Evaluation of Dementia

David Lu , MD Washington VAMC

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Dementia
a growing medical and social problem occurring at all ages but most frequent in the population over age 75 an estimated 600,000 cases of advanced dementia in the United States, and milder degrees of

altered mental status are very common

long-term care cost estimated at $40 billion a year for people age 65 and older
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National Institute of Aging Consensus

Issues addressed
definition diagnosis reversible dementing diseases initial evaluation indicated diagnostic tests priorities of future research
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Definition
a syndrome characterized by progressive decline of intellectual ability from a previously attained level the decline in mental inability usually involves variable deterioration in speech memory judgment mood without alteration of consciousness

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Clinical Presentation
onset of dementia it is usually insidious dementia is often progressive (degenerative disease) but may be static (post-traumatic brain injury) initial presentation may include slight forgetfulness, attention and concentration deficits, and increasing repetitiousness or inconsistencies in usual behavior later presentation may display impaired judgment, inability to abstract or generalized, and personality change with rigidity, perseveration, irritability, and confusion; affective disturbances may be prominent with loss of personality and self-care
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Neurologic Diseases Associated with Intellectual Dysfunction

DISEASE
Alzheimer's disease

PHYSICAL SIGNS
Frontal lobe release signs; extrapyramidal signs Gait disorder , incontinence

CLINICAL FEATURES
Enlarged ventricles and cortical atrophy by CT or MRI Enlarged ventricles with little or no cortical atrophy Stepwise course; multiple areas of infarction, often subcortical by CT or MRI Usual present only after disease evident for several years Often subacute evolution, seizures possible Positive serology serum and CSF Positive HIV, cortical atrophy; dementia may be presenting symptom

Normal pressure hydrocephalus Multi-infarct dementia Focal deficits Parkinson's disease Intracranial tumor Neurosyphilis HIV infection

Extrapyramidal signs Focal signs, papilledema Frontal lobe signs, optic atrophy, Argyll-Robertson pupils Variable systemic involvement

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Neurologic Diseases Associated with Intellectual Dysfunction

DISEASE
Creutzfeldt-Jakob Huntington's disease

PHYSICAL SIGNS
Myoclonus , cerebellar signs, eye movement abnormalities Choreiform movements, corticospinal signs Brainstem signs, optic atrophy, corticospinal signs Extrapyramidal signs , hepatic dysfunction, Kayser-Fleischer rings Failure of vertical downgaze, extrapyramidal signs

CLINICAL FEATURES
Subacute course; EEG has specific abnormalities, brain biopsy diagnostic Often positive family history; caudate atrophy by CT or MRI Usually long-standing disease; episodic illness with remissions; often extensive white matter abnormalities by MRI Onset in adolescence or young adult life, psychiatric disorders Eye movement abnormalities; differentiate from Parkinson's disease; unresponsive or only transiently responsive to levodopa

Multiple sclerosis

Wilson's disease

Progressive supranuclear palsy

* = invariably present; all other physical signs are neither invariably present nor pathognomonic. 6/00 medslides.com 7

Alzheimers Disease
one of the leading cause of dementia with unknown etiology >4% of people over 65 exhibit moderate to severe dementia and about 2/3 of these fall into the category of idiopathic senile dementia or Alzheimers disease most cases are sporadic, although there is a familial autosomal dominant form no specific physical signs - frontal lobe release signs and extrapyramidal features may be present

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Alzheimers Disease
brain atrophy with ventricular enlargement absent or minimal vascular disease neuropathologic studies
neuronal loss neurofibrillary tangles senile plaquesin accumulation of beta-amyloid

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Vascular Dementia
Multi-infarct dementia - multiple strokes can leave the patient with impaired cognition and produced a true dementia
small lacunar strokes may present subclinically large strokes with clear-cut neurologic injury

in the very elderly (>85), vascular dementia rivals Alzheimers disease as the leading etiology groups at high risk include
African-Americans, Japanese elderly patients with hypertension, diabetes, smoking, atrial fibrillation, or known carotid disease

