Atypical parkinsonism combining alpha-synuclein inclusions and polyglucosan body disease

Movement Disorders : Official Journal of the Movement Disorder Society
Elsa KrimFrançois Tison

Abstract

Adult polyglucosan body disease (APGBD) is a rare disorder affecting the central and peripheral nervous systems and in which parkinsonism is unusual. A 71-year-old man presented levodopa-unresponsive parkinsonism with urinary incontinence and recurrent syncopes of 6 years standing masquerading as atypical parkinsonism of the multiple system atrophy (MSA-P) type. Brain histopathology demonstrated massive accumulation of polyglucosan bodies particularly in the putamen. In addition, there were dense alpha-synuclein-positive cytoplasmic oligodendroglial inclusions in the pons and in the middle cerebellar peduncle. These inclusions may be either due to the chance association of MSA-P with APGBD, or pathologically related to APGBD.

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Citations

Mar 30, 2010·Der Nervenarzt·T Weber, W Köhler
Jun 20, 2008·Journal of Inherited Metabolic Disease·F SedelM Vidailhet
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