Raut Tushar Premraj, Garg Ravindra Kumar, Chaudhari Tejendra Singh, Malhotra Hardeep Singh, Singh Maneesh Kumar
Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India.
Ann Indian Acad Neurol. 2013 Oct;16(4):693-5. doi: 10.4103/0972-2327.120464.
Neuromyotonia is characterized by motor, sensory, and autonomic features along with characteristic electrophysiologic findings, resulting from hyperexcitability of the peripheral nerves. We describe the case of a 36-year-old man, who presented with the disabling symptoms suggestive of focal neuromyotonia involving both the lower limbs. His neurological examination revealed continuous rippling of both the calf muscles with normal power, reflexes, and sensory examination. Electrophysiology revealed spontaneous activity in the form of doublets, triplets, and neuromyotonic discharges along with the neurogenic motor unit potentials in bilateral L5, S1 innervated muscles. Magnetic resonance imaging lumbosacral spine revealed lumbar intervertebral disc protrusion with severe foraminal and spinal canal stenosis. Patient had good response to steroids and carbamazepine. The disabling focal neuromyotonia, occurring as a result of chronic active radiculopathy, brought the patient to medical attention. Patient responded to medical management.
神经性肌强直的特征是具有运动、感觉和自主神经功能特点以及特征性的电生理表现,由周围神经的兴奋性过高所致。我们描述了一名36岁男性的病例,他出现了提示双侧下肢局灶性神经性肌强直的致残症状。他的神经系统检查显示双侧小腿肌肉持续波动,肌力、反射和感觉检查正常。电生理检查显示双侧L5、S1支配肌肉出现成对、成三联体的自发电活动以及神经性肌强直放电,同时伴有神经源性运动单位电位。腰椎磁共振成像显示腰椎间盘突出伴严重的椎间孔和椎管狭窄。患者对类固醇和卡马西平反应良好。由慢性活动性神经根病导致的致残性局灶性神经性肌强直引起了患者对医疗的关注。患者对药物治疗有反应。