Section I Introduction

Dementia, a syndrome with many causes, affects >4 million Americans and results in a
total health care cost of >$100 billion annually. It is defined as an acquired deterioration in
cognitive abilities that impairs the successful performance of activities of daily living. Memory is
the most common cognitive ability lost with dementia; 10% of persons >70 and 20–40% of
individuals >85 have clinically identifi able memory loss. In addition to memory, other mental
faculties may be affected; these include language, visuospatial ability, calculation, judgment, and
problem solving. Neuropsychiatric and social deficits also arise in many dementia syndromes,
resulting in depression, apathy, hallucinations, delusions, agitation, insomnia, and disinhibition.
The most common forms of dementia are progressive, but some are static and unchanging or fl
uctuate from day to day or even minute to minute. Most patients with Alzheimer’s disease (AD),
the most prevalent form of dementia, begin with memory impairment, although in other
dementias, such as frontotemporal dementia, memory loss is not a presenting feature.1
Dementia is an acquired, generalized, and usually progressive impairment of cognitive
function. Dementia differs from other disorders of cognitive function, such as coma or
confusional states, in that the level of consciousness (wakefulness or arousal) is preserved in
dementia. Although the prevalence of dementia increases with advancing age (Figure 5-1),
dementia is not an invariable consequence of aging and results instead from diseases involving
the cerebral cortex, its subcortical connections, or both. Normal aging may be associated with
minor alterations in neurologic function (Table 5-1) and with neuroanatomic changes, such as
enlargement of cerebral ventricles and cortical sulci seen on computed tomography (CT) or
magnetic resonance imaging (MRI) scans (Figure 5-2). However, these are not indicative of
dementia. The term mild cognitive impairment (MCI) is sometimes used to describe deficits
that are more severe than are customarily seen with normal aging but are insufficiently
pronounced to warrant a diagnosis of dementia. Nevertheless, patients with MCI have an
increased risk (approximately 10% per year) of developing dementia.2
 In contrast to dementia, which affects multiple spheres of cognitive function, more
limited disorders of cognition may also occur. These include deficits in language function
(aphasia) or motor (apraxia) or sensory integration. Memory disturbance (amnestic disorder or
amnesia, memory may also be impaired in normal aging and in dementia, but in the former
impairment is mild, and in the latter it is accompanied by defects in, for example, reasoning,
judgment, behavior, or language. Some causes of dementia, notably Alzheimer disease, produce
early and disproportionate impairment of memory and, at least in the early stages of disease, may
be difficult to distinguish from a pure amnestic disorder.2 Vascular disease is considered the
second most frequent cause for dementia and is particularly common in elderly patients or
populations with limited access to medical care, where vascular risk factors are undertreated. 2

The first step in evaluating a patient with cognitive impairment of any kind is to
determine the nature of the problem, which should be classified as affecting the level of
consciousness (confusional state or coma) or the content of consciousness. Table 5-2 lists key
differences that may be useful in making this distinction. If the content of consciousness is
impaired, a global cognitive disorder (dementia) should then be distinguished from a more
circumscribed deficit, such as amnesia or aphasia. This distinction is important because
classification of the disorder determines the diagnostic approach.2
 The final step in the diagnosis of dementia or an amnestic syndrome is to identify the
specific cause. Although the greatest emphasis should be on finding a treatable cause, even
untreatable causes can be important to identify. At present, only approximately 10% of
dementias are reversible, but the extent to which the quality and duration of life can be improved
in these cases justifies the effort and expense required to detect them. Diagnosis may be
important in untreatable disorders as well, to provide the patient and family with prognostic
information or genetic counseling, or to alert family members and medical personnel to the risk
of a transmissible disease.2
Less Common Causes of Dementia
Clinical Features Helpful in the Differential Diagnosis of Dementia
 Section II Literature Review
Vascular Dementia

Definition
Vascular dementia (VaD) is considered by some to be the second most common
dementing illness after Alzheimer’s disease (AD). VaD comprises a group of mixed dementing
disorders due to cerebrovascular insufficiency. Consequently, these disorders, may be the result
of brain damage from multiple strokes, or infarcts, caused by small clots from heart or neck
arteries that clog a branch of a blood vessel in the brain. A stroke means that the blocked blood
vessel has deprived an area of brain tissue of essential oxygen and nutrients. The relationship
between Alzheimer's disease and vascular dementia is complex, however, recent evidence
suggests that small strokes may lead to increased clinical presence of Alzheimer's disease.

Incidence
The incidence of vascular dementia is slightly higher among men than among women and
appears typically in those between 60 to 75 years of age, however the prevalence of VaD rises
steeply with age. In United States, VaD may disproportionately affect African-Americans and in
Japan and other Asian countries, VaD may be even more common than AD. Preventable risk
factors are high blood pressure (hypertension), heart disease and diabetes mellitus.

Classification
Vascular disease is generally considered the second most common cause of dementia,
after Alzheimer disease, and many patients have features of both diseases.2 Dementia associated
with cerebrovascular disease can be divided into two general categories: multi-infarct dementia
and diffuse white matter disease (also called leukoaraiosis, subcortical arteriosclerotic
leukoencephalopathy, or Binswanger’s disease)..1
Patients with this diagnosis may have multiple large (>1 cm in diameter) cortical infarcts;
strategic infarcts involving hippocampus or thalamus; multiple small (eg, lacunar) infarcts
affecting subcortical white matter, basal ganglia, or thalamus; diffuse ischemic lesions of
subcortical white matter (Binswanger disease); intracerebral hemorrhages (eg, cerebral
amyloid angiopathy); or combinations of these.2
 Although vascular dementia is usually sporadic, genetic causes are also recognized.
These include autosomal dominant cerebral amyloid angiopathy (usually due to mutations in the
gene for amyloid precursor protein) and cerebral arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL, due to mutations in NOTCH3).2

