Affection of basal ganglia in spinocerebellar ataxia type 3– a transcrainal sonography study

Spinocerebellar ataxia type 3 (SCA3) is the most frequent form of autosomal dominant cerebellar ataxia. Autopsy findings in spinocerebellar ataxia type 3 (SCA3) frequently reveal severe degeneration of the basal ganglia especially the substantia nigra in SCA3. In contrast, clinical signs of basal ganglia involvement are mild and occur only in a minority of SCA3 patients. Transcranial sonography (TCS) is a valuable tool in the detection of basal ganglia alterations in extrapyramidal disorders. To define basal ganglia affection in SCA3 we performed a TCS study in 15 patients with the molecular diagnosis of SCA3 an…