Two otherwise healthy immunocompetent men, ages 62 and 66 years, experienced years of radicular pain without zoster rash. An extensive search for systemic disease and malignancy was negative. Varicella-zoster virus DNA, but not herpes simplex virus DNA, was found in the cerebrospinal fluid of the first patient 5 months after the onset of pain, and in the second patient 8 months after the onset of pain. Prolonged radicular pain without zoster rash combined with the presence of varicella-zoster virus in the cerebrospinal fluid indicates that both men had zoster sine herpete, and strongly supports this syndrome as a clinical variant.
Thirty cases of diffuse Lewy body disease (DLBD) have been reported, primarily 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.
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