SPASTIC PARAPARESIS AS THE ONSET MANIFESTATION OF SPINOCEREBELLAR ATAXIA TYPE 7

Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disorder caused by an unsta-ble CAG repeat in the coding region of ataxin-7 gene1. A wide variety of neurological manifestations have been de-scribed in these patients, including ataxic gait, visual loss, dysartria, nystagmus, ophthalmoplegia, dysphagia, dysto-nia, and pyramidal and extrapyramidal signs2. Different combinations of these manifestations may be found in individual patients, either among as within families3, but the commonly described onset clinical man-ifestations are cerebellar ataxia and visual impairment4,5. We report a molecular proven SCA7 patient whose ini-tial manifestation was spastic paraparesis in the absence of any definite cerebellar or visual impairment at the ini-tial phases of the disease. METHOD We have retrospectively analyzed the onset manifestations of all SCA7 patients whose molecular diagnosis was confirme