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November 1978

Huntington's Disease: Cerebrospinal Fluid GABA Levels in At-risk Individuals

Author Affiliations

From the Neurology Service, Veterans Administration Center, Wilmington, Del (Drs Manyam and Katz); and the Departments of Neurology (Drs Manyam and Katz) and Pharmacology (Drs Hare and Glaeser), Jefferson Medical College of Thomas Jefferson University, Philadelphia. Dr Glaeser is now with the Massachusetts Institute of Technology, Cambridge.

Arch Neurol. 1978;35(11):728-730. doi:10.1001/archneur.1978.00500350032006

• Gamma-aminobutyric acid (GABA) was measured by the ion-exchange fluorometric method in CSF from 22 individuals at risk for Huntington's disease (HD), six individuals with HD, and five neurologically normal controls. The mean (± SD) GABA level in the specimens from patients with HD was 142 ± 27 pmoles/ ml, whereas that of the normal control specimens was 297 ± 87 pmoles/ml. The mean GABA level of the specimens from the individuals at risk for HD was 209 ± 79 pmoles/ml; however, nine of these were in the normal range with a mean value of 281 ± 72 pmoles/ml, while the other 13 were below the normal range with a mean value of 159 ± 27 pmoles/ ml. The data indicate that low GABA levels in CSF are evident prior to the onset of symptoms of HD but a predictive value can only be determined by continued observation of the clinical course of these at-risk individuals.

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