Summary

The clinical features of diffuse Lewy body disease (DLBD) in the elderly were retrospectively reviewed in 12 cases which had been diagnosed on pathological grounds. Their ages at onset and at death were between 60 and 85 years and between 76 and 92 years respectively. All of them had dementia of mild to severe degree, and eight presented with persistent severe hallucination-delusion psychosis with or without delirium. A single case presented with symptoms typical of Parkinson's disease including resting tremor, cogwheel rigidity and akinesia, while four of them showed no parkinsonian features. In the other seven, dementia or psychosis was followed by rigidity and akinesia at a late stage in the disease. Four had a history of orthostatic hypotension with syncope reminiscent of Shy-Drager syndrome. One can suspect DLBD when an elderly patient presents with atypical dementia of varying degrees, in combination with any of persistent delusion-hallucination psychosis, mild parkinsonism, and orthostatic syncope or dizziness.

Introduction

Diffuse Lewy body disease (DLBD) is a neuropathological entity in which abundant Lewy bodies (LBs) are found throughout the cerebral cortex, as well as in the brainstem nuclei in a manner identical to that seen in Parkinson's disease (Kosaka et al., 1980; Kosaka et al., 1984). Marked senile changes including abundant senile plaques and Alzheimer neurofibrillary tangles of diverse degree are also found in the elderly patients.

Summary

Cortical Lewy bodies (LBs), neurofibrillary tangles (NFTs) and senile plaques (SPs) in the limbic and neocortical cortices of the temporal lobe were studied with anti-ubiquitin, anti-tau and anti-β-protein immunohistochemistry in senile dementia of Alzheimer type (SDAT), diffuse Lewy body disease (DLBD), Parkinson's disease (PD) with and without dementia, and normal controls. DLBD was characterized by numerous cortical LBs together with marked senile changes similar to those of SDAT though neocortical NFTs were significantly less than in SDAT. There were rare or no cortical LBs and neocortical NFTs in most PD cases with and without dementia while there were a few cortical LBs and moderate senile changes, in some cases with dementia. These findings suggest that either of the abundant cortical LBs and senile change, or both have produced dementia in DLBD, while the neuropathological background for dementia in PD cases may be heterogeneous and vary among individual cases.

Introduction

Diffuse Lewy body disease (DLBD) is a neuropathological entity in which abundant Lewy bodies (LBs) are found throughout the cerebral cortex as well as in the brainstem nuclei in the manner identical to Parkinson's disease (Kosaka et al., 1980, 1984). Marked senile changes including abundant senile plaques (SPs) and Alzheimer neurofibrillary tangles (NFTs) are also found in this condition. Immunohistochemical stains with tau and β-protein antibodies respectively demonstrate NFTs and SPs selectively and sensitively. Ubiquitin immunohistochemistry can immunostain LBs of both brainstem and cortical types sensitively (Kuzuhara et al., 1988; Lowe et al., 1988), and detect far more LBs than the conventional techniques (Lennox et al., 1989).