RESUMO
INTRODUCTION: Olivopontocerebellar atrophy (OPCA) is a degenerative disease of the nervous system (NS) which currently has no known cure. The neuronal depopulation it brings about produces a number of neurochemical alterations, including a reduction in levels of gamma-aminobutyric acid (GABA) in tissues and in cerebrospinal fluid (CSF). The drug gabapentin (GBP) has proved to be capable of increasing the concentration of this neurotransmitter in the central nervous system, and of improving the cerebellar ataxia in other diseases with a similar neurochemical substrate. CASE REPORTS: We describe two sporadic cases of OPCA, who were administered GBP. In one of the cases, the ataxia was noticeably reduced after taking one 400 mg dose. In the other case, a considerable improvement was observed in a very intense cerebellar dysarthria, and there was less oscillopsia with better vision, following administration of GBP for a period of over 12 months. CONCLUSIONS: GBP has proved to be capable of slowing down the motor disorders reported by patients in the course of OPCA. We discuss how such effects are due to the increased levels of GABA in the NS triggered by the drug. Finally, we suggest that the administration of GBP could constitute an effective symptomatic treatment for the ataxia and the dysarthria caused by OPCA, and that the improvement in symptoms following single doses of GBP could be valuable in cases of OPCA, as well as other types of ataxia, that are ideal for taking advantage of the stimulus of the GABAergic neurotransmission.
Assuntos
Aminas/uso terapêutico , Ácidos Cicloexanocarboxílicos/uso terapêutico , Atrofias Olivopontocerebelares/tratamento farmacológico , Ácido gama-Aminobutírico/uso terapêutico , Feminino , Gabapentina , Humanos , Pessoa de Meia-IdadeRESUMO
Introducción. La atrofia olivopontocerebelosa (OPCA) es una enfermedad degenerativa del sistema nervioso (SN), sin tratamiento curativo en la actualidad. La despoblación neuronal que conlleva ocasiona diversas alteraciones neuroquímicas, entre las que destaca la disminución de ácido Gama-aminobutírico (GABA) tisular y en el líquido cefalorraquídeo (LCR). El fármaco gabapentina (GBP) se ha mostrado capaz de aumentar la concentración de este neurotransmisor en el sistema nervioso central y de proporcionar mejoría de la ataxia cerebelosa en otras enfermedades con un sustrato neuroquímico parecido. Casos clínicos. Se presentan dos casos esporádicos de OPCA a los que se administró GBP. En uno de los casos, la ataxia se redujo de forma ostensible con la toma de una dosis de 400 mg. En el otro caso se comprobó una mejoría considerable de una disartria cerebelosa muy intensa y menor oscilopsia, con una mejor visión, con la administración de GBP durante más de 12 meses. Conclusiones. El fármaco GBP ha demostrado su capacidad para atenuar los trastornos motores referidos, en el curso de la OPCA. Se expone que tales efectos se deben al aumento de GABA que proporciona el fármaco en el SN. Finalmente, se plantea que la administración de GBP podría constituir un tratamiento sintomático efectivo para la ataxia y la disartria causadas por OPCA, y que la mejoría sintomática tras una dosis única de GBP podría seleccionar casos de OPCA, y de otras clases de ataxia, idóneos para beneficiarse del estímulo de la neurotransmisión gabérgica
Introduction. Olivopontocerebellar atrophy (OPCA) is a degenerative disease of the nervous system (NS) which currently has no known cure. The neuronal depopulation it brings about produces a number of neurochemical alterations, including a reduction in levels of -aminobutyric acid (GABA) in tissues and in cerebrospinal fluid (CSF). The drug gabapentin (GBP) has proved to be capable of increasing the concentration of this neurotransmitter in the central nervous system, and of improving the cerebellar ataxia in other diseases with a similar neurochemical substrate. Case reports. We describe two sporadic cases of OPCA, who were administered GBP. In one of the cases, the ataxia was noticeably reduced after taking one 400 mg dose. In the other case, a considerable improvement was observed in a very intense cerebellar dysarthria, and there was less oscillopsia with better vision, following administration of GBP for a period of over 12 months. Conclusions. GBP has proved to be capable of slowing down the motor disorders reported by patients in the course of OPCA. We discuss how such effects are due to the increased levels of GABA in the NS triggered by the drug. Finally, we suggest that the administration of GBP could constitute an effective symptomatic treatment for the ataxia and the dysarthria caused by OPCA, and that the improvement in symptoms following single doses of GBP could be valuable in cases of OPCA, as well as other types of ataxia, that are ideal for taking advantage of the stimulus of the GABAergic neurotransmission