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Normal-Pressure Hydrocephalus
refers to slow ventricular enlargement without cortical atrophy due to poor cerebrospinal fluid (CSF) absorption blockage of CSF absorption due to
remote meningeal inflammation subarachnoid hemorrhage

classic triad (wacky, wobbly, and wet)

dementia, gait disturbance, urinary and fecal incontinence

ventriculoperitoneal shunt may lead to dramatic clinical improvement -response to serial lumbar punctures may predict those who will respond
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Space Occupying Lesions

development of progressive unilateral headache, new neurologic deficit, or changing personality may provide a clue to the presence of a mass lesion chronic subdural hematoma slow-growing tumors
on the orbital surface of the frontal lobe on the medial surface of the temporal lobe

may present primarily with cognitive defects unassociated with other focal signs

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Other Neurologic Conditions

Depression Parkinsons disease Wilsons disease severe multiple sclerosis Jacobs disease neurosyphilis Huntingtons disease

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Systemic Conditions Associated with Intellectual Impairment

Infectious Syphilis with CNS involvement HIV infection with CNS involvement Cryptococcal infection of the CNS Endocrine Hypothyroidism and hyperthyroidism Panhypopituitarism High-dose glucocorticosteroid therapy Metabolic Vitamin B12 deficiency (Pernicious anemia) Thiamine deficiency (Korsakoffs) Niacin deficiency (pellagra) Chemical Poisons Alcohol Metals (lead, mercury) Aniline dyes Drug Intoxications Barbiturates Opiates Anticholinergics Lithium Bromides Haloperidol Antihypertensives

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Differential Diagnosis
Alzheimer's disease Multi-infarct dementia brain tumors unknown causes 70% 10% - 20% 5% 10-15%

Among the very old (over age 85), vascular dementia and Alzheimer's disease account for the vast majority
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Workup
History Physical Examination Laboratory studies

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History - etiology
the most important component of the initial evaluation adequate history with help of a family member is critical
description of
cognitive, memory, and behavior problems effect on daily life - difficulty with driving, work, or family relationships

details on temporal course of illness

chronic progressive (Alzheimer or other neurodegenerative disease) stepwise (multi-infarct) static (traumatic injury, episode of severe hypotension)

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History - treatable causes

Vascular dementia - presence of cardiovascular risk factors (smoking, HTN, chol, diabetes) Normal pressures hydrocephalus - triad of dementia, gait, incontinence with a prior history of meningitis or subarachnoid hemorrhage Mass lesion - history of head trauma, unexplained focal neurologic deficit, unilateral headache worsening over time Parkinsons disease - resting tremor and rigidity Wilsons disease - hepatocellular disease and dementia HIV and neurosyphilis - high-risk sexual behavior hereditary - family history dementia, Downs syndrome, psychiatric disorders
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History - treatable causes

B12 deficiency - previous gastric surgery B12, thiamin, niacin deficiency - inadequate nutrition, alcohol abuse medications - opiates, sedative-hypnotics, analgesics, anticholinergics, anticonvulsants, corticosteroids, centrally acting anti-hypertensives, psychotropics symptoms of hypothyroidism, pituitary insufficiency occupational history - exposure to toxic substances (aniline dyes, heavy metals)

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Mental Status Examination

Examination should be geared to both the detection of focal lesions and to signs of general brain dysfunction
immediate memory testing (three object recall, recite digits forward and backward, recall a short story) remote memory testing (recall of historical events, family milestones, or recent local or international news) reproducible drawings discern similarities among objects decision-requiring tasks (finding a stamped letter or seeing a fire in a theater)

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Mini-Mental Status Tests

Score 5 5

Orientation: What is the (year) (season) (month) (date) (day)? Whare are we (state) (county) (town) (hospital) (floor)? Registration: Name 3 objects: 1 second to say each. Then ask the patient all 3 after you have said them. Give 1 point for each correct answer. Then repeat them until he learns all 3. Count trials and record. Attention and Calculation: Serial 7's. 1 point for each correct. Stop after 5 answers. Alternatively spell "world" backwards.
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Mini-Mental Status Tests

