Atypical parkinsonism combining α-synuclein inclusions and polyglucosan body disease

✍ Scribed by Elsa Krim; Anne Vital; Frederic Macia; Farid Yekhlef; François Tison

• Book ID: 102504009
• Publisher: John Wiley and Sons
• Year: 2004
• Tongue: English
• Weight: 281 KB
• Volume: 20
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.20285

✦ Synopsis

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 α‐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. © 2004 Movement Disorder Society