Hattori N, Ichimura M, Aoki S, Nagamatsu M, Yasuda T, Kumazawa K, Yamamoto K, Mitsuma T, Sobue G
Department of Neurology, Nagoya University School of Medicine, Japan.
J Neurol Sci. 1998 Jan 21;154(1):66-71. doi: 10.1016/s0022-510x(97)00216-5.
The clinical, electrophysiological, and pathological findings, and the therapeutic characteristics in ten children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), whose onset age was less than 16 years, were evaluated. The clinical progression pattern of the initial phase divided the patients into two groups. One group (six patients) showed a subacute progression for up to 2 months after onset and a subsided progression over 3 months. Three patients in this group had a preceding febrile episode. The other group (four patients) showed a chronic insidious progression for more than 3 months. The former group of patients revealed a favourable response to corticosteroid therapy as compared with the latter group. However, other clinical and laboratory features at the peak impairment were not distinguishable between these two groups. Motor dominant neuropathy was common to all patients, and only three cases showed sensory disturbance on the distal limbs. No cases revealed cranial nerve involvement. Motor and sensory nerve conduction and sural nerve biopsy studies revealed the demyelinating nature of the neuropathy. These clinicopathological features suggest that the subacute progression form frequently associated with prodromal episode and rather favourable corticosteroid response is characteristic in childhood CIDP, while the chronic insidious progression form is indistinguishable from the common adult CIDP.
对10例发病年龄小于16岁的慢性炎性脱髓鞘性多发性神经根神经病（CIDP）患儿的临床、电生理、病理表现及治疗特点进行了评估。根据初始阶段的临床进展模式将患者分为两组。一组（6例患者）在发病后长达2个月表现为亚急性进展，随后3个月病情缓解。该组3例患者发病前有发热史。另一组（4例患者）表现为超过3个月的慢性隐匿性进展。与后一组相比，前一组患者对皮质类固醇治疗反应良好。然而，两组在峰值损伤时的其他临床和实验室特征并无差异。所有患者均以运动神经病变为主，仅3例患者远端肢体有感觉障碍。无一例患者出现颅神经受累。运动和感觉神经传导以及腓肠神经活检研究显示为脱髓鞘性神经病。这些临床病理特征表明，亚急性进展型常与前驱发作相关且对皮质类固醇反应较好，是儿童CIDP的特征，而慢性隐匿性进展型与常见的成人CIDP并无区别。
