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以不宁腿综合征为表现的冷球蛋白血症性神经病

Cryoglobulinaemic neuropathy manifesting with restless legs syndrome.

作者信息

Gemignani F, Marbini A, Di Giovanni G, Salih S, Margarito F P, Pavesi G, Terzano M G

机构信息

Institute of Neurology, University of Parma, Italy.

出版信息

J Neurol Sci. 1997 Nov 25;152(2):218-23. doi: 10.1016/s0022-510x(97)00187-1.

Abstract

In a series of 12 patients with essential mixed cryoglobulinaemia (EMC) and peripheral neuropathy as main feature of the disease, restless legs syndrome (RLS) was a major manifestation in four women, aged 55-65 years. In one patient RLS was a presenting manifestation of the disease, and in another patient the diagnosis of EMC was made investigating RLS and polyneuropathy, although prior rheumatological symptoms were retrospectively recognized. All patients with RLS had symmetrical sensory polyneuropathy, but non-RLS patients had also other forms of peripheral neuropathy, and symmetrical sensory polyneuropathy only in two of eight cases (P=0.03). Neurophysiological study showed that sensory action potentials of the sural nerve were more often inelicitable in non-RLS patients (six of eight) than in RLS patients (none of three). Sural nerve biopsy had no distinctive features in three RLS patients, with regard to other patients with cryoglobulinaemic neuropathy. RLS seems not uncommon in cryoglobulinaemic neuropathy, and significantly associated with symmetrical sensory polyneuropathy, whereas patients with other subtypes of cryoglobulinaemic neuropathy do not develop RLS; thus, a disorder of the sensory inputs may be important in the pathogenesis of RLS. The occurrence of RLS, especially in middle-aged women, should prompt investigations for peripheral neuropathy focusing on cryoglobulinaemic neuropathy.

摘要

在一系列以原发性混合性冷球蛋白血症(EMC)和周围神经病变为主要疾病特征的12例患者中,不安腿综合征(RLS)是4名年龄在55 - 65岁女性的主要表现。在1例患者中,RLS是该疾病的首发表现;在另1例患者中,尽管回顾性地认识到先前存在的风湿症状,但对RLS和多发性神经病变进行检查后才做出EMC的诊断。所有患有RLS的患者均有对称性感觉性多发性神经病变,但未患RLS的患者也有其他形式的周围神经病变,且仅8例中的2例有对称性感觉性多发性神经病变(P = 0.03)。神经生理学研究表明,腓肠神经的感觉动作电位在未患RLS的患者(8例中的6例)中比在患RLS的患者(3例中无一例)中更常无法引出。与其他冷球蛋白血症性神经病变患者相比,3例患RLS的患者的腓肠神经活检无明显特征。RLS在冷球蛋白血症性神经病变中似乎并不少见,且与对称性感觉性多发性神经病变显著相关,而其他亚型的冷球蛋白血症性神经病变患者不会发生RLS;因此,感觉输入障碍可能在RLS的发病机制中起重要作用。RLS的出现,尤其是在中年女性中,应促使针对以冷球蛋白血症性神经病变为重点的周围神经病变进行检查。

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