Atypical parkinsonism combining α-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 at...

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Bibliographic Details
Published in:Movement disorders 2005-02, Vol.20 (2), p.200-204
Main Authors: Krim, Elsa, Vital, Anne, Macia, Frederic, Yekhlef, Farid, Tison, François
Format: Article
Language:English
Subjects:
adult polyglucosan body disease
Aged
alpha-Synuclein
Biological and medical sciences
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Glial Fibrillary Acidic Protein - metabolism
Glucans - metabolism
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Immunohistochemistry - methods
Inclusion Bodies - metabolism
Inclusion Bodies - pathology
Lewy Bodies - metabolism
Lewy Bodies - pathology
Male
Medical sciences
multiple system atrophy
Multiple System Atrophy - etiology
Multiple System Atrophy - pathology
Nerve Tissue Proteins - metabolism
Nervous system (semeiology, syndromes)
Neurology
Parkinsonian Disorders - complications
Parkinsonian Disorders - metabolism
Parkinsonian Disorders - pathology
parkinsonism
Review Literature as Topic
Staining and Labeling - methods
Synucleins
α-synuclein inclusions
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Summary: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
ISSN:0885-3185
1531-8257
DOI:10.1002/mds.20285