摘要：

目的 总结误诊为慢性炎性脱髓鞘性多发性神经根神经病（CIDP）的正己烷中毒性周围神经病患者的临床表现与组织病理学特点。方法与结果 2 例密切接触含有正己烷成分粘合剂的患者均于 6 ~ 8 个月后出现亚急性或慢性周围神经病变，以始于双下肢远端的肢体麻木、无力为首发症状，逐渐进展至下肢近端和上肢，脱离有毒作业环境后临床症状呈延迟恶化现象。神经传导检测提示四肢运动和感觉神经传导速度减慢、波幅降低，下肢重于上肢。腓肠神经活检有髓纤维密度呈轻至中度减少，可见有髓纤维形成巨大轴索和 Wallerian 变性，偶见再生簇结构，巨大轴索内充满神经微丝和颗粒物质。治疗原则为迅速脱离有毒作业环境和 B 族维生素营养神经，预后良好。结论 正己烷中毒性周围神经病的临床症状和脑脊液改变与CIDP相似，周围神经组织活检术可明确诊断。

关键词: 周围神经系统疾病, 中毒, 已烷类

Abstract:

Objective  To investigate the clinical and neuropathological features of N-hexane toxic peripheral neuropathy which was misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods and Results Two patients presented with subacute or chronic peripheral neuropathies after close exposure of N-hexane for 6 to 8 months, starting from weakness and numbness of bilateral distal lower limbs and gradually progressing to proximal lower limbs and upper limbs. The worsening of clinical symptoms was delayed after keeping out of toxic working environment. Decreased motor nerve conduction velocity (MNCV) and sensory nerve conduction velocity (SNCV) of all limbs as well as marked decrease of amplitude, with lower extremities predominantly, were detected in both cases. Sural nerve biopsies revealed mild to moderate loss of myelinated fibers, formation of giant axons, Wallerian degeneration and rare regenerating clusters. The giant axons were filled with neurofilaments and granular materials. The patients were required to keep away from toxic working environment and were treated with vitamin B, and the prognosis was good. Conclusions The clinical symptoms and cerebrospinal fluid (CSF) findings of N-hexane toxic peripheral neuropathy are similiar to CIDP. Peripheral nerve biopsy can play a key role in the diagnosis of the disease.

Key words:  Peripheral nervous system diseases, Poisoning, Hexanes