Maurage C A, Udd B, Ruchoux M M, Vermersch P, Kalimo H, Krahe R, Delacourte A, Sergeant N
INSERM U422, Faculté de Médecine, Lille, France.
Neurology. 2005 Nov 22;65(10):1636-8. doi: 10.1212/01.wnl.0000184585.93864.4e.
Neurofibrillary degeneration (NFD) occurs in the brains of patients with myotonic dystrophy (DM) type 1. The authors report a similar tau pathology in the CNS of a patient with DM2 and compare it to that of patients with DM1. A reduced expression of tau exon 2 and exon 3 epitopes is observed in both DM1 and DM2. This suggests a similar physiopathologic process that may contribute to common neurologic features in patients with DM.
神经纤维变性(NFD)发生在1型强直性肌营养不良(DM)患者的大脑中。作者报告了1例DM2患者中枢神经系统中类似的tau蛋白病理学情况,并将其与DM1患者的情况进行比较。在DM1和DM2患者中均观察到tau蛋白外显子2和外显子3表位的表达降低。这表明存在一个类似的生理病理过程,可能导致DM患者出现共同的神经学特征。