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Dementia with Lewy bodies (DLB) is the second leading cause of neurodegenerative dementia. Besides dementia, prominent symptoms and signs include parkinsonism, hallucinations, fluctuating mental status, and orthostatic hypotension. Myoclonus has been estimated to occur in 15% of DLB cases. The source of the myoclonus, its electrophysiologic characteristics, and mechanism are unknown. To define the source and properties of the myoclonus in DLB, we examined seven subjects with DLB who had myoclonus, both clinically and electrophysiologically, and compared these characteristics with those of myoclonus in PD.
Methods.
The seven subjects were diagnosed with DLB using consensus criteria and according to Diagnostic and Statistical Manual of Mental Disorders, 4th ed. criteria for dementia that included dementia before parkinsonism, parkinsonism, and hallucinations.1 We performed a neurologic examination, Folstein Mini-Mental State Examination (MMSE), Unified Parkinson’s Disease Rating Scale (UPDRS) motor exam, and Hoehn & Yahr staging. The Unified Myoclonus Rating Scale (UMRS) was used to assess arm/wrist myoclonus amplitude. Electrophysiologic tests were performed as previously published.2 Briefly, each subject had EEG/electromyogram (EMG) polygraphy, back-averaging of myoclonus EMG discharges off-line, median nerve somatosensory evoked potentials (SEPs) …
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