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El ayuno cetogénico intermitente con triglicéridos de cadena media mejora la ataxia en la deficiencia de la coenzima
Wiebke Hahn1,2, Karla Erffmeier1,2, Maximilian Schulze3
1Department of Neurology, Philipps-University Marburg, Baldingerstraße, 35043 Marburg, Germany.
Molecular genetics and metabolism reports
|February 20, 2026
Resumen
El ayuno intermitente cetogénico mejoró la ataxia en un paciente con deficiencia de la coenzima Q10 relacionada con COQ8A. Esta estrategia dietética se muestra prometedora como terapia adyuvante para la ataxia mitocondrial, lo que justifica una mayor investigación.
Área de la Ciencia:
- Neurología Neurología.
- Trastornos metabólicos Los trastornos metabólicos son trastornos metabólicos que se producen en el cuerpo.
- Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales Enfermedades mitocondriales
Sus antecedentes:
- Las mutaciones en COQ8A causan deficiencia primaria de la coenzima Q10, lo que lleva a diversos síntomas neurológicos como ataxia cerebelosa y epilepsia.
- Si bien la suplementación con coenzima Q10 es común, la evidencia de estrategias complementarias, especialmente para características no epilépticas, es limitada.
Objetivo del estudio:
- Para investigar la eficacia del ayuno intermitente cetogénico (KIF) como terapia adyuvante para la ataxia cerebelosa en un paciente con deficiencia de la coenzima Q10 relacionada con COQ8A.
Principales métodos:
- Una mujer de 46 años con deficiencia de COQ8A genéticamente confirmada y ataxia / epilepsia se sometió a un protocolo KIF de 6 meses (16: 8 ayuno intermitente con triglicéridos de cadena media, seguido de ayuno intermitente solo).
- La ataxia clínica se evaluó utilizando la Escala para la Evaluación y Calificación de la Ataxia (SARA).
- Se realizó una resonancia magnética cerebral, incluyendo imágenes de difusión, antes y después de la intervención.
Principales resultados:
- Se observó una mejora significativa en la ataxia, con las puntuaciones de SARA disminuyendo de 8.5 a 6.0.
- No se observó reducción en las convulsiones mioclónicas.
- La resonancia magnética cerebral indicó una tendencia hacia una mejor integridad microestructural cerebelosa.
Conclusiones:
- El ayuno intermitente cetogénico puede servir como una terapia complementaria beneficiosa para la ataxia mitocondrial.
- Este enfoque dietético muestra potencial para mejorar la ataxia en la deficiencia de COQ10.
- Se necesitan estudios más amplios para validar estos hallazgos.
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