Sakai K, Kato S, Komori T, Tanabe H
Department of Neurology, Tokyo Metropolitan Neurological Hospital.
Rinsho Shinkeigaku. 1996 Jun;36(6):783-5.
We report a 23-year-old female with infantile onset chronic inflammatory demyelinating polyneuropathy. Muscle weakness was noticed when she was 1 year old, and, together with sensory disturbances, gradually progressed in an asymmetric manner. Nerve conduction studies disclosed slowing of conduction velocities, temporal dispersion, and decreased amplitude of compound muscle action potentials, the degrees of which were markedly different amongst different nerves even in the same limbs. The sural nerve biopsy showed various degrees of nerve fiber amongst different fascicles. Marked inter-nerve and intra-nerve differences of involvement and clinical improvement after steroid therapy supported the diagnosis of chronic inflammatory demyelinating polyneuropathy. It should be noted that even after a long clinical course of 23 years, her clinical symptoms remained asymmetrical and multi-focal lesions could be clearly demonstrated.
我们报告一例23岁患婴儿期起病的慢性炎性脱髓鞘性多发性神经病的女性患者。她1岁时被发现有肌无力，并伴有感觉障碍，且以不对称方式逐渐进展。神经传导研究显示传导速度减慢、时间离散以及复合肌肉动作电位波幅降低，即使在同一肢体的不同神经中，这些改变的程度也有显著差异。腓肠神经活检显示不同束间神经纤维存在不同程度改变。类固醇治疗后神经间和神经内受累情况及临床改善的显著差异支持慢性炎性脱髓鞘性多发性神经病的诊断。需要注意的是，即使经过23年的漫长病程，其临床症状仍不对称，且多灶性病变清晰可见。
