Dementias specific diseases

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MULTI-INFARCT (arteriosclerotic dementia)

This is an overdiagnosed condition which accounts for less than 10% of cases of dementia. Dementia occurs 'stroke by stroke', with progressive focal loss of function. Clinical features of stroke profile - hypertension, diabetes, etc. - are present. Diagnosis is obtained from the history and confirmed by CT scan.

Low density areas of infarction

These areas are not space-occupying and do not enhance after intravenous contrast

Treatment: Maintain adequate blood pressure control. Anti-platelet aggregants (aspirin).

PICK'S DISEASE

This progressive condition accounts for 5% of all dementias. Usually sporadic, it more commonly affects women between 40 and 60 years. Frontal lobe dysfunction predominates with apathy, lack of initiative and personality changes. CT scan shows frontal atrophy. Blood flow studies (SPECT (HMPAO)) reveal anterior hypoperfusion. The disorder is characterised pathologically by argyrophilic inclusion bodies within the cytoplasm of cells of the frontotemporal cortex. There is no treatment, death occurring within 2-3 years of the onset.

PRIMARY PROGRESSIVE DYSPHASIA

Dominant hemisphere perisylvian atrophy is associated with progressive disturbance of language which, after many years, develops into a generalised dementia. Pathologically non specific cell loss or spongiform changes distinguish this rare condition from Alzheimer's disease. CT scanning confirms focal atrophy.

AIDS DEMENTIA COMPLEX (see pages 495-496)

Approximately two-thirds of persons with AIDS develop dementia, mostly due to AIDS dementia complex. In some patients HIV is found in the CNS at postmortem. In others an immune mechanism or an unidentified pathogen is blamed. Dementia is initially of a 'subcortical' type.

CT shows atrophy; MRI shows increased T2 signal from white matter. Imaging excludes other infections and neoplastic causes of intellectual decline.

Treatment with Zidovudine (AZT) halts and partially reverses neuropsychological deficit.

METABOLIC DEMENTIA

General medical examination is important in suggesting underlying systemic disease. B12 deficiency may produce dementia rather than subacute combined degeneration of the spinal cord.

In alcoholics, consider not only Wernicke Korsakoff syndrome but also chronic subdural haematoma.

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