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Brainstem involvement in Unverricht-Lundborg disease (EPM1): An MRI and (1)H MRS study
M Mascalchi1, R Michelucci, M Cosottini
1Diagnostic Radiology, Department of Clinical Pathophysiology, University of Florence, Viale Morgagni 85, 50134 Florence, Italy. m.mascalchi@dfc.unifi.it
Neurology
|June 12, 2002
Abstract:
MRI of the brain and proton MRS ((1)H MRS) of the pons and dentate were obtained in 10 patients with genetically confirmed Unverricht-Lundborg disease (EPM1) and 20 control subjects. Patients with EPM1 showed (p < or = 0.01) loss of bulk of the basis pontis, medulla, and cerebellar hemispheres. Cerebral atrophy was present in six patients. The N-acetylaspartate/creatine and choline/creatine ratios were reduced in the pons but not in the dentate (p < or = 0.005). Brainstem involvement could play a role in pathophysiology of EPM1.