Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis
ABSTRACT Background Head‐shaking stereotypies have been described in patients with neurological impairment. We noted an unusual preponderance of head shaking in patients with rhombencephalosynapsis (RES). We sought to delineate the movements further and determine whether oculomotor and vestibular te...
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Published in | Movement disorders Vol. 28; no. 14; pp. 2019 - 2023 |
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Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
Blackwell Publishing Ltd
01.12.2013
Wiley Subscription Services, Inc |
Subjects | |
Online Access | Get full text |
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Summary: | ABSTRACT
Background
Head‐shaking stereotypies have been described in patients with neurological impairment. We noted an unusual preponderance of head shaking in patients with rhombencephalosynapsis (RES). We sought to delineate the movements further and determine whether oculomotor and vestibular testing could reveal their cause.
Methods
Information was collected from direct observation, video review and parental questionnaire from 59 patients with RES. Oculomotor and vestibular testing was performed in 4 children.
Results
Of 59 patients, 50 had persistent head shaking that was often observed years before RES was recognized. Three affected children demonstrated abnormal central vestibular processing.
Conclusions
Head‐shaking is common in RES. These characteristic movements may provide input to a defective vestibular system or may represent a motor pattern that is usually suppressed by vestibular feedback. Persistent head shaking should alert clinicians to the possible presence of a congenital hindbrain abnormality that affects the vestibulocerebellum, particularly RES. © 2013 International Parkinson and Movement Disorder Society |
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Bibliography: | ark:/67375/WNG-494WF68X-C ArticleID:MDS25634 istex:71D41589D667D021AB7ED3B4AC8D5EA2028EC3F5 Relevant conflicts of interest/financial disclosures Full financial disclosures and author roles may be found in the online version of this article. Nothing to report. ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
ISSN: | 0885-3185 1531-8257 |
DOI: | 10.1002/mds.25634 |