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

Dentatorubropallidoluysian Atrophy (DRPLA) With Comitant Esotropia

• Yoo JK
• Koo YS
• Kwon DY
• Park MH
• Park KW

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

MeSH Terms

Adult
Ataxia
Atrophy
Cerebellar Ataxia
Esotropia
Female
Humans
Spinocerebellar Ataxias
Spinocerebellar Degenerations