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Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2
Authors:Cornelia?Kornblum,Jürgen?Reul,Wolfram?Kress,Christoph?Grothe,Niki?Amanatidis,Thomas?Klockgether,Rolf?Schr?der  mailto:rolf.schroeder@ukb.uni-bonn.de"   title="  rolf.schroeder@ukb.uni-bonn.de"   itemprop="  email"   data-track="  click"   data-track-action="  Email author"   data-track-label="  "  >Email author
Affiliation:(1) Dept. of Neurology, University of Bonn, Sigmund-Freud-Straße 25, 53105 Bonn, Germany;(2) Dept. of Neuroradiology, Medizin Centrum Bonn, Bonn, Germany;(3) Dept. of Human Genetics, University of Würzburg, Würzburg, Germany
Abstract:
Abstract. Cranial magnetic resonance imaging (MRI) in 19 German patients with genetically proven myotonic dystrophy Type 1 (DM1, n = 10) or Type 2 (DM2, n = 9) showed pathological findings consisting of white matter lesions (WML) and/or brain atrophy in 9/10 DM1 and 8/9 DM2 patients. Anterior temporal WML (ATWML) were exclusively seen in DM1 patients. Our findings indicate a high frequency of central nervous system (CNS) involvement in both disorders. However, temporopolar pathology, previously associated with intellectual dysfunction, seems to be restricted to DM1.
Keywords:myotonic dystrophy  proximal myotonic myopathy (PROMM)  brain magnetic resonance imaging
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