Baba M, Takada H, Tomiyama M, Ozaki I, Miura H, Matsunaga M, Muranaka H, Kashiwamura H, Kurihara A
Department of Neurology, Hirosaki University School of Medicine, Japan.
No To Shinkei. 1993 Mar;45(3):233-40.
Clinical, electrophysiological and histopathological findings in 6 children with steroid-responsive acquired demyelinating neuropathy are presented. The clinical features and nerve conduction findings are basically similar to those of chronic inflammatory demyelinating neuropathy (CIDP) in adults, although early-onset cases had prominent pes cavus deformity and thickened nerves, which are rare findings in acquired neuropathies in adults. The diagnostic criteria of adult CIDP can be adopted for most of the cases, however, repeated electrophysiological tests may be required to identify multifocality of the nerve lesion, especially when conduction block is not apparent before treatment. The biopsied sural nerves showed many thinly-myelinated fibers, subperineurial and endoneurial edema, and cellular infiltrations. Varied fascicular involvements were common. Two cases with almost complete or considerable loss of myelinated fibers in the biopsied sural nerve revealed good clinical response to steroid therapy. The degree of nerve degeneration in the sural nerve thus, may not be helpful to estimate the prognosis and the responsiveness to treatment. Therapeutic trials should be employed when the main conduction findings are those of demyelinating neuropathies, even if genetically-determined neuropathy is suggested from the clinical pictures.
本文介绍了6例对类固醇治疗有反应的获得性脱髓鞘性神经病患儿的临床、电生理和组织病理学表现。临床特征和神经传导检查结果与成人慢性炎症性脱髓鞘性多发性神经病（CIDP）基本相似，不过早发型病例有明显的高弓足畸形和神经增粗，这在成人获得性神经病中较为罕见。大多数病例可采用成人CIDP的诊断标准，然而，可能需要反复进行电生理检查以确定神经病变的多灶性，尤其是在治疗前传导阻滞不明显时。活检的腓肠神经显示有许多薄髓鞘纤维、神经束膜下和神经内膜水肿以及细胞浸润。不同程度的束状受累很常见。两例活检的腓肠神经中髓鞘化纤维几乎完全或大量丧失的病例对类固醇治疗显示出良好的临床反应。因此，腓肠神经的神经变性程度可能无助于评估预后和对治疗的反应性。当主要传导检查结果为脱髓鞘性神经病时，即使从临床症状怀疑为遗传性神经病，也应进行治疗试验。
