Ohkoshi N, Harada K, Nagata H, Yato M, Shoji S
Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Japan.
Eur Neurol. 2001;45(4):241-8. doi: 10.1159/000052136.
We investigated clinical and pathological features of the sural nerves of 5 patients with the ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and compared these features with those of chronic ataxic neuropathies due to other causes. The CIDP patients presented with slowly progressive ataxia with deep sensory impairment. The durations of the symptoms from onset were relatively short in CIDP (4-8 months) and cancer (3 and 10 months), but long in chronic idiopathic ataxic neuropathy (24-260 months). Corticosteroid therapy elicited a good response in all the patients with CIDP, but a poor response in the patients with other ataxic neuropathies. Sural nerve biopsy of CIDP patients showed a slight or moderate loss of myelinated fiber. This report suggests that ataxic form of CIDP is a steroid-responsive ataxic neuropathy, and large myelinated fibers of the sural nerves in ataxic form of CIDP were better preserved than those in nerves with other chronic ataxic neuropathies.
我们研究了5例慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）共济失调型患者腓肠神经的临床和病理特征，并将这些特征与其他原因所致慢性共济失调性神经病的特征进行比较。CIDP患者表现为缓慢进展的共济失调伴深感觉障碍。CIDP患者（4 - 8个月）和癌症患者（3个月和10个月）症状出现至发病的持续时间相对较短，而慢性特发性共济失调性神经病患者的症状持续时间较长（24 - 260个月）。皮质类固醇治疗对所有CIDP患者均产生良好反应，但对其他共济失调性神经病患者反应不佳。CIDP患者的腓肠神经活检显示有轻度或中度有髓纤维丢失。本报告提示，CIDP共济失调型是一种对类固醇有反应的共济失调性神经病，与其他慢性共济失调性神经病相比，CIDP共济失调型患者腓肠神经的大的有髓纤维保存得更好。
