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

Research output: Contribution to journalArticle

28 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1154-1156
Number of pages3
JournalArchives of Neurology
Volume62
Issue number7
DOIs
StatePublished - Jul 1 2005

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Lactic Acidosis
Brain Diseases
Muscular Diseases
Central Nervous System
Stroke
Magnetic Resonance Imaging
Electromyography
Denervation
Electron Transport Complex IV
White Matter
Cardiomyopathies
Mitochondrial DNA
Psychotic Disorders
Young Adult
Angiography
Ischemia
Biopsy
Muscles
Mutation
Brain

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

Cite this

Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS. / Apostolova, Liana G.; White, Matthew; Moore, Steven A.; Davis, Patricia H.

In: Archives of Neurology, Vol. 62, No. 7, 01.07.2005, p. 1154-1156.

Research output: Contribution to journalArticle

Apostolova, Liana G. ; White, Matthew ; Moore, Steven A. ; Davis, Patricia H. / Deep white matter pathologic features in watershed regions : A novel pattern of central nervous system involvement in MELAS. In: Archives of Neurology. 2005 ; Vol. 62, No. 7. pp. 1154-1156.
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