Kinsella L J, Lange D J, Trojaborg W, Sadiq S A, Younger D S, Latov N
Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY 10032.
Neurology. 1994 Jul;44(7):1278-82. doi: 10.1212/wnl.44.7.1278.
We reviewed the clinical and electrophysiologic features of 36 patients with increased titers of IgM anti-GM1 antibodies. Mildly elevated titers of up to 3,200 were not associated with any particular clinical syndrome or disease. Clinically, 14 of 16 patients with highly elevated titers of 6,400 or higher had progressive weakness with lower motor neuron signs; six had active tendon reflexes and eight had absent reflexes, but none had definite upper motor neuron signs. Electrophysiologic studies showed spontaneous activity in all 14 patients, one or more motor conduction blocks in nine, slowed motor conductions in one, and normal conductions in four patients. None had abnormal sensory conductions. These patients presented with a syndrome that has features of, but is distinct from, both motor neuron disease and demyelinating neuropathy.
我们回顾了36例IgM抗GM1抗体滴度升高患者的临床和电生理特征。滴度轻度升高至3200与任何特定的临床综合征或疾病均无关联。临床上，16例滴度高度升高至6400或更高的患者中，14例出现进行性肌无力并伴有下运动神经元体征；6例腱反射活跃，8例腱反射消失，但均无明确的上运动神经元体征。电生理研究显示，14例患者均有自发活动，9例有一个或多个运动传导阻滞，1例运动传导减慢，4例传导正常。无一例感觉传导异常。这些患者表现出一种具有运动神经元病和脱髓鞘性神经病特征，但又与之不同的综合征。
