA
Large vessel disease , cardiovascular risks,
hypoperfusion
Late 60s / 70s, Almost =,with perhaps slight
excess in .
Onset frequently more acute than in AD (after
frank CVA) and patchy nature of deficits.
Cognitive impairmentfluctuate in severity & progression to
be stepwise.
If gradual, emotional & personality changes
antedate memory and intellectual impairment
Somatic symptoms - headache, dizziness,
apoplectiform features punctate the progress due to
episodes of cerebral infarction.
Abrupt episodes of hemiparesis, sensory change,
dysphasia or visual disturbances
Each further episode leaves permanent neurological
deficits and increase in severity in dementia
Pseudobulbar palsy (dysarthria, dysphagia and
emotional incontinence), urinary incontinence,
psychomotor slowing, bradykinesia, gait
abnormalities, frequent falls, difficulty in set shifting.
Unequal DTR, plantars extensor, pupils RL- impaired.
Parkinsonian features , epileptic seizures (20%) seen.
Basic personality, capacity for judgement,
insight preserved for long time.(presents
anxiety and depression)
Lability- explosive emotional outbursts without
accompanying subjective distress/ elation.
Neuroimaging MRI > CT
usually evidence of cerebral
atrophy,
old &
recent infarctions revealed,
EEG-- similar to AD, but
severe and focal /lateralizing
abnormalities in infarct region-- low-ampl delta
SMALL-VESSEL DISEASE
SUBCORTICAL VASCULAR
DEMENTIA
SIVD
Pathology falling short of infarct.
Major cause of cognitive impairment and indeed
dementia. (Lishman)
Sub-cortical disorder slow evolving dementia- acc
by prominent motor signs or subacute FNDs in HTN
pts in 40 or 60s.
Damage to micro-vasculature in the brain,
demyelination, axonal loss and gliosis.
Cardianal features1} WMLs as
a)periventricular lucency (aka Leucoaraiosis),
b)deep white matter hyperintensities.
2} central grey matter lacunae
Initially thought of neuro-radiological marker
but found in 92% of elderly with or without
dementia and predicted by mid-life HTN.
Peri-ventricular lesions predictive of dementia
than deep white matter hyperintensities.
Periventricular lucency- marker of VCI
Deep white matter hyperintensities end
organ damage - lifelong vascular damage due
to DM with or without HTN--ass with
depression & motor deficits.
Variable manifestation some show ebullience
(zestful enthusiasm), some progressive loss of
spontaneity, memory disorder not invariably
prominent.
Word fluency and clock- reading -- differentiate
from AD.(Cortical activation duringclock readingquadratic function ofdementiastate)
Persistent HTN, pure motor hemiparesis, bulbar
signs, dysarthria, depression, emotional lability and
deficits in executive functioning.
lengthy course, FNDs- subacute progression over
wks/mnts, stabilizing with long plateau periods.
BINSWANGERS
DISEASE
Sub-type of small vessel disease
Encephalitis subcorticalis chronica progerssivaBiswanger.
Diffuse ischemia- subacute HTN encephalopathy/
chronic hypoperfusion in watershed areas in white
matter
Pathology- long perforating vessels to deep white
matter and subcortical nuclear masses- lacunes and
diffuse demyelination of white matter (cardinal
feature)
Arcuate fibres beneath sulci and cortex spared.
POST-STROKE DEMENTIA
STRATEGIC INFARCT
DEMENTIA
1/3rd above 55 yrs have dementia in 5 yr
period after stroke- 9 times greater than
predicted in gen pop.
Hemispheric lesions increase the risk
In some incipient AD become apparent after
stroke b/c cognitive or brain reserve is
diminished or effects of stroke make cognitive/
functional deficits apparent.
CADASIL
cerebral autosomal dominant arteriopathy with subcortical
infarcts and leukoencephalopathy
Onset 40s, Familial, AD inheritance,NOTCH3 gene
mutations
Free from typical vascular risk factors
C/f - migraine usually with aura,
recurrent small sub-cortical infarcts leading to dementia,
TIAs, sometimes severe affective disturbance.
