Vascular Dementia Clinical Presentation

Updated: Mar 26, 2018
  • Author: Kannayiram Alagiakrishnan, MD, MBBS, MPH, MHA; Chief Editor: Glen L Xiong, MD  more...
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Presentation

History

Cognitive impairment, acutely or subacutely, after an acute neurologic event with a stepwise progression is a typical history suggestive of vascular dementia. However, this classic history is usually observed with multi-infarct dementia and may not be observed with lacunar state. 

Binswanger disease

The average age of onset is between the fourth and seventh decades of life, and 80% of patients have a history of hypertension. Patients also show progressive motor, cognitive, mood, and behavioral changes over a period of 5-10 years. Mood and behavioral changes are observed early and, in some patients, may be the presenting feature. Patients may be apathetic or abulic.

Intellectual deficits are also observed early in the disease, and patients are frequently described as disoriented, having memory deficits, inattentive, and vague.

Patients with Binswanger dementia often have early-onset urinary incontinence and gait disturbances.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

The onset of the disease occurs between the third and fourth decades of life.

The clinical picture is similar to Binswanger disease but without a history of hypertension and risk factors for cerebrovascular disease.

Vascular dementia in general

Health professionals can perform a Mini-Mental Status Exam (MMSE), [12] depression assessment screen using DSM-5 criteria, [1] the Geriatric Depression Scale (GDS), [13] or the Cornell Scale for depression in dementia, [14]  Health professionals should directly ask patients about suicidal or homicidal ideation (thoughts), intent, and plan.

The mental status is a bedside or interview assessment and includes:

  • Appearance
  • Interactions with examiner
  • Mood
  • Affect
  • Pattern of speech
  • Thought process and associations
  • Thought content (common themes, ideas of reference, delusions)
  • Perceptual changes (illusions, hallucinations)
  • Level of arousal (Alertness)
  • Orientation
  • Attention/Concentration
  • Memory (Immediate/Short-term/Long-term) 
  • Suicidal ideation, intent, plan, preparation, and protective factors 
  • Homicidal ideation, intent, plan, preparation, and protective factors 
  • Judgment and insight

Major depression is widely observed mood disorder in vascular dementia. Severe depression is more common in persons with vascular dementia than in those with AD. Elderly demented patients may not endorse depressed mood and may be socially withdrawn with decreased psychomotor activity. Suicidal thoughts, intent, passive wishes to die and feeling that life is not worthy is seen in these patients and they should be followed closely. Suicide attempts were observed in fewer than 1% of patients with dementia, but those attempts are often associated with depression. [15]

Demented patients may develop psychosis, delusions, hallucinations and paranoia at some point in their disease and sometimes agitation can be dangerous when it manifests into abnormal behavior and in rare circumstances can lead to attempts of homicide.

Patients with vascular dementia commonly have mood and behavioral changes. In some patients with lacunar state and Binswanger disease, such problems may be more prominent than intellectual deficits.

Executive functioning deficits are seen prior to severe memory loss in the early stages of subcortical vascular cognitive impairment. [16]

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Physical

A commonly used cognitive screening tool is the Folstein Mini-Mental State Examination. Patchy defects are present in persons with vascular dementia. The deficits are global in persons with Alzheimer dementia.

The Folstein Mini-Mental State Examination is as follows:

  • Orientation: First, ask the patient the date, day, month, year, and season. The maximum score is 5. Second, ask the patient their current location, i.e., facility, floor, town, state, and country. The maximum score is 5.

  • Registration: Name 3 objects (e.g., ball, flag, door), and ask the patient to repeat them. The maximum score is 3.

  • Attention: Ask the patient to spell the word "world" backwards or to subtract 7 from 100 serially backwards (stop after 5 answers). The maximum score is 5.

  • Recall: Ask the patient to remember the 3 objects from the Registration portion of the test. The maximum score is 3.

  • Language

  • Ask the patient to identify a pencil and a watch. The maximum score is 2. Ask the patient to repeat the phrase "no ifs, ands, or buts." The maximum score is 1. Ask the patient to follow a 3-step command. The maximum score is 3. Ask the patient to read and obey the phrase "close your eyes." The maximum score is 1. Ask the patient to write a sentence. The maximum score is 1. Ask the patient to copy a set of interlocking pentagons. The maximum score is 1.

  • Scoring: The maximum score possible is 30. Generally, any score less than 24 is considered abnormal, but the cutoff varies with the patient's level of education. Because the results for this test can vary over time, and for some people results can vary during the day, record when (i.e., the time and date) this test was performed. [17]

Diagnostic criteria

DSM-5

Several specific diagnostic criteria can be used to diagnose vascular dementia, including the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) criteria, the International Classification of Diseases, Tenth Edition criteria, the National Institute of Neurological Disorders and Stroke-Association International pour la Recherché at L'Enseignement en Neurosciences (NINDS-AIREN) criteria, the Alzheimer's Disease Diagnostic and Treatment Center criteria, and the Hachinski ischemic score.

