Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2.
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.
Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 Bonn, Germany.
Cornelia
C
Kornblum
Jürgen
J
Reul
Wolfram
W
Kress
Christoph
C
Grothe
Niki
N
Amanatidis
Thomas
T
Klockgether
Rolf
R
Schröder
D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology*; D001931:Brain Mapping / Q000379:methods; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000145:classification / Q000150:complications / Q000235:genetics / Q000473:pathology*; D009483:Neuropsychological Tests