CEREBRAL MR IN OPHTHALMOPLEGIA PLUS

PURPOSE: To evaluate MR patterns in ophthalmoplegia plus and correlate them with clinical symptoms. METHODS: MR was performed on a 1.5-T who le-body scanner with T2-weighted gradient-echo and spin-echo images. T he retrospective analysis included 19 patients with clinically establi shed diagnoses of ophthalmoplegia plus. RESULTS: Two types of cerebral MR abnormalities were found in ophthalmoplegia plus: brain atrophy an d hyperintensities restricted to the white matter and basal ganglia, w hich appeared as either focal or diffuse areas of high signal intensit y and were of strictly supratentorial location. No specific distributi on was found. These findings differ markedly from infarction-like lesi ons found in mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. CONCLUSIONS: MR is sensitive for the detectio n of central nervous system involvement in ophthalmoplegia plus, but f indings are nonspecific. However, cerebral MR in ophthalmoplegia plus is different from other mitochondrial encephalomyopathies and underlin es the clinical differentiation of mitochondrial encephalomyopathies.