[Lafora's disease and movement disorders: report of 2 cases].
Two cases of Lafora's disease with prominent movement disorders portraying rare initial manifestations are reported. In both patients, the first manifestations were cerebellar ataxia, dysartria and startle phenomenon. These symptoms occurred before seizures, myoclonic and progressive dementia, which are more well known as manifestations of Lafora's disease. The diagnosis was confirmed by the identification of PAS positive inclusion bodies in deep skin biopsy samples. Our patients presented an unexpected slow progression of the disease, with longer survival. Lafora's disease should be remembered among diseases causing slowly progressive ataxia associated with epileptic seizures.
de Quadros
A
A
Departamento de Clínica Médica, Hospital de Clínicas, Universidade Federal do Paraná.
Sá
D S
DS
Kowacs
P A
PA
Teive
H A
HA
Werneck
L C
LC
D000328:Adult; D017668:Age of Onset; D001706:Biopsy; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D020192:Lafora Disease / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D009069:Movement Disorders / Q000209:etiology*; D009679:Nuclear Family