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与意义未明的IgA单克隆丙种球蛋白病相关的多发性神经病

Polyneuropathy associated with IgA monoclonal gammopathy of undetermined significance.

作者信息

Simmons Z, Bromberg M B, Feldman E L, Blaivas M

机构信息

Department of Neurology, University of Michigan, Ann Arbor.

出版信息

Muscle Nerve. 1993 Jan;16(1):77-83. doi: 10.1002/mus.880160113.

Abstract

Although polyneuropathies associated with IgM and IgG monoclonal gammopathies have been well described, polyneuropathy with IgA monoclonal gammopathy of undetermined significance (MGUS) is less commonly seen and has not been well studied. We reviewed the clinical and electrodiagnostic features of 5 such patients, and the sural nerve biopsy findings in 4 of them. One patient was diabetic, while 4 were free of other diagnoses commonly associated with neuropathy. Clinical presentations were varied. Electrodiagnostic and histological studies ranged from primary demyelination to primary axon loss to a mixed axonal/demyelinating picture. Three patients who were treated appeared to respond to prednisone or intravenous gamma globulin, despite clear clinical, electrodiagnostic, and histological differences. We conclude that the polyneuropathy associated with IgA MGUS is heterogeneous, similar to that in IgM and IgG MGUS. A trial of immunomodulating therapy appears to be warranted in such patients if the neuropathy is sufficiently severe.

摘要

虽然与IgM和IgG单克隆丙种球蛋白病相关的多发性神经病已得到充分描述,但意义未明的IgA单克隆丙种球蛋白病(MGUS)相关的多发性神经病较少见且尚未得到充分研究。我们回顾了5例此类患者的临床和电诊断特征,以及其中4例患者的腓肠神经活检结果。1例患者患有糖尿病,而另外4例无其他常见的与神经病相关的诊断。临床表现多样。电诊断和组织学研究结果从原发性脱髓鞘到原发性轴索丧失,再到混合性轴索/脱髓鞘表现。3例接受治疗的患者尽管在临床、电诊断和组织学上存在明显差异,但似乎对泼尼松或静脉注射丙种球蛋白有反应。我们得出结论,与IgA MGUS相关的多发性神经病是异质性的,与IgM和IgG MGUS中的情况相似。如果神经病足够严重,在此类患者中进行免疫调节治疗试验似乎是有必要的。

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