NEUROLOGY 1991;41:216
© 1991 American Academy of Neurology
Delayed-onset dystonia due to perinatal or early childhood asphyxia
M-H. Saint Hilaire, MD, FRCP(C),
R. E. Burke, MD,
S. B. Bressman, MD,
M. F. Brin, MD and
S. Fahn, MD
Department of Neurology (Dr. Saint Hilaire), Boston University, Boston, MA; and the Department of Neurology (Drs. Burke, Bressman, Brin, and Fahn), College of Physicians and Surgeons, Columbia University, and The Dystonia Clinic Research Center at Columbia-Presbyterian Medical Center, New York, NY.
We report 10 patients with delayed-onset dystonia associated with perinatal asphyxia and 2 associated with asphyxia in childhood. In the perinatal group, the mean age of onset was 12.9 years. Among these patients, dystonia continued to progress for a mean of 7 years, and as long as 28 years. These patients had moderate motor disability; none was wheelchair-bound, and thus their prognosis was better than that of the childhood-onset idiopathic torsion dystonias. The most frequently beneficial drugs were anticholinergics. Since some of these patients closely resembled cases of idiopathic torsion dystonia, the prior occurrence of asphyxia should be used as a criterion of exclusion for that diagnosis.
Address correspondence and reprint requests to Dr. Robert E. Burke, Box 67, The Neurological Institute. 710 West 168th Street, New York. NY 10032.
The Dystonia Clinical Research Center at Columbia-Presbyterian Medical Center is supported by a grant from the Dystonia Medical Research Foundation (DMRF). R.E.B. is supported by NINDS TIDA # 1 KO7 NS00746, R29 NS26836, by grants from the DMRF and the Parkinson's Disease Foundation. S.B.B., M.F.B., and S.F. are supported in part by NINDS 26656.
Received May 15, 1990. Accepted for publication in final form July 23, 1990.
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