Abstract

Myotonic dystrophy type 1 (DM1) is a multisystemic disease involving multiple organ systems including central nervous system (CNS) and muscles. Few studies have focused on the central motor system in DM1, pointing to a subclinical abnormality in the CNS. The aim of our study was to investigate patterns of cerebral activation in DM1 during a motor task using functional MRI (fMRI). Fifteen DM1 patients, aged 20 to 59 years, and 15 controls of comparable age were scanned during a self-paced sequential finger-to-thumb opposition task of their dominant right hand. Functional MRI images were analyzed using SPM99. Patients underwent clinical and genetic assessment; all subjects underwent a conventional MR study. Myotonic dystrophy type 1 patients showed greater activation than controls in bilateral sensorimotor areas and inferior parietal lobules, basal ganglia and thalami, in the ipsilateral premotor area, insula and supplementary motor area (corrected P<.05 analysis="" of="" the="" interaction="" between="" disease="" and="" age="" showed="" that="" correlation="" with="" was="" significantly="" greater="" in="" patients="" than="" controls="" bilateral="" sensorimotor="" areas="" contralateral="" parietal="" areas.="" other="" clinical="" mr="" characteristics="" did="" not="" correlate="" fmri.="" functional="" changes="" dm1="" may="" represent="" compensatory="" mechanisms="" such="" as="" reorganization="" redistribution="" networks="" to="" compensate="" for="" ultrastructural="" neurochemical="" occurring="" part="" accelerated="" aging="" process.="">