Choreiform movements in spinocerebellar ataxia type 1

We describe the unusual case of a 51-year-old woman with spinocerebellar at axia type 1 (SCAI) who showed choreiform movements in addition to cerebella r ataxia. To date, extrapyramidal signs including involuntary movements hav e been rarely reported in SCA1. Surface electromyogram in our patient revea led grouped discharges whose duration was longer than that of chorea observ ed in HD, indicating that the involuntary movements represented choreoathet osis rather than pure chorea. These choreiform movements have not been seen in non-hereditary spinocerebellar ataxia. Therefore, if 'sporadic' cases o f cerebellar ataxia show such movements, the possibility of genetic origin of the ataxia is high and a surveillance of various forms of hereditary spi nocerebellar ataxia including SCAI is required. (C) 2001 Elsevier Science B .V. All rights reserved.