eMedicine Specialties > Neurology > Behavioral Neurology and Dementia
Vascular Dementia
Updated: Mar 5, 2009
Introduction
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 disease1,2 , 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 angiopathy as 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.3
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 in Alzheimer disease brains, could further alter cerebral hemodynamics. Despite 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.4
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).5 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.
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 cognitive impairment 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).6
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.
Clinical
History
Criteria for the diagnosis of dementia require impairment in memory and at least 1 other cognitive domain (eg, orientation, language, praxis, executive functions, visuospatial abilities). These should be serious enough to affect activities of daily living and be consistently present to distinguish dementia from episodic impairments of consciousness such as delirium.
- Currently, several sets of diagnostic criteria for vascular dementia exist. This list summarizes the main common criteria.
- Vascular risk factors such as hypertension, coronary disease, and diabetes mellitus7
- Specific evidence of cerebrovascular disease, eg, strokes and transient ischemic attacks: The cerebrovascular insult should precede (by no more than 3 mo) or coincide with the onset or worsening of cognitive abnormalities.
- Neuroimaging evidence of strokes
- Lateralizing neurologic signs
- Psychiatric disturbances (eg, emotional lability, depression, apathy)
- Depression is a common comorbidity in patients with cerebrovascular disease and vascular dementia.
- Medications should be reviewed because of the potential of drugs to interfere with alertness and cognition.
- Differences between the cognitive disturbances in vascular dementia and Alzheimer disease are of limited value in discriminating vascular dementia from Alzheimer disease in a clinical setting.
- Vascular dementia is thought to be associated with less significant memory dysfunction than Alzheimer disease.8
- Frontal dysfunction due to widespread involvement of subcortical structures in vascular dementia is thought to lead to a dysexecutive syndrome with abulia and apathy.
- A cognitively impaired patient with vascular risks factors but no history of cerebrovascular disease is most likely to have Alzheimer disease. Patients with dementia and vascular disease frequently have mixed pathology (ie, both Alzheimer disease and vascular dementia).
Physical
The physical examination should be focused on the cardiovascular system and neurologic localizing signs.
- The temporal arteries may show decreased pulsatility, local tenderness, and thickening associated with giant cell arteritis.9
- Funduscopic examination provides important information regarding end-organ effects of hypertension and diabetes mellitus.
- Cardiac auscultation may detect rhythmic and valvular abnormalities.
- Low scores on a standardized instrument (eg, Mini Mental Status Examination, Short Blessed questionnaire) can provide corroborating evidence of a cognitive disturbance.
- Spasticity, hemiparesis, visual field defects, pseudobulbar palsy, and extrapyramidal signs confirm focal pathology.
Causes
Vascular dementia and cerebrovascular disease share risk factors, including age, male sex, diabetes mellitus, hypertension, cardiomyopathy, and possibly homocysteine levels.
- So far, no relationship between cholesterol, serum lipoproteins, and the risk of vascular dementia is clearly indicated.
- Evidence for tobacco consumption as a risk factor for vascular dementia is conflicting.
- Limited alcohol consumption may be protective.10
Differential Diagnoses
Alzheimer Disease
Cortical Basal Ganglionic Degeneration
Dementia in Motor Neuron Disease
Dementia With Lewy Bodies
Frontal and Temporal Lobe Dementia
Workup
Laboratory Studies
- All patients with dementia should have laboratory testing to rule out reversible causes of dementia.
- CBC, electrolytes, thyroid-stimulating hormone (TSH), folate, and vitamin B-12 levels should be obtained. 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
- Patients with newly diagnosed dementia undergo neuroimaging studies to rule out treatable causes of dementia and to aid the differential diagnosis.
- Either CT scanning or MRI of the head should be performed.
- In patients with 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 are observed.
- Decreased white matter density (on CT scanning) or decreased T1 or increased T2 signal intensities (on MRI) are associated with dementia. Multiple pathologies, including small vessel disease and decreased integrity of the blood-brain barrier, have been associated with these findings.
- Measurement of cerebral blood flow using single photon emission computerized tomography (SPECT) or positron emission tomography (PET) scanning is not recommended for diagnostic purposes.
Treatment
Medical Care
The treatment of vascular dementia is symptomatic. Behavioral and psychiatric disturbances such as agitation, depression, and psychosis are common. Cerebrovascular disease should be treated by an internist and/or a neurologist familiar with the management of cerebrovascular disease.
