Abstract
Purpose: To investigate the pathophysiology of Unverricht-Lundborg disease (ULD) is using magnetoencephalography (MEG) and positron emission tomography using 18-fluoro-2-deoxyglucose (FDG-PET) in a ULD patient. Methods: MEG was recorded using a 204-channel whole-head MEG system, and the cortical activities preceding myoclonus of the patient were averaged. Equivalent current dipoles (ECDs) were calculated for the averaged spike. FDG-PET was performed in the interictal state. Results: The result of MEG demonstrated an electromagnetic cortical activity in the left centroparietal area, and the peak of this activity preceded the onset of myoclonic discharge on the electromyogram by 16.0 ms. ECD calculated at the peak was located in the left post central area. FDG-PET images showed regional hypometabolism in the left part of the pons. Conclusions: Functional abnormalities in the sensorimotor cortex and the brainstem coexist in this patient with ULD. Both dysfunctions were considered to be related to the pathogenesis of ULD.
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