Neurological Manifestations of Vascular Dementia

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Neurological Manifestations of Vascular Dementia

Author: Jasvinder Chawla, MD, MBA; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA

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Updated: Feb 16, 2013

Practice Essentials

Signs and symptoms

Vascular dementia is a heterogeneous entity with a large clinicopathological spectrum that has been classically

linked to cortical and subcortical ischemic changes resulting from systemic, cardiac, or local large- or small-vessel

disease occlusion.

Symptoms of vascular dementia include the following:

Memory impairment

Impairment in at least 1 other cognitive domain (eg, orientation, language, praxis, executive functions,

visuospatial abilities)

Worsening of cognitive abnormalities

Impact on activities of daily living

In a clinical setting, differences between the cognitive disturbances in vascular dementia and Alzheimer disease are

of limited value in distinguishing the 2 conditions. Patients with dementia and vascular disease frequently have mixed

pathology (ie, both Alzheimer disease and vascular dementia; mixed dementia).[1, 2]

Vascular dementia may have less significant memory dysfunction than Alzheimer disease.[3]

It is also thought that

frontal dysfunction due to widespread involvement of subcortical structures in vascular dementia may lead to a

dysexecutive syndrome with abulia and apathy. In contrast, a cognitively impaired patient with vascular risks factors

but no history of cerebrovascular disease is most likely to have Alzheimer disease.

See Clinical Presentationfor more detail.

Diagnosis

The diagnosis of vascular dementia is usually made on the basis of clinical, neuroimaging, or neuropathologic

evidence of cerebral ischemia in the presence of progressive cognitive decline.

Examination for vascular dementia includes the following:

Evaluation of the temporal arteries: Decreased pulsatility, local tenderness, and thickening associated with

giant cell arteritis may be noted

[4]

Funduscopic evaluation: End-organ effects of hypertension and diabetes mellitus may provide important

information

Cardiac auscultation: Cardiac rhythmic and valvular abnormalities may be detected

Neurologic assessment: Spasticity, hemiparesis, visual field defects, pseudobulbar palsy, and extrapyramidal

signs confirm focal pathology

Cognitive assessment with a standardized tool (eg, Mini Mental Status Examination, Short Blessed

questionnaire): Low scores may provide corroborative evidence of a cognitive disturbance

Testing

All patients with dementia should have laboratory testing to rule out reversible causes of dementia. The fo llowing

studies may be useful for identifying or excluding other disorders:

Complete blood count (CBC)

Electrolyte levels

Thyroid-stimulating hormone (TSH) levels

Folate and vitamin B-12 levels

The American Academy of Neurology no longer recommends syphilis screening in the routine evaluation of dementia

if patients come from geographic regions with a very low base rate of syphilis. In specific cases, screening for

syphilis is indicated.

If the clinician has reason to suspect an angiitis affecting cerebral vessels, then an erythrocyte sedimentation rate

(ESR) and specific panels may be ordered.

Imaging studies

In patients with newly diagnosed dementia, obtain neuroimaging studies (ie, CT scanning or MRI of the head) to rule

out treatable causes of dementia and to aid in the differential diagnosis. The following findings may be observed:

Vascular dementia: Multiple cortical, and more commonly subcortical, infarcts or single strokes affecting the

thalamus, angular gyrus, and the territory supplied by the anterior cerebral arteries

Dementia: Decreased white-matter density on CT scanning or decreased T1 or increased T2 signal

intensities on MRI; multiple pathologies, including small vessel disease and decreased integrity of the

blood-brain barrier, have been associated with these findings

See Workupfor more detail.

Management

Nonpharmacologic strategies may help with behavior problems in patients with vascular dementia.[5, 6]

No approved

pharmacologic treatment exists for vascular dementia, so pharmacologic therapy is directed toward risk factors or

symptoms.

rological Manifestations of Vascular Dementia http://emedicine.medscape.com/article/1135408-overview

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Treat patients with risk factors for cerebrovascular disease. The individual approach combines a vascular risk factor

modification and various therapies addressing the specific subtypes of stroke (eg, antiplatelet drugs to prevent

cerebral infarction in large and small artery diseases of the brain, carotid endarterectomy or stenting for tight carotid

artery stenosis, and oral anticoagulants to prevent cardiac emboli).[7]

Administer antiplatelet agents when indicated, depending on the nature of the patient's underlying vascular

pathology. Management of vascular disease and dementia in a young patient with suspected uncommon causes of

stroke (eg, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]

or angiitis) involves ruling out these conditions with the appropriate testing procedures (ie, skin biopsy, cerebral

angiography). The decision to use anticoagulation therapy in these patients may be challenging due to the increased

risk of falls and potential noncompliance in this group.