Clinical Findings
Individuals who have had several strokes may develop chronic cognitive deficits,
commonly called multi-infarct dementia. The strokes may be large or small (sometimes lacunar)
and usually involve several different brain regions. The occurrence of dementia depends partly
on the total volume of damaged cortex, but it is also more common in individuals with left-
hemisphere lesions, independent of any language disturbance. Patients typically report previous
discrete episodes of sudden neurologic deterioration. Many patients with multi-infarct dementia
have a history of hypertension, diabetes, coronary artery disease, or other manifestations of
widespread atherosclerosis. Physical examination usually shows focal neurologic deficits such as
hemiparesis, a unilateral Babinski sign, a visual field defect, or pseudobulbar palsy with
dysarthria, dysphagia, and pathologic nmotionality (pseudobulbar affect); focal motor and
sensory deficits; ataxia; gait apraxia; hyperreflexia; and extensor plantar responses.. Recurrent
strokes result in a stepwise disease progression.1,2 Neuroimaging reveals multiple areas of
infarction. Thus, the history and neuroimaging findings differentiate this condition from AD;
however, both AD and multiple infarctions are common and sometimes co-occur.1

Cognitive and Neuropsychiatric Examination

Brief screening tools such as the minimental status examination (MMSE) help to confirm
the presence of cognitive impairment and to follow the progression of dementia. The MMSE, a
simple 30-point test of cognitive function, contains tests of orientation, working memory (e.g.,
spell world backwards), episodic memory (orientation and 3-word recall), language
comprehension, naming, and figure copying. In most patients with MCI and some with clinically
apparent AD, the MMSE may be normal and a more rigorous set of neuropsychological tests will
be required. When the etiology for the dementia syndrome remains in doubt, a specially tailored
evaluation should be performed that includes tasks of working and episodic memory, executive
function, language, and visuospatial and perceptual abilities. In AD the early deficits involve
episodic memory, category generation (“name as many animals as you can in one minute”), and
visuoconstructive ability.
Usually deficits in verbal or visual episodic memory are the first neuropsychological
abnormalities detected, and tasks that require the patient to recall a long list of words or a series
of pictures after a predetermined delay will demonstrate deficits in most patients. In FTD, the
earliest deficits on cognitive testing involve executive or language (speech or naming) function.
DLB patients have more severe deficits in visuospatial function but do better on episodic
memory tasks than patients with AD. Patients with vascular dementia often demonstrate a
mixture of executive and visuospatial deficits, with prominent psychomotor slowing. In delirium,
the most prominent deficits involve attention, working memory, and executive function, making
the assessment of other cognitive domains challenging and often uninformative.1
Disease Course l_
l_ step-wise
A person with vascular dementia may often experience a more abrupt loss of intellectual
skills as compared to a gradual loss in Alzheimer's disease. The course of vascular dementia
frequently progresses in "steps", with the person's abilities remaining steady for a period of time
and then declining rapidly. The reason for this variable and "step-wise" course is the underlying
cause of the vascular dementia, the strokes. On average, individuals with vascular dementia live
several years less after diagnosis than those with Alzheimer's disease--the cause of death often
being a heart attack or major stroke.

Investigative Studies
The MRI (Figure 5-10) may show multiple large infarcts, multiple small (lacunar)
infarcts, areas of low density in subcortical white matter, or combinations of these findings and is
more sensitive than CT scan for detecting these abnormalities.2 Termed diffuse white matter
disease, often occurring in association with lacunar infarctions (Third Figure). The dementia
may be insidious in onset and progress slowly, features that distinguish it from multi-infarct
dementia, but other patients show a stepwise deterioration more typical of multi-infarct
dementia.1 Additional laboratory studies should be performed to exclude cardiac emboli,
polycythemia, thrombocytosis, cerebral vasculitis, and meningovascular syphilis as causes of
multiple infarctions, particularly in younger patients or those without a history of hypertension.
Diagnosis
As with most dementing illnesses, a definite diagnosis is possible only on autopsy with
examination of the brain tissue. However, a probable diagnosis is determined by:
 Dementia, impairment of memory and 2 or more cognitive domains
 Cerebrovascular disease: with focal signs on neurological examination (i.e., hemiparesis,
lower facial weakness)
 Evidence of relevant cerebrovascular disease by brain scan imaging (CT or MRI scan)
 Probable association results when there is a temporal (time) relationship between
cognitive deficits and cerebrovascular lesions, as an example, abrupt or stepwise
deterioration

Treatment

Vascular dementia is neither reversible nor curable, but treatment of underlying

conditions, such as high blood pressure, diabetes mellitus, may prevent further progression of the
disorder. Treatment of any associated symptoms of depression is also recommended. As with
other dementing illnesses, families must ensure the safety and well-being of the affected
individual and also adapt to the person’s changing cognitive status. The individual will
eventually require full time supervision and care, so it is essential that the caregiver and family
members seek support along the way. The Alzheimer's Association has many programs to assist
families with education, emotional support and care planning throughout the course of a
dementing illness. There are formal support services such as home care or adult day programs,
and informal support such as friends, neighbors or church members all supports essential for
caregiver survival during very challenging caregiving experience.
 Section III Bibliography

1. Hauser S L. Harrison’s, Neurology And Clinical Medicine. 3rd rev. ed. United States:
McGraw-Hill, 2013. 310-32p

2. Aminoff M J, Greenberg D A, Simon R P. Lange, Clinical Neurology. 9th rev. ed. United
States: McGraw-Hill, 2015. 105-33p