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OTHER DEMENTIAS
Dementia
• 5. “Possible” refers to those who meet the criteria for dementia but
have other illness that may contribute, such as hypothyroidism or CVD
• 90% sensitivity for the diagnosis of probable or possible AD
• 60% specificity
• There are no specific changes in routine laboratory exam
• CSF: normal (there may be slight increase in protein)
• EEG: generalized slowing
• Neuropsychological testing can detect minimal or subtle
cognitive impairment early in the disease, can document
global impairment, or can follow the course of the disease
• CT/MRI: dilatation of the lateral ventricles, widening
of cortical sulci, particularly in frontal and temporal
regions
• Parkinson Disease
• As many as 40% with PD can develop dementia
• Risk of dementia with PD is 4x that of other
people of same age
• Dementia associated with PD – 3rd most
common form of dementia overall
• Three distinct neuropathological changes:
– senile plaques and neurofibrillary tangles
– Lewy bodies
– primary nigral degeneration
• Lewy Body Dementia (diffuse Lewy Body disease or
DLBD)
– Cognitive decline, visual hallucinations, parkinsonism,
repeated falls, sensitivity to neuroleptics (like haloperidol)
– Fluctuating cognitive function; episodic confusion, delusions,
hallucinations may differ from AD
• PRION-RELATED DISEASES
– rare
– Creutzfeldt-Jakob disease (CJD) – most common prion
disease
– 50-70 years old
– Spongy degeneration, neuronal loss, astrocyte proliferation
– Progressive dementia, myoclonus, pyramidal signs,
cerebellar or extrapyramidal signs
– EEG: periodic sharp waves
– Subacute – decline in weeks or months; most die within 1
year of onset
INFECTIOUS DISEASES
• HIV TYPE 1-ASSOCIATED DEMENTIA COMPLEX
– AIDS Dementia Complex (ADC)
– Other terms: HIV encephalitis, HIV encephalopathy
– Frequent sequela of AIDS
– Severe dementia, behavioral changes; motor disorder,
myelopathy and peripheral neuropathy in 25%
– SYMPTOMS:
• Early – apathy, social withdrawal, slow thinking, poor
concentration, forgetfulness; psychiatric symptoms are
psychosis, depression and mania
INFECTIOUS DISEASES
• Motor signs – slow movements, leg weakness, gait ataxia
• Advanced stage – akinetic mutism; immobile; global cognitive
impairment, urinary incontinence
– Diagnosis – clinical features and laboratory tests
– Frontotemporal atrophy, multinucleated giant cells, microglial
nodules, perivascular infiltrates
– Treatment:
• Zidovudine (AZT), selegiline, nimodipine – mixed results
(some improved; some no effect)
– Predictors of dementia: CD4+ count <100 cell/uL, anemia, or
AIDS-defining infection or neoplasm (19% to 25% risk in 2
years)
INHERITED METABOLIC DISEASES