You are on page 1of 57

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
S

+
+
+
+
+
+
+

+
+
+
+
+
+
+

+
+
+
+

+
+
+
+
+
+
+

+
-

+
-

+
+
+
+

+
+
+
+
+

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

You might also like