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Effect of Gamma-Vinyl GABA in Friedreich’s Ataxia

Published online by Cambridge University Press:  18 September 2015

Y. De Smet ,
J.Y. Mear ,
G. Tell ,
P.H. Schechter ,
F. Lhermitte  and
Y. Agid
Y. De Smet
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
J.Y. Mear
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
G. Tell
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
P.H. Schechter
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
F. Lhermitte
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
Y. Agid
Affiliation:
Clinique de Neurologie et neuropsychologie Hôpital de la Salpêtrière, Paris, France and * Centre de recherche Merrell International Strasbourgh, France
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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.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 171 - 173
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

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