家族性遗传性小脑共济失调是一组以小脑共济失调为主要表现的中枢神经系统变性疾病,通常有家族遗传史。病变主要影响小脑与脊髓,但基底节、大脑皮层、周围神经也可被波及。本病多年来已为临床医生所熟知。 Familial hereditary cerebellar ataxia is a group of central nervous system degenerative diseases with cerebellar ataxia as the predominant manifestation, usually with a family history. Lesions mainly affect the cerebellum and spinal cord, but basal ganglia, cerebral cortex, peripheral nerves can also be affected. The disease has been well known to clinicians for many years.