Journal
JOURNAL OF NEUROSURGERY
Volume 94, Issue 1, Pages 133-136Publisher
AMER ASSOC NEUROLOGICAL SURGEONS
DOI: 10.3171/jns.2001.94.1.0133
Keywords
MELAS syndrome; angiography; hyperperfusion; pathogenesis
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A 28-year-old woman presented with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The diagnosis was based on the results of molecular genetic analysis, which indicated a typical point mutation at the nucleotide pair 3243. Xenon computerized tomography scans obtained during the strokelike episodes revealed the lesion responsible for the symptoms to be an area of focal hyperperfusion, and scans obtained after the episodes revealed an area of hypoperfusion. Pathogenesis of the strokelike episodes appears to be metabolic dysfunction, although the involvement of a vascular event cannot be excluded.
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