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An unusual case of Fahr's disease

2009; Thieme Medical Publishers (Germany); Volume: 67; Issue: 2b Linguagem: Inglês

10.1590/s0004-282x2009000300029

1678-4227

George Mendes, Gervásio Teles Cardoso de Carvalho, Paulo Pereira Christo, Leandro Fernandes Malloy‐Diniz, Atos Alves de Sousa,

Glycogen Storage Diseases and Myoclonus

Fahr’s disease (FD), also called idiopathic striopallidodentate calcinosis, nonarteriosclerotic cerebral calcification or idiopathic basal ganglia calcification1,2, is a rare clinical entity characterized by movement disorders, dementia and behavioral disorders related to symmetric and bilateral calcifications of the basal ganglia. Sporadic and family cases have been reported1-3. FD should be differentiated from Fahr’ syndrome. The latter is defined as symmetric and bilateral calcifications of the basal ganglia associated with neuropsychiatric manifestations that preferentially occur in patients with parathyroid disorders, especially hypoparathyroidism2 [...]