[Hereditary degenerative spinocerebellar diseases in Tunisia with manometric studies in bladder disorders (author's...

During a survey conducted in Tunisia in 1978, 204 cases of hereditary degenerative spinocerebellar diseases were discovered among members of 117 families. The cases included 109 patients with Friedreich's ataxia, 28 with Piere Marie's heredo-ataxia, 20 with Strumpell-Lorrain's disease, and 47 with intermediary forms. The latter group included incomplete forms of Friedreich's and Pierre Marie's diseases. The onset or progression of the disease was linked to a febrile episode in 25 p. cent of the cases. Emphasis is placed on the presence of bladder sphincter disorders in approximately one third of the patients with Friedreich's or Pierre Marie's diseases. Manometric studies in 17 cases demonstrated the presence of normal bladders in 4 cases, hypertonicity of the bladder in 5 patients, and hypesthesic retention-type bladders in 5 other cases. In 3 patients the disorder was difficult to classify. These results show that sphincter disorders should not constitute a criterium for exclusion of the diagnosis of spinocerebellar degeneration.