Ando T, Fukatsu H, Kameyama T, Takahashi A, Yamada H
Department of Neurology, Nagoya University School of Medicine.
Rinsho Shinkeigaku. 1993 May;33(5):575-8.
A 16-year-old man visited our clinic because of right-sided weakness of shoulder abduction and elbow flexion. He was well until about three weeks previously. Neurological examination revealed only that he had severe weakness together with mild atrophy of the muscles innervated by the C5 and C6 segments (the deltoid, biceps brachii, brachioradialis), slight hypesthesia on the thumb, and loss of deep reflexes of the biceps brachii on the right. No pyramidal signs were found in the lower extremities. EMG showed neurogenic changes of the atrophied muscles. Neuroradiological studies revealed right-sided atrophy of the spinal cord at C4-5 disk level, anterior shift of the posterior cervical dura mater and congestion of the internal vertebral venous plexus mainly at C4 and C5 vertebral level with his neck flexed. Conservative physiotherapy provided a good recovery within a few months. The clinical and neuroradiological findings of this case resemble those of juvenile type of distal and segmental muscular atrophy of upper extremities excluding distribution of involved muscles because of the different level of the spinal cord lesion. Pathomechanism of this case is considered to be the same as that of juvenile type of distal and segmental muscular atrophy of upper extremities.
一名16岁男性因右侧肩部外展和肘部屈曲无力前来我院就诊。他此前身体状况良好，直至大约三周前。神经系统检查仅发现他存在严重无力，以及由C5和C6节段支配的肌肉（三角肌、肱二头肌、肱桡肌）轻度萎缩，拇指轻度感觉减退，右侧肱二头肌深反射消失。下肢未发现锥体束征。肌电图显示萎缩肌肉有神经源性改变。神经放射学研究显示，在颈部屈曲时，C4 - 5椎间盘水平右侧脊髓萎缩，颈后硬膜向前移位，主要在C4和C5椎体水平的椎内静脉丛充血。保守物理治疗在数月内带来了良好恢复。该病例的临床和神经放射学表现与青少年型上肢远端和节段性肌肉萎缩相似，但由于脊髓病变水平不同，受累肌肉分布有所差异。该病例的发病机制被认为与青少年型上肢远端和节段性肌肉萎缩相同。
