Atypical parkinsonism combining alpha-synuclein inclusions and polyglucosan body disease.
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.
Krim
Elsa
E
Service de Neurologie, Centre Hospitalier Universitaire de Bordeaux, France.
Vital
Anne
A
Macia
Frederic
F
Yekhlef
Farid
F
Tison
François
F
D000368:Aged; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D005936:Glucans / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D016631:Lewy Bodies / Q000378:metabolism / Q000473:pathology; D008297:Male; D019578:Multiple System Atrophy / Q000209:etiology* / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D020734:Parkinsonian Disorders / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D012196:Review Literature as Topic; D013194:Staining and Labeling / Q000379:methods; D051843:Synucleins; D051844:alpha-Synuclein
D005904:Glial Fibrillary Acidic Protein; D005936:Glucans; D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein; C083094:polyglucosan