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[伴有混合性冷球蛋白血症的周围神经病]

[Peripheral neuropathy with mixed cryoglobulinemia].

作者信息

Manganelli P, Pavesi G, Fiocchi A, Gemignani F, Ferraccioli G, Nervetti A, Marbini A

机构信息

II Divisione medica e Reumatologia, USL 4, Parma.

出版信息

Recenti Prog Med. 1990 Nov;81(11):681-5.

PMID:1962889
Abstract

Among 54 patients with mixed cryoglobulinemia a peripheral neuropathy was demonstrated in 23 patients (42.6%). Five patients had a secondary form while the majority (18) had essential cryoglobulinemia. The immunoglobulin components were polyclonal in 5 cases, only present in trace amounts in 5 and of type II in 13 patients. Fifteen patients had symptoms of polyneuropathy, but in 18 cases the symptomatology had symmetrical features and in two cases asymmetrical characteristics. In 8 patients a mono-polyneuropathy was observed. The most significant electrophysiological finding was the decreased amplitude of the sensory action potential of the sural nerve, which we observed in 95% of the instances, whereas a decreased sensory conduction velocity was seen in 8/23 cases only. A biopsy of the sural nerve, performed in eleven cases, always showed abnormal epi- and endoneurial vessels. Epineurial vessel vasculitis was found in 4 cases and fibrous thickening of the vessel wall in two cases. Endothelial swelling of the endoneurial vessels was observed in 7 patients and luminal obliteration in 3 samples. Axonal degeneration demonstrated in 7 of the 11 processed sample tissues was the main pathological finding. Signs of de/remyelination were associated in 3 cases, and were the dominant lesions in a single specimen. The simultaneous biopsy of the peroneus brevis muscle showed signs of denervation in all samples, vasculitis in 7 cases and deposits of filaments plus annular bodies of the vessel wall in 2 samples. All these data support the hypothesis that ischaemic damage may be the final common pathway of the majority of peripheral neuropathies in mixed cryoglobulinemia patients.

摘要

在54例混合性冷球蛋白血症患者中,23例(42.6%)出现周围神经病变。5例为继发性,而大多数(18例)为原发性冷球蛋白血症。免疫球蛋白成分5例为多克隆性,5例仅微量存在,13例为II型。15例患者有多发性神经病变症状,但18例症状具有对称性,2例具有不对称特征。8例患者观察到单发性或多发性神经病变。最显著的电生理发现是腓肠神经感觉动作电位幅度降低,我们在95%的病例中观察到这一现象,而仅在8/23例中发现感觉传导速度降低。11例患者进行了腓肠神经活检,结果均显示神经外膜和神经内膜血管异常。4例发现神经外膜血管血管炎,2例发现血管壁纤维性增厚。7例患者观察到神经内膜血管内皮肿胀,3个样本出现管腔闭塞。11个处理过的样本组织中有7个显示轴突变性,这是主要的病理发现。3例伴有脱髓鞘/髓鞘再生迹象,且在单个标本中这些是主要病变。同时对短腓骨肌进行活检,所有样本均显示失神经迹象,7例有血管炎,2个样本有血管壁细丝和环状体沉积。所有这些数据支持这样一种假说,即缺血性损伤可能是混合性冷球蛋白血症患者大多数周围神经病变的最终共同途径。

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