Aysun SOYSAL, Sibel ÇETİN, Feriha ÖZER, Okan DOĞU, Hüseyin SARI, Baki ARPACI

Bakırköy Ruh ve Sinir Hastalıkları Hastanesi, 1. Nöroloji Kliniği, İstanbul

Keywords: Hereditary cerebellar ataxias, hereditary spastic paraplegias, Harding classification, World Health Organization classification

Abstract

It is well known that there are many problems in classifying hereditary ataxias. In thisstudy, we rewiewed the history, family history and clinical findings of 64 patients ( 40 male, 24 female ) with hereditary ataxic who were hospitalized in our clinics between January 1984 -June 1995; and classified them according to WHO and Harding classification. The mean age of onset was 27.36. There were 18 patients with Friedreich's ataxia, 8 patients with early onset cerebellar ataxia with retained tendon reflexes, 2 patients with Ramsay Hunt syndrome, 1 patients with X-linked recessive cerebellar ataxia, 8 patients with otosomal dominant late onset cerebellar ataxia, 23 patients with sporadic late onset cerebellar ataxia and 4 patients with hereditary spastic paraplegias.