Manage behavioral and psychiatric disturbances such as depression and psychosis with serotonin reuptake

inhibitors. In patients with agitation, environmental modification and/or pharmacologic intervention with sedation canbe useful.

There is limited evidence that cholinesterase inhibitors (eg, donepezil, rivastigmine, galantamine) may have a role in

the treatment of vascular dementia. These agents have proven symptomatic efficacy in Alzheimer disease, and their

use in vascular dementia may have some justification given the prevalence of dementia with mixed pathology.

Although lipid-lowering agents given in late l ife have not demonstrated a reduction in the risk of cognitive decline and

dementia, women who are treatment-resistant for high levels of low-density lipoprotein (LDL) cholesterol may be at

increased risk of decline in visual memory.[8]

See Treatmentand Medication for more detail.

Background

Dementia is a common neurologic syndrome with significant impact on the mortality and morbidity of elderly persons

with the most common forms being Alzheimer disease and vascular dementia. Vascular dementia is a

heterogeneous entity with a large clinicopathological spectrum that has been classically linked to cortical and

subcortical ischemic changes resulting from systemic, cardiac, or local large- or small-vessel disease occlusion.

Thus, the diagnosis of vascular dementia is usually made on the basis of clinical, neuroimaging, or neuropathological

evidence of cerebral ischemia in the presence of progressive cognitive decline. On the other hand, vascular

pathology often coexists with Alzheimer disease[1, 2]

, and this poses an additional diagnostic challenge. This has led

to the existence of the diagnostic term of mixed dementia.

This diagnosis is made in the presence of neuropathologic hallmarks of Alzheimer disease such as accumulation of

extracellular amyloid plaques, intracellular neurofibrillary tangles, and cerebral amyloid angiopathyas well as

evidence of significant ischemic events. Significant associations between both, deep white matter and periventricular

hyperintensities, and focal atrophy of medial temporal lobe structures have been described. These findings might

indicate that not only vascular factors alone but also degenerative factors favor the occurrence of white matter

hyperintensities after the age of 75 years.[9]

The frequent coexistence of Alzheimer disease and vascular dementia pathologies in postmortem studies has led

many to suggest that these 2 entities are mechanistically related. Further evidence for this comes from the significant

overlap in risk factors for Alzheimer disease and vascular disease such as hypertension, diabetes, and apoE4

genotype. Furthermore, cerebral hypoperfusion as detected by positron emission tomography (PET) has been

demonstrated in early stages of Alzheimer disease. Also cerebral amyloid angiopathy, which is prevalent inAlzheimer disease brains, could further alter cerebral hemodynamics. Despi te these observations, the mechanisms

of vascular-Alzheimer disease interactions are poorly understood, and the question remains as to whether these two

entities interact in a synergistic fashion.

Pathophysiology

Vascular dementia results from brain injury caused by stroke and cerebral ischemia.

Single ischemic or thromboembolic infarcts occurring in strategic areas of the dominant hemisphere (eg, angular

gyri, mediodorsal thalamus, anterior thalamus) may cause a dementia-like syndrome without the involvement of

large volumes of cerebral matter. In general, volume of tissue loss is a poor predictor of the severity of the cognitive

impairment.

More commonly, progressive cognitive deficits and dementia can result from multiple temporally staggered small

cerebral infarcts. Frontal subcortical regions supplied by small penetrating arterioles may be especially prone to

degenerative changes in patients with poorly controlled hypertension, diabetes mellitus, or both.

A less common cause of vascular dementia is global hypoxic-ischemic injury (eg, following cardiac arrest).

Irreversible cognitive impairment is frequently observed following coronary bypass surgery.[10]

Whether chronic cerebral ischemia associated with carotid artery stenosis (CAS) may alter cognitive function has not

been conclusively demonstrated and remains a controversial concept. Neuropsychometric evaluation of patients

undergoing carotid endarterectomy has not conclusively shown cognitive impairment or reductions in the probability

of developing dementia in the long term.

An ill-understood form of vascular dementia is Binswanger encephalopathy. Postmortem, myelin loss is observed

and is most prominent in the hemispheric deep white matter. Axonal drop out is also observed with little or no signs

of inflammation. Neuroimaging shows decreased white matter density on CT scanning and decreased white matter

intensity on T1-weighed MRI. Frequently, but not invariably, lacunar strokes are also observed.

Dementia associated with cerebrovascular disease is also observed in a rare genetic condition, ie, cerebral

autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).[11]

Affected patients

often present with migraines with aura. Recurrent strokes start when the patients are aged 30-50 years. Multiple

lacunar infarcts, mainly in the frontal white matter and basal ganglia, lead to progressive cognitive decline and finally

dementia. However, cognitive decline is thought to begin even before strokes occur, suggesting that chronic cerebral

hypoperfusion in the absence of overt stroke might be sufficient to cause significant neuronal circuit disruption.