Score 3 2 1 3 1 1 1

Recall: Ask for 3 objects repeated above. Give one point for each. Language: Name a pencil and watch (2 points). Repeat the following: "No ifs ands or buts." Follow a 3-stage command: "Take a paper in your right hand: fold it in half, and put it on the floor." (3 points). Read and obey the following: "Close your eyes." Write a sentence. Copy design. Total Score: [ ] Maximum Score: 30
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Physical and Neurologic Examinations

Check for focal evidence of neovascular risk factors - carotid bruits, signs of alcoholism, hepatocellular injury, renal insufficiency, other systemic illnesses specific neurologic abnormalities
frontal lobe release signs (grasp, suck, snout, root) visual field cut and extraocular movement limitations abnormal pupillary reactions extrapyramidal features (carditis dyskinesis, tumors, asterixis, Korea, monoclonal disc, it) sensory deficit and gait disorder

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Screening Laboratory Studies

1. Complete blood count and sedimentation rate 2. Chemistry panel (electrolytes, calcium, albumin, BUN, creatinine, transaminase) 3. Thyroid-stimulating hormone (TSH) 4. VDRL test for syphilis 5. Urinalysis 6. Serum B12 and folate levels 7. Chest x-ray 8. Electrocardiogram 9. Head computed tomography (CT)

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Neuroimaging
Head CT or MRI is appropriate in the presence of
1) 2) 3) 4) 5) history suggestive of a mass lesion focal neurologic signs or symptoms dementia of abrupt onset history of seizures history of stroke

MRI with gadolinium contrast enhancement is superior to CT for the diagnosis of multi-infarct dementia and problems referrable to the posterior fossa

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Other Ancillary Studies

Lumbar puncture
routine LP for initial evaluation of dementia is not justified may be indicated when other clinical findings suggest an active infection or vasculitis and as part of the evaluation of normal pressure hydrocephalus sugar, protein, cell count, cultures, gamma globulins, the serology for stiffness should be obtained

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Other Ancillary Studies

Electroencephalogram (EEG)
usually normal or with nonspecific rhythm slowing indicated in patients with episodic altered consciousness and in whom seizures may be suspected may occasionally raise suspicion of a particular etiology:
focal, delta slowing is seen with tumor unilateral attenuation of voltage may suggest an extracranial mass such as subdural hematoma excessive beta activity may be consistent with drug ingestion Creutzfeldt-Jakob disease has a highly specific EEG pattern
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Other Ancillary Studies

Formal neuropsychologic evaluation
appropriate for more specific information when the diagnosis is in doubt also helpful in providing additional information about the nature of impairment following focal brain injury

Speech analysis
may improve patient and family communication with therapy

Formal psychiatric assessment

may be desirable if depression in addition to dementia is suspected

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Studies of Limited or Uncertain Utility

Cerebral blood flow and metabolism measurements
PET and SPECT scans have no routine use at present

Brain biopsy
rarely justified for non-neoplastic or noninfectious diseases Progressive multifocal leukoencephalopathy or Creutzfeldt-Jakob disease is diagnosed by biopsy

Noninvasive neurovascular studies (carotid ultrasound, Doppler flow studies)

if MRI or CT demonstrates infarction, or clinical course or physical examinations is suggestive of cerebralvascular disease
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Symptomatic Management and Counseling

Improving mental functioning Management of confusion and agitation Maintaining the patient at home

Risk factor reduction and attention to underlying etiologies

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Improving Mental Functioning

no established treatment for Alzheimers disease or for patients with multi-infarct dementia findings of degeneration of cholinergic neurons and depletion of cholineacetyl transferase in Alzheimer's disease have led to attempts at improving cholinergic transmission lecithin supplements (dietary choline repletion) tacrine (a centrally active, reversible cholinesterase inhibitor) There is no evidence to support the use of: restorative therapy with nerve growth factor, protective therapy with antioxidants, preventive therapy with drugs that inhibit beta amyloid formation, and cerebral vasodilators (papaverine, dihydroergotoxine) to improve memory

