Takada H, Baba M, Nunomura J, Okushima T, Miura H, Matsunaga M
Department of Neurology, Hirosaki University School of Medicine.
Rinsho Shinkeigaku. 1993 Jan;33(1):36-9.
We describe a 9-year-old boy with chronic inflammatory demyelinating polyneuropathy (CIDP), whose distal dominant, slowly progressive motor sensory involvement developed since infancy. Thickened peripheral nerves were visible in his neck and palpable in the extremities. The hand muscles were atrophic and both feet showed pes cavus deformity. He was unable to walk. Nerve conduction study revealed no sensory potentials. Compound muscle potential (CMP) was absent in the legs. In the arms, CMP showed markedly low amplitude and temporal dispersion. Motor conduction velocities were 1.7 to 2.7 m/sec, showing extremely high threshold to electrical stimuli. Conduction block was not convincing, nevertheless the asymmetric conduction abnormalities strongly suggested multifocality of the lesion. In the biopsied sural nerve no myelinated fibers were found. Considerable improvements in muscle power and electrophysiological findings were brought about by corticosteroid therapy. CIDP in childhood may masquerade hereditary neuropathies presenting hypertrophic peripheral nerves and pes cavus deformity. A careful nerve conduction study is thus desirable to find out electrophysiological multifocality.
我们描述了一名9岁患有慢性炎症性脱髓鞘性多发性神经病（CIDP）的男孩，其自婴儿期起就出现以远端为主的、缓慢进展的运动感觉受累。在他的颈部可见增粗的周围神经，在四肢可触及。手部肌肉萎缩，双足呈高弓足畸形。他无法行走。神经传导检查未引出感觉电位。腿部复合肌肉动作电位（CMP）消失。在手臂，CMP显示波幅明显降低和时限离散。运动传导速度为1.7至2.7米/秒，对电刺激显示极高阈值。传导阻滞并不明确，但不对称的传导异常强烈提示病变的多灶性。在活检的腓肠神经中未发现有髓纤维。皮质类固醇治疗使肌肉力量和电生理结果有了显著改善。儿童期的CIDP可能会伪装成表现为周围神经肥大和高弓足畸形的遗传性神经病。因此，需要仔细进行神经传导检查以发现电生理多灶性。
