Abstract Details

Title Clinicopathological Findings in Autopsy Confirmed Cases of Premotor Multiple System Atrophy

Topic Movement Disorders

Presentation(s) P04 - (-)

Poster/Presentation
Number 154

Objective

Background BACKGROUND: Current diagnostic criteria for MSA focus on the combination of motor and autonomic manifestations. However, MSA patients often show isolated autonomic failure in the early course of illness, and some patients may die during this premotor MSA phase. In addition, the clinicopathological findings in premotor MSA are extremely limited.

Design/Methods DESIGN/METHODS: We investigated 102 consecutive cases (54 men and 48 women) of pathologically confirmed MSA registrated in Aichi Medical University. The diagnosis of MSA was based upon the presence of ?-synuclein- and Gallyas-Braak-positive glial cytoplasmic inclusions (GCIs), and GCIs were confirmed in all cases. The mean age at death was 65.5±7.4 years (range, 47-85 years). The mean disease duration was 6.9±4.0 years (range, 1-25 years). To investigate the pathological spectrum of MSA, we used the grading scales of the olivopontocerebellar (OPC) and striatonigral (SN) systems, as previously described (Jellinger, et al. 2005). We defined the patients with mild or no OPC and SN pathology and no evidence of clinical motor features as premotor MSA.

Results RESULTS: Six of the 102 cases (5 men and 1 women) showed extremely mild pathology in both the OPC and SN, but showed significant cell loss and gliosis in the dorsal motor nuclei of the vagus and ventrolateral medulla, the intermediolateral column of the thoracic cord, and Onuf's nucleus. Clinically, 4 of the 6 patients did not show cerebellar ataxia and parkinsonism, but showed >1 significant autonomic symptom (urinary symptoms, 4; orthostatic hypotension, 3; and respiratory symptoms, 2). These 4 patients showed shorter survival (? 2y) than the other MSA types and sudden death.

Conclusions CONCLUSIONS: Recognition of premotor MSA patients who demonstrate sudden death prior to fulfilling the current diagnostic criteria for MSA is needed.

Authors/Disclosures
Hirohisa Watanabe, MD, PhD (Fujita Health University) PRESENTER	Dr. Watanabe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Takeda. Dr. Watanabe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Kyowa Kirin. Dr. Watanabe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Eisai. Dr. Watanabe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Ono . Dr. Watanabe has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Avvie.
Adnan Subei, DO, FAAN (Neurology Consultants of Dallas)	Dr. Subei has received personal compensation for serving as an employee of Neurology Consultants of Dallas. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Bristol Meyer Squibb. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for TG Therapeutics. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD Serono. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Amgen. Dr. Subei has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech. The institution of Dr. Subei has received research support from Novartis. The institution of Dr. Subei has received research support from Sanofi. The institution of Dr. Subei has received research support from Dianthus.
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Gen Sobue, MD (Nagoya University School Of Medicine/Dept. of Neurology)	Dr. Sobue has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Mistubishi Tanabe Pharma Corporation. Dr. Sobue has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Nippon Chemiphar Co., Ltd.. Dr. Sobue has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Mistubishi Tanabe Pharma Corporation. Dr. Sobue has received personal compensation in the range of $0-$499 for serving as an Expert Witness for Takeda Pharmaceutical Company Limited..

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