Imaging- abn in subcortical white matter and basal ganglia
Identifying families presymptomatic counselling &
testing
INHERITED
ANGIOPATHIES
Heriditary cerebral hemorrahage with amyloidosis
(Dutch type)
AD inheritance APP gene mutations
Pathology cerebral amyloid angiopathy (CAA)-decrease in A-42 in contrast to increase in
Alzheimers.
Hemorrhagic strokes and dementia
Other angiopathies- heriditary cerebral
hemorrhage with amyloidosis (Iceland type) & chr
13 familial dementia in British and Danish kindreds.
DIAGNOSIS
ICD 10 F01 VASCULAR
DEMENTIA
Vascular (formerly arteriosclerotic) dementia
presence of a dementia
Cognitive impairment is commonly uneven, so that
there may be memory loss, intellectual impairment
and FN signs.
Insight and judgement may be relatively well
preserved.
An abrupt onset or stepwise deterioration, as well as
the presence of FN signs and symptoms, increases
the probability of the diagnosis;
in some cases, confirmation can be provided only by
CT or, ultimately, neuropathological examination.
Associated features are: HTN, carotid bruit, emotional
lability with transient depressive mood, weeping or
explosive laughter, and transient episodes of clouded
consciousness or delirium, often provoked by further
infarction.
Personality is believed to be relatively well preserved, but
personality changes may be evident in a proportion of cases
with apathy, disinhibition, or accentuation of previous traits
such as egocentricity, paranoid attitudes, or irritability.
DD: delirium (F05.-); other dementia, particularly in AD
(F00.-); mood disorders (F30-F39); MR (F70-F71);
SDH(traumatic (S06.5), nontraumatic (162.0)).
Vascular dementia may coexist with dementia in AD (to be
coded F00.2), as when evidence of a vascular episode is
superimposed on a clinical picture and history suggesting
AD
F01.0 Vascular dementia of acute onset
Usually develops rapidly after a succession of
strokes from cerebrovascular thrombosis,
embolism, or haemorrhage, In rare cases, a single
large infarction may be the cause.
F01.1 Multi-infarct dementia
dementia
Incl: pred cortical
gradual onset than
ac form, following no of minor ischemic eps
producing accumulation of infarcts in cerebral
parenchyma.
F01.2 Subcortical vascular dementia
F01.3 Mixed cortical and subcortical vascular
dementia
Mixed cortical and subcortical
components of the vascular dementia may be
suspected from the clinical features, the results
of investigations (including autopsy), or both.
F01.8 Other vascular dementia
F01.9 Vascular dementia, unspecified
DSM IV TR CRITERIA FOR
290.4X VASCULAR
DEMENTIA
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)
(2) one (or more) of the following cognitive
disturbances:
(a) aphasia (language disturbance)
(b) apraxia (impaired ability
to carry out motor activit ies despite intact motor
function)
(c) agnosia
(failure to recognize or identify objects despite intact
sensory function)
(d)
disturbance in execut ive f unctioning (i .e., planning,
organizing, sequencing, abstracting)
B. The cognitive deficits in Criteria Aland A2 each cause
significant impairment in social or occupational
functioning and represent a significant decline from a
previous level of functioning.
C. Focal neurological signs and symptoms
(e.g- exaggeration of DTRs, extensor plantar,
pseudobulbar palsy, gait abn, weakness of an extremity)
or
laboratory evidence indicative of
cerebrovascular disease (e.g., multiple infarctions
involving cortex and underlying white matter) that are
judged to be etiologically related to the disturbance.
D. The deficits do not occur exclusively during the
course of a delirium.
Code based on predominant features:
290.41 With Delirium: if delirium superimposed
on dementia
290.42 With Delusions: if delusions are
predominant feature
290.43 With Depressed Mood: if depressed mood
(including presentations that meet full symptom
criteria for a MDD episode) is the predominant
feature.
A
separate diagnosis of Mood dis due to GMC is
not given.
290.40 Uncomplicated: if none of the above
ICD DCR
F 01
G1. The general criteria for dementia (G1 to G4)
must be met.
G2. Unequal distribution of deficits in higher
cognitive functions, with some affected & others
relatively spared. Thus memory may quite
markedly affected while thinking, reasoning &
information processing may show only mild
decline.
G3. clinical evidence of focal brain damage, as at
least 1of the:
(1) unilateral spastic
weakness of the limbs;
(2) unilaterally increased tendon reflexes;
(3) an extensor plantar
response;
(4)
The following criteria may be used to
differentiate subtypes of vascular dementia,
but it should be remembered that the
usefulness of this subdivision may not be
generally accepted.
