Rapidly Progressive Dementia and Myoclonus

SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and pathological correlation: progressive disabling mental impairment with peculiar features such as hallucinations, fluctuating cognition or motor parkinsonism, and cortical with or without subcortical Lewy bodies.¹ We report briefly two cases with unusual features of DLB.

Case 2. A 72-year-old man with history of tobacco addiction and asbestosis presented with a 1-year history of progressive forgetfulness. Examination disclosed mild temporospatial confusion, dressing and ideomotor apraxia, and bilateral cogwheel rigidity. Language and attention were well preserved. Brain CT was normal. Six months later, the patient came to the hospital for cognitive impairment with shuffling gait. Examination showed akinesia and rigidity. MMSE was 9/30. Routine biological investigations were unrevealing, with no changes on brain CT. EEG showed slow left activities. Four months later, the patient was admitted to the hospital bedridden in the course of continuous and progressive decline. He was alert but unresponsive to voice. Examination disclosed rest myoclonus increased by noise, with no pyramidal features or focal signs. There was biological evidence of dehydration, with elevated natremia and creatinemia. CSF was germ-free, without pleocytosis. EEG showed slow delta activity without periodic discharges. The patient died 5 days after admission. Autopsy disclosed numerous senile plaques, neurofibrillary tangles, and cortical and subcortical Lewy bodies without spongiform changes.