Subacute necrotizing encephalomyelopathy (Leigh's disease): report of a case.

M. L. Tsai, K. L. Hung, T. Y. Chen

研究成果: 雜誌貢獻文章

1 引文 斯高帕斯(Scopus)

摘要

A 5-month-old male infant was referred to us for evaluation of progressive hypotonia. He had growth retardation, feeding difficulty and general weakness. Brain sonography and computed tomography demonstrated symmetrical lesions in the caudate, lenticular nuclei, thalamus and hypothalamus, suggesting bilateral necrosis. Lactate and pyruvate levels in the blood and cerebral spinal fluid were persistently elevated. A biopsy of the quadriceps muscle showed increased subsarcolemmal mitochondrial enzyme activity on light microscopy. Electron microscopy of the muscle showed deformed and bizarre mitochondria. The patient eventually died at the age of 8 months. Autopsy showed bilateral necrotic foci in the caudate, lenticular nuclei, thalamus, hypothalamus, midbrain, and pons. Histopathologic findings included spongiform degeneration of the affected brain tissue. The characteristic clinical and pathological findings confirmed this case as subacute necrotizing encephalo-myelopathy of Leigh's type. To our knowledge, this is the first autopsy-proven case of Leigh's disease in Taiwan.

原文英語
頁(從 - 到)799-802
頁數4
期刊Journal of the Formosan Medical Association = Taiwan yi zhi
89
發行號9
出版狀態已發佈 - 九月 1 1990
對外發佈Yes

ASJC Scopus subject areas

  • Medicine(all)

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