- Established protocols for the evaluation and treatment of stroke are available. The presence of a rapidly progressive dementia and multiple strokes in a young patient may indicate uncommon causes of stroke such as CADASIL or angiitis. If suspected on clinical grounds, these conditions should be excluded with the appropriate testing procedures (ie, skin biopsy, cerebral angiography). The decision to use anticoagulation in patients with vascular disease and dementia is particularly challenging because of the increased risk of falls and potential noncompliance in this group.
- Patients with vascular dementia are prominently affected by depression and emotional incontinence. Both conditions respond well to treatment with serotonin reuptake inhibitors.
- Patients with agitation may respond to environmental modification. Pharmacologic treatment can be useful in controlling agitation through sedation.
Surgical Care
No surgical intervention is established for treatment of vascular dementia.
Medication
No approved pharmacologic treatment exists for vascular dementia.
Risk factors for cerebrovascular disease should be treated when present.
Retrospective data indicate that normotensive patients with vascular dementia may have greater cognitive decline than hypertensive patients. This finding, as well as the known loss of cerebral blood flow autoregulation in chronic hypertension and cerebrovascular disease, should temper overenthusiastic attempts to normalize the blood pressure in patients with vascular dementia.
Treatment with antiplatelet agents may be initiated as indicated by the nature of the patient's underlying vascular pathology.
Cholinesterase inhibitors, which include donepezil, rivastigmine, and galantamine, have proven symptomatic efficacy in Alzheimer disease and may also have a role in the treatment of vascular dementia according to the findings of limited studies. Presently, their use may have some justification given the prevalence of dementia with mixed pathology.
Follow-up
Prognosis
The rate of progression of cognitive impairment in vascular dementia is variable; some patients progress at a slower rate than patients with Alzheimer disease. The mortality rate, however, is higher in patients with vascular dementia than in patients with Alzheimer disease, with 50% of patients with vascular dementia not living longer than 4 years.
Patient Education
- Once the diagnosis of dementia is established, patients and patients' families should be educated about the nature of the condition with great sensitivity. The clinician should balance his or her explanation to avoid provoking negative reactions from the patient or the family.
- Potentially dangerous activities (eg, driving) should be discussed; provide advice regarding possible interventions (eg, driving evaluation).
- The patient should be advised about electing a power of attorney in case the patient becomes incapacitated.
- Caregivers require support as well. Provide contact information to caregivers for the local chapter of the Alzheimer's Association, which organizes support groups for caregivers of individuals with dementia.
- For excellent patient education resources, visit eMedicine's Dementia Center and Stroke Center. Also, see eMedicine's patient education articles Dementia Overview, Dementia Medication Overview, Alzheimer Disease, Stroke, and Stroke-Related Dementia.
Miscellaneous
Medicolegal Pitfalls
The patient and the patient's family should be advised regarding potentially hazardous daily activities such as driving. Evaluation by an occupational therapist may be indicated to determine the risk level.
References
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Keywords
vascular dementia, multi-infarct dementia, cerebrovascular dementia, dementia treatment, dementia symptoms, dementia care, Alzheimer's disease, Alzheimer's dementia, dementia in stroke, vascular cognitive impairment, leukoaraiosis, Binswanger encephalopathy, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL, Alzheimer disease, AD, VCI, MID
Contributor Information and Disclosures
Author
Jasvinder Chawla, MBBS, MD, MBA, Associate Professor of Neurology, Director of Neurology Residency Training Program, Director of Clinical Neurophysiology Laboratory, Assistant Director of Neurology Clerkship Program, Department of Neurology, Loyola University Medical Center
Jasvinder Chawla, MBBS, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, and American 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 Neurology and Society for Neuroscience
Disclosure: Nothing to disclose.
Giovanni d'Avossa, MD, Clinical Fellow, Department of Neurology, Washington University School of Medicine at Saint Louis
Disclosure: Nothing to disclose.
Fredy J Revilla, MD, Assistant Professor of Neurology, Head of Division of Movement Disorders, Department of Neurology, University of Cincinnati College of Medicine, Cincinnati Veterans Affairs Medical Center
Fredy J Revilla, MD is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society
Disclosure: Nothing to disclose.
Medical Editor
Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
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Jorge Kattah, MD, Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria
Jorge 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
CME Editor
Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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, and American Medical Association
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Chief Editor
Howard A Crystal, MD, Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center
Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association
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