J Korean Neurol Assoc.  2009 Nov;27(4):428-431.

Dentatorubropallidoluysian Atrophy (DRPLA) With Comitant Esotropia

Affiliations
  • 1Department of Neurology, Korea University College of Medicine, Ansan, Korea. kwondoya@hanmail.net

Abstract

The possibility of a central origin should be considered for late-onset concomitant esotropia. Concomitant esotropia has been reported to occur with spinocerebellar ataxia types 1, 2, and 3, but not with other degenerative cerebellar ataxia disorders. We report on a 28-year-old woman with ataxia in whom a detailed ophthalmologic examination revealed concomitant esotropia. She was subsequently diagnosed with dentatorubropallidoluysian atrophy (DRPLA). We suggest that the presence of concomitant esotropia could be used to differentiate DRPLA from other hereditary ataxias.

Keyword

Dentatorubropallidoluysian atrophy (DRPLA); Concomitant esotropia
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