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.
Kornblum
Cornelia
C
Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 Bonn, Germany.
Reul
Jürgen
J
Kress
Wolfram
W
Grothe
Christoph
C
Amanatidis
Niki
N
Klockgether
Thomas
T
Schröder
Rolf
R
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