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VASCULAR DEMENTIA

I NTRODUCTION

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 cerebrovascular disease.

Presentation of VaD is variable and the clinical spectrum is wide

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.

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In India AD>VaD

H ISTORY

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) Organic psychosis 293.0 Psychosis associated with cerebral arteriosclerosis 293.1 Psychosis associated with other cerebrovascular disturbances ICD 9 (1975) 290.4 Arteriosclerotic dementia ICD 10 (1990) 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, unspecified

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 white-matter lesions) and haemodynamic mechanisms Less frequent causes include hereditary disorders, arteriopathies (CADASIL), amyloidopathies(CAA), haemorrhage (intracranial haemorrhage, SAH), haematological factors, venous disease, vasculitis.. New concept-cholinergic deficit in vascular dementia similar to that seen in AD

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).

C LINICAL

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/sub-cortical) Behavioral and psychological symptoms depression, anxiety, emotional lability, psychomotor retardation.. Associated features HTN, cardiac murmurs, carotid bruits, AF..

The ischemic index by Hachinski et al (1975) widely employed to distinguish multi-infarct dementia from AD.

A score 7 or above suggests multi-infarct dementia.(4 or less nonvascular dementia)


Laboratory and radiological investigationsdepending on suspected aetiology. Diagnosis based on criteria (ICD 10, ICD DCR, DSM IV, NINDS-AIREN, ADDTC)

S UB - 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)

M ULTI - 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, tinnitus, syncope main c/o initially.

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 focus. Paroxysmal or normal EEG also common frontal delta activity if delirium

S MALL - 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 during clock reading - quadratic function of dementia state) 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.

B INSWANGER S

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.

P OST - 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

I NHERITED

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 Incl: pred cortical dementia gradual onset than ac form, following no of minor ischemic eps producing accumulation of infarcts in cerebral parenchyma. F01.2 Subcortical vascular dementia h/o HTN and foci of ischaemic destruction in the deep WM of the cerebral hemispheres, susp on clinical grounds and CT. cerebral cortex is preserved and contrasts to AD. (if diffuse demyelination of white matter- Binswanger's encephalopathy)

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 290.4 X VASCULAR D EMENTIA


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 predominates in the current clinical presentation
Specify if: With Behavioral Disturbance Coding note: Also code cerebrovascular condition on Axis III.

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) pseudobulbar palsy.
G4. evidence from the history, examination, or tests, of significant cerebrovascular disease, which may reasonably be judged to be etiologically related to the dementia (e.g. a history of stroke; evidence of cerebral infarction).

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 of possible vascular 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 of definite vascular dementia are (a) clinical criteria for probable vascular 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.

A LZHEIMER ' S D ISEASE D IAGNOSTIC AND T REATMENT C ENTERS (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 UBJEC TS

ID ENTIF I E D AS HAVING

VA D

AC C O RD IN G TO

VARIO U S D IAGNO S T IC C RITE R I A .

DSM IV 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 Temporal relationship Structural brain imaging Un-supporting features Different levels of certainty + + + + + + + + -

ICD 10 + + + + + + + + -

ADDTC NINDS + + + + + + + + + + + + + + + + + + + +

D IFFERENTIAL D IAGNOSIS

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 NINDSAIREN 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 - 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

Alzheimer's Disease - 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

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 Apathy, social withdrawal,tremor,hypertonia,hyperreflexia common Choreiform movements precede dementia

Parkinsons Disease Frontotemporal Dementia

Creutzfeldt-Jakob Disease HIV dementia Huntingtons

mild cognitive impairment (MCI) with multiple impaired cognitive domains (mcd-MCI) 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)

C OURSE

AND

P ROGNOSIS

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

T REATMENT

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 apnea-positive 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 -dose-related 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

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