Encefalomielopatía necrotizante subaguda. Síndrome de Leigh: a propósito de una observación
1978
espanolSe presenta una nina de seis meses de edad con antecedente de dos hermanas muertas en los primeros meses de la vida y sin antecedentes personales. Ingresa por un cuadro neurologico, que empeora progresivamente hasta su fallecimiento. En su evolucion presenta una acidosis tubular proximal. Los niveles de acido lactico y piruvico son normales y el estudio necropsico es compatible con una encefalomielopatia necrotizante subaguda o sindrome de Leigh. EnglishA 6-month-old girl with familiar history of two dead sisters few months old, was admitted to the Hospital with a neurological process. After her admission progressively worsened until her death. The patient had clinical manifestation of proximal tubular acidosis. Levels of lactic and piruvic acid were normal. Necropsic study was compatible with the diagnoses of subacute necrotizing encephalomielopathy of Leigh syndrome.
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