Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS

Liana G APOSTOLOVA; Matthew WHITE; Steven A MOORE; Patricia H DAVIS

doi:10.1001/archneur.62.7.1154

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Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS

Journal article

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Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS

Liana G APOSTOLOVA, Matthew WHITE, Steven A MOORE and Patricia H DAVIS

Archives of neurology (Chicago), Vol.62(7), pp.1154-1156

2005

DOI: 10.1001/archneur.62.7.1154

PMID: 16009776

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Abstract

Background Myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome typically manifests in adults younger than 40 years with encephalopathy, stroke-like episodes, and lactic acidosis. Magnetic resonance imaging (MRI) abnormalities typically involve the cortical gray and the adjacent subcortical white matter. Objective To describe a 58-year-old woman diagnosed with MELAS who was initially seen with acute myopathy, cardiac ischemia, psychosis, and MRI changes in a watershed distribution. Results Initial MRI of the brain showed the characteristic parieto-occipital gray matter lesions involving the adjacent white matter. Follow-up MRI revealed striking deep white matter involvement in a watershed distribution. A cerebral angiogram and thorough hypercoagulable workup results were normal. Electromyography showed acute denervation and myopathy. A muscle biopsy specimen revealed ragged red and cytochrome-c oxidase–negative fibers. Mitochondrial DNA analysis revealed an A3243G mutation. Conclusions Myopathy, encephalopathy, lactic acidosis, and stroke-like episodes should be considered in older patients with myopathy, cardiomyopathy, encephalopathy, and unaccountable MRI findings. Watershed pathologic features are a rare pattern of cerebral involvement in MELAS.

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Details

Title: Subtitle: Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS
Creators: Liana G APOSTOLOVA - Department of Neurology, University of California, Los Angeles, United States
Matthew WHITE - Department of Radiology, University of Iowa, Iowa City, United States
Steven A MOORE - Department of Pathology, University of Iowa, Iowa City, United States
Patricia H DAVIS - Department of Neurology, University of Iowa, Iowa City, United States
Resource Type: Journal article
Publication Details: Archives of neurology (Chicago), Vol.62(7), pp.1154-1156
DOI: 10.1001/archneur.62.7.1154
PMID: 16009776
NLM abbreviation: Arch Neurol
ISSN: 0003-9942
eISSN: 1538-3687
Publisher: American Medical Association; Chicago, IL
Language: English
Date published: 2005
Academic Unit: Neurology; Pathology
Record Identifier: 9984047725202771

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