| Congenital Leigh's disease: panencephalomyelopathy and peripheral neuropathy. | |
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MedLine Citation:
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PMID: 6236667 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A dystrophic newborn girl (38th week of gestation) presented as a floppy infant with relapsing episodes of lactic acidosis and progressive cerebral deterioration. She died after serious apnoea at the age of 8 weeks. Neuropathological examination demonstrated widespread changes of Leigh's subacute polioencephalomyelopathy affecting the cerebral cortex, basal ganglia, mesencephalon and spinal cord. In addition, there was severe leukoencephalopathy of the cerebral hemispheres with sudanophilic tissue degregation, advanced axonal loss and reactive astrocytic gliosis. There was marked demyelination of the sciatic nerves. Electron-microscopical examination of the hypertrophic heart revealed pathological mitochondria with tubular inclusions suggestive of primary mitochondrial cardiomyopathy. Similar mitochondrial changes were not detected in the CNS and PNS. The complex of neuropathological alterations in this case is nevertheless consistent with a congenital variant of Leigh's disease. |
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Authors:
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R J Seitz; K Langes; H Frenzel; G Kluitmann; W Wechsler |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta neuropathologica Volume: 64 ISSN: 0001-6322 ISO Abbreviation: Acta Neuropathol. Publication Date: 1984 |
Date Detail:
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Created Date: 1984-10-25 Completed Date: 1984-10-25 Revised Date: 2007-11-09 |
Medline Journal Info:
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Nlm Unique ID: 0412041 Medline TA: Acta Neuropathol Country: GERMANY, WEST |
Other Details:
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Languages: eng Pagination: 167-71 Citation Subset: IM |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Brain
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pathology* Brain Diseases / congenital* Cardiomegaly / pathology Demyelinating Diseases / congenital Female Humans Infant, Newborn Mitochondria, Heart / ultrastructure Necrosis Peripheral Nerves / pathology* Syndrome |
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