Oculomotor testing in the differential diagnosis of degenerative ataxic disorders.

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Abstract
AS A RESULT of recent advances in molecular genetics, the hereditary ataxic disorders can now be classified partly on the basis of the genotype of the family.1,2 The issue is complicated by the fact that 1 clinical subtype, eg, autosomal dominant cerebellar ataxia (ADCA) I, includes cases with lesions on different genes, for example on chromosome 6p (spinocerebellar atrophy type 1 [SCA 1]), on chromosome 14q (SCA 3; Machado-Joseph disease), or on chromosome 12q (SCA 2).3 Furthermore, only about half of the cases with ADCA can now be classified on the basis of a neurogenetic analysis, because in the rest the gene locus is still unknown or at least speculative.4 On the other hand, cases with idiopathic cerebellar ataxia5 also belong to the clinical group of the degenerative ataxic disorders. Therefore, many patients with progressive cerebellar ataxia still have to undergo diagnosis based on medical history, clinical features, neurophysiological tests,6 and neuroradiological examination.7-9