Dystonia and parkinsonism in GM1 type 3 gangliosidosis.

Article Details

Citation

Roze E, Paschke E, Lopez N, Eck T, Yoshida K, Maurel-Ollivier A, Doummar D, Caillaud C, Galanaud D, Billette de Villemeur T, Vidailhet M, Roubergue A

Dystonia and parkinsonism in GM1 type 3 gangliosidosis.

Mov Disord. 2005 Oct;20(10):1366-9.

PubMed ID
15986423 [ View in PubMed
]
Abstract

GM1 gangliosidosis is due to beta-galactosidase deficiency. Only patients with type 3 disease survive into adulthood and develop movement disorders. Clinical descriptions of this form are rare, particularly in non-Japanese patients. We describe four new patients and systematically analyze all previous reports found by a literature search and contacts with the authors for additional information. Generalized dystonia remained the predominant feature throughout the disease course and was often associated with akinetic-rigid parkinsonism. GM1 gangliosidosis must be considered as a cause of early-onset generalized dystonia, particularly in patients with short stature and skeletal dysplasia.

DrugBank Data that Cites this Article

Polypeptides
NameUniProt ID
Beta-galactosidaseP16278Details