Brown P, Thompson P D, Rothwell J C, Day B L, Marsden C D
MRC Human Movement and Balance Unit, Institute of Neurology, London, UK.
Brain. 1991 Feb;114 ( Pt 1A):197-214.
Three patients are described with nonrhythmic repetitive axial myoclonic jerks causing symmetric flexion of the neck, trunk, hips and knees. No electrophysiological evidence of a cortical or brainstem reticular origin for the myoclonus was found. In the first patient the axial jerks only occurred spontaneously. The latencies of recruitment of spinal segments during a jerk indicated that the discharge arose in the mid thoracic cord and then slowly spread at about 5 ms-1 up and down the cord to involve rostral and caudal segments. No structural lesion was identified in this patient. In the second patient spontaneous and reflex axial jerks developed following the excision of a cervical haemangioblastoma. In the stimulus-induced jerks the relative latencies of muscles innervated by rostral and caudal spinal segments suggested that the myoclonus originated between the upper cervical and midthoracic cord. In the final patient, EMG activity during spontaneous and stimulus-induced jerks commenced in the rectus abdominis, and was followed by later activity in muscles innervated by rostral spinal segments, suggesting that the myoclonus originated in the midthoracic cord. No structural lesion was identified in this patient. Electrophysiological evidence is used to argue a spinal origin for these axial jerks in all 3 cases. There are striking features common both to this form of human myoclonus and to long propriospinal pathways identified in animals. The new concept of propriospinal myoclonus is suggested.
本文描述了3例患者，他们出现无节律性的重复性轴向肌阵挛性抽搐，导致颈部、躯干、臀部和膝盖对称屈曲。未发现肌阵挛起源于皮质或脑干网状结构的电生理证据。在第一例患者中，轴向抽搐仅自发出现。抽搐期间脊髓节段募集的潜伏期表明放电起源于胸段中部脊髓，然后以约5毫秒/秒的速度在脊髓上下缓慢扩散，累及头端和尾端节段。该患者未发现结构性病变。在第二例患者中，颈椎血管母细胞瘤切除后出现了自发和反射性轴向抽搐。在刺激诱发的抽搐中，由头端和尾端脊髓节段支配的肌肉的相对潜伏期表明肌阵挛起源于颈上段和胸段中部脊髓之间。在最后一例患者中，自发和刺激诱发的抽搐期间的肌电图活动始于腹直肌，随后是由头端脊髓节段支配的肌肉的后期活动，提示肌阵挛起源于胸段中部脊髓。该患者未发现结构性病变。电生理证据支持这3例患者的轴向抽搐均起源于脊髓。这种人类肌阵挛形式与动物中发现的长脊髓固有通路具有显著的共同特征。文中提出了脊髓固有性肌阵挛的新概念。