DSM-5 categorizes vascular dementia as an etiological subtype of either major or mild neurocognitive disorder. A summary of the DSM-5 diagnostic criteria is as follows: [1]

  • Evidence of modest (mild) or significant (major) cognitive decline from a previous level of performance in one or more cognitive domains (complex attention, executive function, learning and memory, language, perceptual-motor or social cognition based on: 1) Concern of the individual, a knowledgeable informant, or the clinician that there has been a decline in cognitive function and 2) An impairment in cognitive performance (modest or significant) documented by standardized testing or another qualified assessment.

  • The clinical features are consistent with a vascular etiology as suggested by either of the following: 1) Onset of the cognitive deficits is temporally related to one or more cerebrovascular events; or 2) Evidence for decline is prominent in complex attention (including processing speed) and frontal executive functions.

  • There is evidence of the presence of cerebrovascular disease from history, physical examination, and/or neuroimaging considered sufficient to account for the neurocognitive deficits.

  • The symptoms are not better explained by another brain disease or systemic disorder.

  • Probable vascular neurocognitive disorder is diagnosed if one of the following is present:1) Clinical criteria are supported by neuroimaging evidence of significant parenchymal injury attributed to cerebrovascular disease;2) The neurocognitive syndrome is temporarily related to one or more documented cerebrovascular events; 3)Both clinical and genetic evidence of cerebrovascular disease is present

  • Possible vascular neurocognitive disorder is diagnosed if the clinical criteria are met but neuroimaging is not available and the temporal relationship of the neurocognitive syndrome with one or more cerebrovascular events is not established.

NINDS-AIREN

The NINDS-AIREN criteria are the most specific of all available criteria and are used most commonly in research. They provide 3 levels of certainty: definite, probable, and possible.

Lateralizing signs such as hemiparesis, bradykinesia, hyperreflexia, extensor plantar reflexes, ataxia, pseudobulbar palsy, and gait and swallowing difficulties may be observed.

Subcortical vascular dementia signs include balance problems, gait disorder, and urinary incontinence; focal lesions may be subtle.

Neuropsychological testing is as follows:

Patients with vascular dementia have patchy neuropsychological deficits. With vascular dementia, patients have better free recall and fewer recall intrusions compared with patients with AD. Apathy early in the disease is more suggestive of vascular dementia because it usually occurs in the later stages of AD.

Patients with vascular dementia have poor verbal fluency and more perseverative behavior compared with patients with AD. They may even have other signs of executive dysfunction such as cognitive slowing, difficulty in shifting sets, and problems with abstraction. Commonly used mental status tests include the Folstein Mini-Mental State Examination and the Cognitive Abilities Screening Instrument.

Some cognitive patterns may help to differentiate vascular dementia clinically from AD. Patients with vascular dementia tend to show greater deficits on measures of frontal executive functioning than patients with AD, whereas patients with AD show greater long-term memory deficits than patients with vascular dementia.

Neuropsychological findings vary with the site and severity of cerebrovascular disease.

For patients with single or multiple large infarcts, deficits correlate with the site and extent of the infarct.

In patients with extensive deep white matter disease, impairments may be observed in tests of psychomotor speed, dexterity, executive function, and motor aspects of speech (e.g., dysarthria, reduced verbal output). Patients with subcortical vascular dementia show reduced ability to set and reach goals with mental slowing and gradual executive dysfunction.

Cerebral amyloid angiopathy may present with progressive cognitive impairment, transient ischemic attacks or amyloid spells and sudden focal neurological deficits related to intracerebral hemorrhage. Amyloid spells could be early clinical indicators of cerebral amyloid angiopathy. [18]

Behavioral problems assessment: Behavioral disturbances are common in dementia and are associated with adverse outcomes, increased disability, caregiver stress, and earlier institutionalization. Patients should be assessed for the following disturbances:

Agitation/aggression: Patient exhibits restlessness, physical agitation, or verbal or sexual aggression. Patient is hard to handle or resistant to care.

Hallucinations: Patient sees or hears things that are not there.

Delusions/paranoia: Patient harbors false beliefs, is suspicious of family members regarding stealing money or belongings, or suspects neighbors are planning to harm him.

Sundowning: Abnormal behaviors typically occur in the late afternoon or evening in a circadian rhythm fashion. Patients may exhibit mood swings, become upset or disoriented, or wander.

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Causes

Risk factors for vascular dementia include hypertension, smoking, hypercholesterolemia, diabetes mellitus, and cardiovascular and cerebrovascular disease.

A large cohort study published in 2010 followed 21,123 heavy midlife smokers (more than 2 packs per day) for a mean of 23 years. These individuals were found more than 2 decades later to have a greater than 100% increased risk of dementia, Alzheimer disease, and vascular dementia in both sexes and across ethnic groups. [19]

Vascular dementia development after stroke can be influenced by many factors. Some of the important factors that can lead to the development of dementia are older age, lower education level, family history of dementia, left-sided lesions, larger lesions, larger periventricular white matter ischemic lesions, and strokes in thalamic artery territory, inferomedian temporal lobes, hippocampus, and watershed infarcts involving superior frontal and parietal regions. [20]

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