MR of Leigh's disease (subacute necrotizing encephalomyelopathy)

Abstract

MR images of three patients with Leigh's disease (subacute necrotizing encephalomyelopathy) were compared with CT findings. In all patients typical lesions in the basal ganglia were identified with both MR and CT. In two patients MR permitted identification of additional lesions not detected with CT. In one patient progression of MR abnormalities over a 4-month period correlated well with clinical deterioration in neurologic status. T2-weighted images with a repetition time (TR) greater than 1950 msec and an echo time (TE) greater than or equal to 60 msec or inversion-recovery images with a 50-msec TE, 1213-msec inversion time, and 3000-msec TR were advantageous in identifying multiple necrotic lesions in the brainstem, deep gray matter, periventricular white matter, and cerebral cortex. In this series MR was more sensitive in detecting and localizing multifocal necrotic lesions of Leigh's disease than CT was, and thus may be a useful diagnostic tool for patients with the appropriate clinical and laboratory abnormalities.