Diffuse Lewy body disease and progressive dementia

C. R. Burkhardt, C. M. Filley, B. K. Kleinschmidt-DeMasters, S. de la Monte, M. D. Norenberg, S. A. Schneck

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Abstract

Thirty cases of diffuse Lewy body disease (DLBD) have been reported, primarly by neuropathologists, but an associated clinical syndrome has not been clearly defined. Four recent cases have led us to examine the clinicopathologic correlations. Patients are usually elderly, with symptoms lasting from 1 to 20 years. Progressive dementia or psychosis is typically the first and most prominent feature. Parkinsonian signs, initially mild or absent, become common eventually, and rigidity is usually severe. Involuntary movements, myoclonus, quadriparesis in flexion, orthostatic hypotension, and dysphagia have also been noted. Classic, concentric Lewy bodies are found profusely in the brainstem, basal forebrain, and hypothalamic nuclei, while less well defined 'Lewy-like' bodies occur in limbic structures and in deep neocortical layers. In addition, focal spongiform changes in the mesial temporal lobe were found in two of our cases. We suggest that DLBD may be another specific cause of progressive dementia.

Original languageEnglish (US)
Pages (from-to)1520-1528
Number of pages9
JournalNeurology
Volume38
Issue number10
StatePublished - Oct 1988

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ASJC Scopus subject areas

  • Clinical Neurology

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Burkhardt, C. R., Filley, C. M., Kleinschmidt-DeMasters, B. K., de la Monte, S., Norenberg, M. D., & Schneck, S. A. (1988). Diffuse Lewy body disease and progressive dementia. Neurology, 38(10), 1520-1528.