Free and conjugated CSF and plasma GABA in Huntington's chorea

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Abstract
Free and conjugated GABA concentrations were measured in CSF and plasma from 28 patients with manifest Huntington''s chorea (HC) and 30 age- and sex-matched controls. GABA was determined by ion-exchange chromatography with fluorimetric detection (IE/F). Free and conjugated CSF GABA was significantly decreased in prolonged HC with advanced disease states and was suggested practicable as an additional diagnostic tool. However, in younger patients (< 40 yrs) with a short period of HC (< 2 yrs) an overlap with the age-matched normal range indicated GABA measurement inadequate to early diagnosis nor predictive for offspring at risk. An age-dependent decrease of conjugated CSF GABA was observed in patients and controls. The more pronounced decrease in patients might reflect the neurodegenerative features of HC.