Nohl M, Doose H, Gross-Selbeck G, Jensen H P
Eur Neurol. 1978;17(3):129-35. doi: 10.1159/000114935.
A 5-year-old boy is reported with spinal myoclonus caused by cervical astrocytoma due to neurofibromatosis. The essential clinical and electromyographical signs of spinal myoclonus are the following: (1) Continuous rhythmical appearance in muscles innervated by the affected segments of the spinal cord. (2) Synchronous myoclonus in muscles innervated by the same spinal segments of one body side, asynchronous myoclonus in muscles of different segments as well as in contralateral muscles of the same segments. (3) Usually stable frequency, which may increase to a manifold under the influence of different stimuli. (4) Accentuation of the myoclonus under mental distress, disappearance during deeper sleep. The pathophysiological basis of spinal myoclonus might be a disinhibition in the area of the spinal formatio reticularis. Supraspinal stimuli can influence the myoclonus.
据报道，一名5岁男孩因神经纤维瘤病引发颈椎星形细胞瘤，进而导致脊髓性肌阵挛。脊髓性肌阵挛的主要临床和肌电图表现如下：(1) 在脊髓受累节段所支配的肌肉中持续出现节律性活动。(2) 身体一侧同一脊髓节段所支配肌肉的同步性肌阵挛，不同节段肌肉以及同一节段对侧肌肉的非同步性肌阵挛。(3) 频率通常稳定，在不同刺激影响下可能会增加数倍。(4) 在精神困扰时肌阵挛加重，深度睡眠时消失。脊髓性肌阵挛的病理生理基础可能是脊髓网状结构区域的去抑制。脊髓上的刺激可影响肌阵挛。
