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Recent neuropathologic autopsy studies found that 15 to 25%of elderly demented patients have Lewy bodies(LB)in their brainstem and cortex,and in hospital series this may constitute the most common pathologic subgroup after pure Alzheimer's disease(AD).We identified progressive disabling mental impairment progressing to dementia as the central feature of DLB. Attentional impairments and disproportionate problem solving and visuospatial difficulties are often early and prominent.Fluctuation in cognitive function,persistent well-formed visual hallucinations,and spontaneous motor features of parkinsonism are core features with diagnostic significance in discriminating DLB from AD and other dementias. Appropriate clinical methods for eliciting these key symptoms are described.Brainstem or cortical LB are the only features considered essential for a pathologic diagnosis of DLB,although Lewy-related neuritis,Alzheimer pathology,and spongiform change may also be seen.We identified optimal staining methods for each of these and devised a protocol for the evaluation of cortical LB frequency based on a brain sampling procedure consistent with CERAD.This allows cases to be classified into brainstem predominant,limbic(transitional),and neocortical subtypes,using a simple scoring system base on the relative distribution of semiquantitative LB counts.Alzheimer pathology also frequently present in DLB,usually as diffuse or neuritic plaques,neocortical neurofibrillary tangles being much less common.The precise nosological relationship between DLB and AD remains uncertain,as does that between DLB and patients with Parkinson's disease who subsequently develop neuropsychiatric features.Finally,we recommend procedures for the selective sampling and storage of frozen tissue for a variety of neurochemical assays,which together with developments in molecular genetics,should assist future refinements of diagnosis and classification. |
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dementia
falling
hallucination
hallucination,visual
Lewy body disease,diffuse
neuroleptic
neuroleptic sensitivity
neurologic disease,diagnoses of
neuropathology
Parkinson disease
Parkinsonism syndrome
review article
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