Comparative analysis of brain structure, metabolism, and cognition in myotonic dystrophy 1 and 2

• DOI: 10.1212/wnl.0b013e3181d8c35f
• Publication Date: 2010-03-11T04:05:00Z
• Authors: Y.G. Weber; R. Roebling; J. Kassubek; S. Hoffmann; A. Rosenbohm; M. Wolf; P. Steinbach; K. Jurkat-Rott; H. Walter; S.N. Reske; F. Lehmann-Horn; F.M. Mottaghy; H. Lerche
• Keywords: Voxel-based morphometry; Statistical parametric mapping

<b>Objective:</b> Myotonic dystrophy type 1 and 2 (DM1/DM2) are multisystemic diseases with common cognitive deficits beside the cardinal muscular symptoms. We performed a comprehensive analysis of cerebral abnormalities to compare neuropsychological defects findings in different imaging methods same cohort patients. <b>Methods:</b> Neuropsychological investigations, structural MRI including brain parenchymal fraction (BPF) voxel-based morphometry (VBM), ¹⁸F-deoxy-glucose PET (FDG-PET) were patients (20 DM1 9 DM2) matched healthy controls, analyzed using statistical parametric mapping (SPM2). <b>Results:</b> DM2 showed typical pronounced impairment nonverbal episodic memory. Both patient groups reduction global gray matter (measured by BPF), which could be localized frontal parietal lobes VBM. Interestingly, VBM revealed bilateral hippocampal volume that was correlated specifically both clinical score memory deficits. also change thalamic matter. White lesions found &gt;50% their extent psychomotor speed. FDG-PET frontotemporal hypometabolism, independent decrease cortical All similar but more for DM1. <b>Conclusions:</b> Our results suggest 1) some characteristic these linked specific changes, 2) decreases metabolism processes, 3) widespread