Miyanomae Y, Takeuchi Y, Nishimura A, Kawase S, Hirai K, Ochi M, Sawada T
Department of Pediatrics, Kyoto City Child Welfare Center, Japan.
Acta Paediatr Jpn. 1996 Dec;38(6):576-9. doi: 10.1111/j.1442-200x.1996.tb03711.x.
Median and posterior tibial motor nerve conduction studies were performed on 10 children with spinal muscular atrophy (SMA). Three patients with SMA type I, in whom rapid deterioration occurred, showed reduced motor nerve conduction velocity and a remarkably low M-wave amplitude in both nerves. In type II and III patients, the motor nerve conduction velocity was normal in the median nerve, although the M-wave amplitude was small in the tibial nerve. In four patients, a reduction of the M-wave amplitude was observed as clinical symptoms advanced. These findings may suggest that motor conduction studies in spinal muscular atrophy provide complementary information for understanding the pathogenesis and are also useful to clarify the heterogeneity of this disease.
对10名脊髓性肌萎缩症（SMA）患儿进行了正中神经和胫后运动神经传导研究。3例I型SMA患儿病情迅速恶化，两条神经的运动神经传导速度均降低，M波幅明显较低。II型和III型患儿中，正中神经运动神经传导速度正常，尽管胫神经M波幅较小。4例患儿随着临床症状进展，M波幅降低。这些发现可能提示，脊髓性肌萎缩症的运动传导研究为理解发病机制提供了补充信息，也有助于阐明该疾病的异质性。
