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Montreal Neurological Institute and Hospital (Drs. Matthews, Tampieri, Berkovic, Andermann, and Arnold), and the Departments of Pediatric Neurology (Dr. Silver) and Biochemical Genetics (Dr. Chityat), Montreal Children's Hospital, Montreal, PQ, Canada.
The MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) can be difficult to identify. We report MRI abnormalities that we believe are specific to this disorder in three patients with complete or partial MELAS syndrome. The patients all showed an unusual pattern on T2-weighted MRI with multifocal areas of hyperintense signal confined to the cortex of the cerebrum, cerebellum, and immediately adjacent white matter. Some images suggested selective cortical involvement of deeper layers only. Deep white matter was relatively spared, distinguishing this from usual cerebrovascular disease or the edema after status epilepticus. Specificity of these findings is further suggested by a good correlation of these findings with the previously described unique postmortem brain pathology of MELAS.
Address correspondence and reprint requests to Dr. D.L. Arnold, Montreal Neurological Institute and Hospital, 3801 University Street, Montreal, PQ, Canada, H3A-2B4.
D.L.A. is a chercheur boursier of the Fonds de la récherche en santé du Québec.
Received October 29, 1990. Accepted for publication in final form December 27, 1990.
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