F01.0 Vascular dementia of acute onset
A. The general criteria for vascular dementia
(F01) must be met.
B. The dementia develops rapidly (i.e. usually
within one month, but within no longer than
three months) after a succession of strokes,
or (rarely) after a single large infarction.
F01.1 Multi-infarct dementia
A. The general criteria for vascular dementia
(F01) must be met.
B. The onset of the dementia is gradual (i.e.
within three to six months), following a
number of minor ischaemic episodes.
It is presumed that there is an accumulation of
infarcts in the cerebral parenchym.
Between the ischaemic episodes there may
be periods of actual clinical improvement.
F01.2 Subcortical vascular dementia
A. The general criteria for vascular
dementia (F01) must be met.
B. A history of hypertension.
C. Evidence from clinical examination
and special investigations of vascular
disease located in the deep white
matter of the cerebral hemispheres,
with preservation of the cerebral cortex.
F01.3 Mixed cortical and subcortical
vascular dementia
Mixed cortical and subcortical
components of the vascular dementia
may be suspected from the clinical
features, the results of investigations
(including autopsy), or both.
F01.8 Other vascular dementia
F01.9 Vascular dementia, unspecified
NINDS-AIREN
CONTD
IV. Clinical diagnosis ofpossiblevascular dementia
1.dementia with focal neurologic signs but brain imaging
to confirm definite CVD are missing;
2.absence of clear temporal relationship between
dementia and stroke;
3. subtle onset & variable course
(plateau or improvement) of cognitive deficits and
evidence of relevant CVD.
V. Criteria for diagnosis ofdefinitevascular dementia are
(a) clinical criteria forprobablevascular dementia;
(b) histopathologic evidence of CVD from
biopsy or autopsy;
(c) absence of neurofibrillary tangles and
neuritic plaques exceeding those expected for age; and
(d) absence of other clinical or
pathological disorder capable of producing dementia.
ALZHEIMER'S DISEASE
DIAGNOSTIC AND TREATMENT
CENTERS(ADDTC)
A. 1. Dementia
2. Evidence of two or more ischemic strokes
by History, neurological signs, and/or
Neuroimaging studies (CT or T1-weighted
MRI),
Occurrence of a single stroke with a clearly
documented temporal relationship to the
onset of dementia
3. Evidence of 1 infarct outside the
cerebellum by CT or T1-weighted MRI
B. Diagnosis of probable IVD is supported by
1. Evidence of multiple infarcts in brain
regions known to affect cognition (as defined
by NINDS-AIREN criteria)
2. History of multiple transient ischemic
attacks.
3.History of vascular risk factors (e.g.,
hypertension, heart disease, diabetes
mellitus)
4. Elevated Hachinski Ischemia Scale score
(7)
C. Clinical features that are thought to be
associated with IVD but await further
research
1. Relatively early appearance of gait
disturbance and urinary incontinence
2. Periventricular and deep white matter
changes on T2-weighted MRI that are
excessive for age
3. Focal changes in
electroencephalographic studies
D. Other clinical features that do not
constitute strong evidence either for or
against a diagnosis of probable IVD
1. Periods of slowly progressive symptoms
2. Illusions, psychoses, hallucinations,
delusions
3. Seizures
E. Clinical features that cast doubt on a
diagnosis of probable IVD
1. Transcortical sensory aphasia in the
absence of corresponding focal lesions on
neuroimaging studies
2. Absence of central neurological
symptoms/signs other than cognitive
disturbance
S UB J EC TS I DEN TIF I ED AS HAVIN G VAD
A C C O RD IN G TO VARIO U S D IA G N O ST IC
C RI TER IA .
Concept of mixed
aphasia
Infraction/ischemic
stroke
Hemorrhage
Memory disturbance
Stepwise deterioration
Patchy cognitive
deficits
Focal neurological signs
Focal neurological
symptoms
Evidence of stroke
events
Etiological relation to
disturbance
DSM
IV
ICD
10
ADD
TC
NIND
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DIFFERENTIAL
DIAGNOSIS
Vascular dementia is differentiated from other dementias on the
basis of its onset, mode of progression, neurological signs and
radiographical evidence.