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Management of Confusion and Agitation

The chronic use of sedatives and psychoactive agents in the confused patient should be avoided unless persistent extreme agitation hampers care The lowest possible doses should be used and for the shortest time possible
thioridazine (10 to 25 mg qhs) haloperidol (0.5 to 1 mg bid or tid ) often a first choice in the setting of delusions and hallucinations; must be careful to avoid long-term use because of the risk of inducing tardive dyskinesia

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Management of Confusion and Agitation

Avoid regular use of sedative/hypnotic agents for sleep Beta-blocking agents and anticholinergics may exacerbate confusion Patients with depression may improve with a tricyclic compound with low anticholinergic side effects desipramine (25 to 50 mg qhs) A recent study of nursing home patients demonstrated substantial improvement in many patients when chronically prescribed psychotropic drugs were discontinued or reduced in dose
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Maintaining the Patient at Home

An important task is helping the family maintain and care for the patient at home The goal is to sustain the highest level of function possible:
facilitate and promote an orderly home situation regular routine use of calendars, television, newspapers, and other means of orientation limit the use of potentially dangerous appliances provide convenient toilet facilities advice against driving when early impairment of judgment and spatial concepts is present

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Maintaining the Patient at Home

Families can often find help in local support groups, day care and group therapy services, and social service agencies When care at home begins to exhaust and strain the family, sensitive counseling can do much to help a family cope with the difficult decision regarding institutionalization some dementing diseases are infectious (eg, HIV infection) and that the bodily fluids and tissues of such patients require special handling to avoid transmission. It is particularly important to emphasize when home care is rendered by lay persons

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Risk factor reduction and attention to underlying etiologies

Central to an effective outcome :
control of cerebrovascular risk factors as hypertension, diabetes mellitus, smoking , hyperlipidemia , and coronary artery disease endarterectory deserves consideration when a vascular etiology is strongly suspected and a significant stenosis is found Avoidance of toxins, correction of vitamin deficiencies, discontinuation of causative drugs, initiation of hormonal replacement therapy in cases of deficiency, and treatment of underlying infectious etiologies

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References

Avorn J, Soumerai SB, Everitt DE, et al. A randomized trial of a program to reduce the use of psychoactive drugs in nursing homes. N Engl J Med 1992;327:168. Clarifield AM. The reversible dementias: Do they reverse? Ann Intern Med 1988;109:476. Consensus Conference. Differential diagnosis of dementing diseases. JAMA 1987;258:3411. Growdon JH. Treatment for Alzheimer's disease. N Engl J Med 1992;327:1306. ( Excellent summary of current approaches to therapy.) Jenkyn LR. Examining the aging nervous system. Semin Neurol 1989;9:82. ( Good overview of signs associated with normal aging.) Larson EB, Reiffler BV, Sumi SM, et al. Diagnostic tests in the evaluation of dementia. Arch Intern Med 1986;146:1917. Lindenbaum J, Healton EB, Savage DG, et al. Neuropsychiatric disorders caused by cobalamin deficiency in the absence of anemia or macrocytosis. N Engl J Med 1988;318:1720.

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References
Mace NL. The 36 hour day: A timely guide to caring for persons with Alzheimer's disease. Baltimore, Johns Hopkins Press, 1981. Petersen RC. Memory function in normal aging. Neurology 1992;42:396. Price RW, Brew BJ. The AIDS dementia complex. J Infect Dis 1988;158:1079 Siu AL. Screening for dementia and investigating its causes. Ann Intern Med 1991;115:122. Skoog I, Nilsson L, Palmertz B, et al. A population-based study of dementia in 85-yearolds. N Engl J Med 1993;328:153.

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