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NEUROLOGY 1983;33:369
© 1983 American Academy of Neurology

Decreased ventricular fluid norepinephrine metabolite in childhood-onset dystonia

Leslie I. Wolfson, MD, Nansie S. Sharpless, PhD, Leon J. Thal, MD, Joseph M. Waltz, MD and Kenneth Shapiro, MD

Departments of Neurology (Drs. Wolfson, Sharpless, and Thal).

3-methoxy-4-hydroxyphenylglycol (MHPG), the primary brain metabolite of norepinephrine (NE), was measured in ventricular fluid from 51 patients with dystonia, other movement disorders, or hydrocephalus. The dystonic patients were divided into three categories: childhood-onset form with early limb dystonia and rapid progression to generalized symptoms, more localized and benign adult-onset dystonia, and symptomatic dystonia. Patients with the childhood form had significantly lower ventricular fluid MHPG levels (8.7 ± 0.6 ng per milliliter) than other dystonic patients (11.4 ± 1 ng per milliliter), age-matched controls with neurologic disease (11.7 ± 1.1 ng per milliliter), or other movement disorders (11.8 ± 0.7 ng per milliliter). Decreased ventricular fluid MHPG levels suggest a possible abnormality of brain NE function in childhood dystonia.

Address correspondence and reprint requests to Dr. Wolfson, Department of Neurology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461.

Psychiatry (Dr. Sharpless) and Neurosurgery (Dr. Shapiro), Albert Einstein College of Medicine, and the Department of Neurosurgery, St. Barnabas Hospital (Dr. Waltz).

Presented in part at the thirty-fourth annual meeting of the American Academy of Neurology, Washington, DC, April 1982.

This investigation was supported in part by NIH Research Grant No. N.S. 09649 and a grant from the Dystonia Medical Research Foundation.

Accepted for publication, June 24, 1982.






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