Kornblum Cornelia, Reul Jürgen, Kress Wolfram, Grothe Christoph, Amanatidis Niki, Klockgether Thomas, Schröder Rolf
Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 Bonn, Germany.
J Neurol. 2004 Jun;251(6):710-4. doi: 10.1007/s00415-004-0408-1.
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.
对19名经基因检测确诊为1型强直性肌营养不良（DM1，n = 10）或2型强直性肌营养不良（DM2，n = 9）的德国患者进行的头颅磁共振成像（MRI）检查显示，9/10的DM1患者和8/9的DM2患者存在包括白质病变（WML）和/或脑萎缩在内的病理表现。颞叶前部白质病变（ATWML）仅见于DM1患者。我们的研究结果表明，这两种疾病中枢神经系统（CNS）受累的频率都很高。然而，以前认为与智力功能障碍有关的颞极病变似乎仅见于DM1。
