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  • Title: Effect of gamma-vinyl GABA in Friedreich's ataxia.
    Author: De Smet Y, Mear JY, Tell G, Schechter PH, Lhermitte F, Agid Y.
    Journal: Can J Neurol Sci; 1982 May; 9(2):171-3. PubMed ID: 7104882.
    Abstract:
    Gamma-Vinyl GABA, an irreversible inhibitor of GABA-transaminase, was administered orally in two daily doses of 250 mg to 10 patients with cerebellar ataxia (9 with Friedreich's ataxia, one with olivo-ponto-cerebellar atrophy) for at least one month in an open study. No significant difference occurred in the disability scores of cerebellar symptomatology for the group as a whole, but seven patients showed some improvement in scores with treatment and two patients claimed marked subjective amelioration. Tolerance to Gamma-Vinyl-GABA treatment was excellent. These preliminary results suggest that further studies with well-tolerated agents which enhance CNS GABA-ergic function are warranted in patients with cerebellar ataxia.
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