Premium
Atypical parkinsonism combining α‐synuclein inclusions and polyglucosan body disease
Author(s) -
Krim Elsa,
Vital Anne,
Macia Frederic,
Yekhlef Farid,
Tison François
Publication year - 2005
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.20285
Subject(s) - parkinsonism , atrophy , pathology , pons , putamen , medicine , disease , anatomy
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