Steroid-responsive chorea in moyamoya disease

Authors

  • Dr. Steven G. Pavlakis,

    1. Departments of Pediatrics of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
    2. Departments of Neurology of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
    Current affiliation:
    1. Division of Pediatric Neurology, New York Hospital-Cornell Medical Center, New York, New York 10021, U.S.A.
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  • Steven Schneider,

    1. Departments of Neurosurgery of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
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  • Karen Black,

    1. Departments of Radiology of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
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  • Robert J. Gould

    1. Departments of Pediatrics of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
    2. Departments of Neurology of North Shore University Hospital and New York Hospital-Cornell Medical Center, Cornell University, Manhasset, and New York City, New York, U.S.A.
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Abstract

We report the case of a 13-year-old boy with disabling chorea due to moyamoya disease. His chorea seemed to improve with steroid therapy. We conclude that steroid therapy may ameliorate moyamoya-associated chorea, and perioperative steroids can confound neurosurgical outcome. We are unable to assess the effect of cerebrovascular bypass procedures on the outcome of chorea in this patient.

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