| Alpers' syndrome presenting with seizures and multiple stroke-like episodes in a 17-year-old male. | |
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MedLine Citation:
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PMID: 8605737 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Alpers' syndrome is a progressive neurodegenerative disorder with liver disease that usually presents in the first few years of life. Only rarely have patients presented later in life with delayed onset of Alpers' syndrome. Herein we present a case of a 17-year-old male with a progressive 8-month course of severe headaches, multiple stroke-like episodes with visual deficits, and seizures that concluded with acute hemorrhagic pancreatitis. Neuropathological findings were characteristic for Alpers' syndrome: neurodegeneration and astrogliosis of the occipital cortices including the striate cortices, similar but less advanced changes in the parietal cortices, right Ammons horn sclerosis, degeneration of the posterior columns, and mild cerebellar Purkinje cell loss. Examination of the liver revealed extensive centrilobular hepatocyte necrosis. Skeletal muscle did not contain ragged red fibers, nor were there mitochondrial DNA point mutations characteristic for mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). |
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Authors:
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T J Montine; J M Powers; F S Vogel; R A Radtke |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Clinical neuropathology Volume: 14 ISSN: 0722-5091 ISO Abbreviation: Clin. Neuropathol. Publication Date: 1995 Nov-Dec |
Date Detail:
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Created Date: 1996-05-17 Completed Date: 1996-05-17 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 8214420 Medline TA: Clin Neuropathol Country: GERMANY |
Other Details:
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Languages: eng Pagination: 322-6 Citation Subset: IM |
Affiliation:
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Department of Pathology (Neuropathology), Duke University Medical Center, Durham, NC 27710, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Brain / pathology* Cerebral Cortex / pathology Cerebrovascular Disorders / genetics, pathology* DNA / genetics Diagnosis, Differential Diffuse Cerebral Sclerosis of Schilder / genetics, pathology* Humans Liver / pathology Magnetic Resonance Imaging Male Nerve Degeneration / physiology Neurons / pathology Point Mutation / genetics Purkinje Cells / pathology Seizures / genetics, pathology* |
| Chemical | |
Reg. No./Substance:
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9007-49-2/DNA |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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