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A Novel Pattern of Central Nervous System Involvement in MELAS

Liana G. Apostolova, MD; Matthew White, MD; Steven A. Moore, MD, PhD; Patricia H. Davis, MD

Arch Neurol. 2005;62:1154-1156.

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.

Author Affiliations: Department of Neurology, University of California, Los Angeles (Dr Apostolova); Departments of Radiology (Dr White), Pathology (Dr Moore), and Neurology (Dr Davis), University of Iowa, Iowa City.

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