Rebolledo-García D, Espay A, Espinoza GA, Contreras-Garduño S, Rebolledo-Rodríguez Z

Idioma: Español
Referencias bibliográficas: 23
Paginas: 478-484
Archivo PDF: 499.90 Kb.

RESUMEN

La degeneración hepatolenticular no wilsoniana (parkinsonismo asociado con cirrosis hepática) es una encefalopatía crónica que manifiesta un espectro clínico motor extrapiramidal debido a la disfunción de los ganglios de la base en el contexto de enfermedad hepática severa. El diagnóstico de la enfermedad es subestimado por catalogarse dentro del espectro de encefalopatía hepática. Existe evidencia de que los depósitos de manganeso en los ganglios basales sinérgicamente con el amonio interfieren en la modulación GABAérgica sobre los sistemas dopaminérgicos y glutamatérgicos, lo que conlleva a un estado de neurodegeneración aparentemente irreversible, fisiopatológica y clínicamente distinta a la enfermedad de Parkinson idiopática. Actualmente no se conoce un marcador histopatológico específico para el diagnóstico de esta enfermedad.

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