Alzheimer Disease with Cerebrovascular Disease
If patients meet both the NINCDS-ADRDA criteria for AD, the
NINDS-AIREN criteria for VaD, they are diagnosed with both.
If they meet the criteria for AD but not for vascular dementia,
then they are given a diagnosis of Alzheimer's disease with
cerebrovascular disease
Features that generally exclude a diagnosis of vascular dementia
are early onset and progressive decline of a memory deficit.
In addition, absence of focal neurological signs and symptoms
and absence of cerebrovascular lesions on structural
neuroimaging studies also exclude a diagnosis of vascular
dementia.
Vascular Dementia
Alzheimer's Disease
- History of atherosclerotic
diseases: present
- Onset sudden or gradual
- Progression slow or stepwise
- Neurological deficits
- Gait often disturbed early
- Memory mild impairment in
early phase
- Executive function marked
impairment and early
- Type of dementia subcortical
- Hachinski Ischemic Score 7
- Neuroimaging infarction or
white matter lesions
- History of atherosclerotic
diseases less common
- Onset gradual
- Progression slow, progressive
decline
- Neurological examination
normal
- Gait usually normal
- Memory impairment prominant
in early phase
- Executive impaired later
- Type of dementia cortical
- Hachinski Ischemic Score 4
- Neuroimaging normal or
hippocampal atrophy
Dementia with
Lewy bodies
Parkinsons
Disease
Frontotemporal
Dementia
CreutzfeldtJakob
Disease
Visual hallucinations, muscle rigidity & tremors
common.
Alertness & severity of cognitiion may fluctuate daily.
Hallmarks include Lewy bodies
develop dementia in the later stages of the disease.
Hallmark abnormality is Lewy bodies.
similar presentation as subcortical VaD with slow
insidious onset, frontal executive deficits, relatively
preserved memory early in the course, and personality
change
Neuroimaging -frontal and/or temporal atrophy, blood
flow, or metabolic reductions.
Rapidly fatal disorder that impairs memory and
coordination and causes behaviour changes
HIV dementia
Huntingtons
Apathy, social
withdrawal,tremor,hypertonia,hyperreflexia common
mild cognitive impairment (MCI) with
multiple impaired cognitive domains (mcdMCI) is a prodromal manifestation of
vascular dementia (VaD)
Amnestic disorder no global intellectual
impairment differentiating from dementia
Post-concussional disorder- difficulty in
attention (concentrating, shifting focus of
attention, performing simultaneous
cognitive tasks)
COURSE AND
PROGNOSIS
dependent on the nature and course of the
vascular disease that causes it.
Onset sudden (large vessel disease/strategic) or
insidious (small vessel disease)
Course may be static if there are not further
vascular events, or remitting or progressive
often with a fluctuating stepwise decline
coinciding with further vascular events
may also be continued decline even in the
absence of clearly defined vascular events
TREATMENT
primary prevention and symptomatic treatment.
Primary prevention -controlling or ameliorating vascular
risk factors to prevent vascular damage occurring in the
brain.
HTN, DM, hyperlipidemia adequately controlled.
AF-Rx with anticoagulants to prevent thrombus
formation.
carotid stenosis -endarterectomy or angioplasty.
H/o TIAs /stroke- antiplatelet therapy/aspirin to
prevent rec
sleep apneapositive airway pressure to optimize cardiopulmonary
functioning
healthy lifestyle changes such as diet, exercise, weight
loss, stress reduction, decreased salt intake, and
cessation of smoking
Secondary prevention-no evidence it
prevents further cognitive deterioration
no FDA-approved treatments for vascular
dementia
cholinesterase inhibitors-Donepezil -doserelated improvement in cognitive function,
activities of daily living, and global
functioning, and functional deterioration
was slowed compared to placebo
galantamine and rivastigmine
Memantine- in more severe disease
CONCLUSION
Vascular factors may be the leading cause of
cognitive impairment world wide as opposed when
dementia is considered.
concept of vascular dementia continues to
evolve and undergone considerable revision in
last 2 to 3 decades
Presentation of VaD is variable and the clinical
spectrum is wide
Variable diagnostic criteria
New ways of treatment evolving apart from
preventive measures
THANK U