Lastly, cognitive decline has been reported in association with several other vasculopathies such as temporal

arteritis, polyarteritis nodosa, primary cerebral angiopathy, lupus erythematosus, and moyamoya disease.

Qian et al looked into early biomarkers for poststroke cognitive impairment. They revealed that only -secretase

enzyme and soluble receptor levels for advanced glycation end products, not neprilysin or APOE genotypes, may be


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biomarkers diagnosing poststroke cognitive impairment.[12]

Epidemiology

Frequency

United States

The overall incidence of vascular cognitive impairment or vascular dementia ranges between 10% and 40% with

most accepted figures around 20%. This variability is likely to be due to uneven diagnostic criteria used in different

studies. Furthermore, the diagnosis requires clinical, neuroimaging, or neuropathological evidence of ischemic

events. This may lead to an underestimation of the role of microvascular occlusion and chronic hypoperfusion, which

are difficult to detect in routine neuropathological examination. Therefore, the incidence of vascular cognitiveimpairment may be higher than currently thought.

The incidence of dementia associated with acute stroke may be high, with 10-35% of patients developing dementia

within 5 years following a hemispheric stroke. Patients with symptomatic hemispheric strokes have an approximate

4-fold increase in the risk of dementia compared with age-matched controls.

International

Incidence of vascular dementia in Southeast Asia may be greater than in Western countries because of a higher

incidence of cerebrovascular disease in that part of the world. For example, in Japan, 50% of cases of dementia are

thought to have a vascular etiology. However, geographic differences may reflect diagnostic biases rather than true

epidemiologic differences.

Mortality/Morbidity

Median survival depends on whether cognitive decline follows a single large hemispheric stroke or instead is the

result of slowly progressive cognitive decline resulting from microvascular pathology. The progression of vascular

cognitive impairment is highly variable. In general, when vascular dementia occurs shortly after large hemispheric

strokes, the mortality is relatively high (around 4 y).[13]

Race

In the United States, individuals of African descent have a higher incidence of dementia than whites. Vascular

dementia may be the most common type of dementia affecting blacks.

Sex

Incidence of vascular dementia is higher in males than in females. The converse is true for Alzheimer disease. This

difference probably reflects known sex differences in the incidence of cerebrovascular disease.

Age

The incidences of vascular dementia and Alzheimer disease increase similarly with age.

Contributor Information and DisclosuresAuthorJasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Associate Professor of

Neurology, Loyola University Medical Center

Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology,

American Associa tion of Neuromuscular and Electrodiagnostic Medicine,American Clinical Neurophysiology

Society, andAmerican Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jaime Grutzendler, MD Assistant Professor, Department of Neurology and Physiology, Northwestern University

School of Medicine

Jaime Grutzendler, MD is a member of the following medical societies: American Academy of Neurologyand

Society for Neuroscience


Giovanni d'Avossa MD, Lecturer, School of Psychology, Bangor University; NHS Honorary Consultant in

Neurology


Fredy J Revill a, MD Associate Professor of Neurology, Director, Movement Disorders Center, James J and Joan

A Gardner Family Center Endowed Chai r for Parkinson's Disease and Other Movement Disorders, University of

Cincinnati College of Medicine; Staff Physician/Neurologist, Cincinnati Veterans Affairs Medical Center; Staff

Physician/Neurologist, Huntington's Disease Clinic, Cincinnati Children's Hospital Medical Center

Fredy J Revilla, MD is a member of the following medical societies:American Academy of Neurology, Movement

Disorders Society, and Society for Neuroscience


Specialty Editor Board

Christopher Luzzio, MD Clinical Assistant Professor, Department of Neurology, University of Wisconsin atMadison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology


Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College


5 10/7/2014 11:27 AM


4/5

of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge C Kattah, MD Head, Associate Program Director, Professor, Department of Neurology, University of

Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies:American Academy of Neurology,American

Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and

Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology,

American Academy of Sleep Medicine,American Clinical Neurophysiology Society,American Epilepsy Society,

andAmerican Medical Associat ion

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics

Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Sleepmed/DigiTrace

Honoraria Speaking, consulting; Sunovion Consulting fee None

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA Professor of Neurology, University of Central Florida

College of Medicine; Director of Cognitive Neurology, Director of Stroke Program, James A Haley Veterans Affairs

Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies:

American Academy of Neurology,American Headache Society,American Heart Association, andAmerican

Society of Neuroimaging


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