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A 6 year-old girl with a history of seizures while playing video games who was admitted with pancreatitis. She had an episode of transient blindness and had the following MRI.

Axial FLAIR  images. Note the area of increased signal in the occipital lobes bilaterally. Family history revealed others with MELAS and biopsy was confirmatory. Mitochondrial Encephalmyopathy with Lactic Acidosis and Stroke-like episodes (MELAS) is a maternally inherited disorder, which usually has onset during childhood. The stroke-like episodes occur predominantly posteriorly, and patients' deficits are usually transient. Besides these episodes and lactic acidosis, patients also often complain of seizures, vomiting, and vascular headaches. While the muscle biopsy typically shows ragged red fibers, the myopathy rarely produces frank weakness.

Revised 11/04/05.
The Electronic Curriculum is copyrighted 1998,  Case Western Reserve University School of Medicine.