Objective: There are few cases of Lewy body disease who have been initially diagnosed as pure autonomic failure(PAF), because they had only autonomic symptoms for several years prior to parkinsonism. We clarified the clinical characteristics in those cases.

Methods: We analyzed the clinical features in 9 similar cases (72±7 YO; mean±SD) recruited from literature, including our own case. All cases had cardiac sympathetic denervation in cardiac MIBG or PET study, but initially no signs of parkinsonism or cognitive dysfunction.

Results: 1. Gender ratio: all cases were male. 2. Age at onset of autonomic symptoms: Mean age was 61±9 YO. 3. Duration between the appearance of parkinsonism and the onset of autonomic symptoms; The mean duration was 10±7 years.4. Cognitive dysfunction at the appearance of parkinsonism: Six out of 9 cases (67%: Three cases had dementia and parkinsonism at same time and 3 case presented dementia within 3 years after the onset of parkinsonism) showed dementia within the observation period. Two cases were not followed after the appearance of parkinsonism.

Conclusions: 1.These cases provides the evidence that cardiac sympathetic denervation and autonomic failure including orthostatic hypotension can precede striatal dopaminergic denervation, supporting the Braak staging concept of Lewy body. 2. They frequently accompany cognitive dysfunction at same time or in a short time after the onset of parkinsonism, which is rapidly progressive.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-clinical-characteristics-of-lewy-body-disease-showed-autonomic-failures-preceding-motor-signs/