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Dystonia in Patients With Spinocerebellar Ataxia Type 2
Author(s) -
Markovic Vladana,
DragasevicMiskovic Natasa T.,
Stankovic Iva,
Petrovic Igor,
Svetel Marina,
Kostić Vladimir S.
Publication year - 2015
Publication title -
movement disorders clinical practice
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.754
H-Index - 18
ISSN - 2330-1619
DOI - 10.1002/mdc3.12274
Subject(s) - dystonia , spinocerebellar ataxia , cervical dystonia , medicine , ataxia , neurological disorder , pediatrics , surgery , central nervous system disease , psychiatry
Dystonia has been described in various genetically proven spinocerebellar ataxias ( SCA s), most often in SCA 3, SCA 17, and SCA 2 patients. In this report, we describe different types of dystonia observed in 5 of our 11 SCA 2 patients. All our patients had cranial and/or cervical dystonia with focal or segmental distribution. Except for 1 case with isolated cervical dystonia, all other patients had lower cranial affection of variable severity. Although it is difficult to describe ataxia‐dystonia syndrome that would be highly characteristic for SCA 2, we suggest that occurrence of dystonia in a patient with slowly evolving cerebellar disease should, besides SCA 3 and SCA 17, also suggest SCA 2 testing. In patients with lower cranial dystonia, especially jaw and tongue dystonia, SCA 2 should be considered during the diagnostic workup.