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意义未明的单克隆丙种球蛋白病相关神经病变

Neuropathy associated with monoclonal gammopathies of undetermined significance.

作者信息

Gosselin S, Kyle R A, Dyck P J

机构信息

Peripheral Neuropathy Research Laboratory, Mayo Clinic, Rochester, MN 55905.

出版信息

Ann Neurol. 1991 Jul;30(1):54-61. doi: 10.1002/ana.410300111.

DOI:10.1002/ana.410300111
PMID:1656848
Abstract

Monoclonal proteins (IgM, IgG, and IgA) in the serum or urine of patients with neuropathy may provide a marker for amyloidosis, myeloma, lymphoma, leukemia, Waldenström's macroglobulinemia, or monoclonal gammopathy of undetermined significance (MGUS). The clinical characteristics, course, and electromyographic features among neuropathies associated with monoclonal IgM (IgM-MGUS, 31 patients), monoclonal IgG (IgG-MGUS, 24 patients), and monoclonal IgA (IgA-MGUS, 10 patients) evaluated between 1980 and 1986 were compared. Four statistically significant differences set IgM-MGUS neuropathies apart from IgG-MGUS and IgA-MGUS neuropathies: (1) higher frequency of sensory loss and ataxia, (2) higher frequency of nerve conduction abnormality--10 attributes were significantly worse (none were significantly better), (3) higher frequency of dispersion of the compound muscle action potential, and (4) higher frequency of IgM-MGUS in the MGUS neuropathy cohort than is characteristic of MGUS without neuropathy seen at our institution or than is encountered in epidemiological surveys. These differences were not thought to be due to selection or severity biases. Neither the amount of IgM nor the estimated size of the monoclonal peak was associated with severity of neuropathy. The type and severity of IgM-MGUS neuropathies with anti-myelin-associated glycoprotein antibodies were not significantly different from those without anti-myelin-associated glycoprotein antibodies. A simple relationship between the presence and amount of IgM-MGUS or anti-myelin-associated glycoprotein antibodies and neuropathy cannot be assumed.

摘要

患有神经病变的患者血清或尿液中的单克隆蛋白(IgM、IgG和IgA)可能是淀粉样变性、骨髓瘤、淋巴瘤、白血病、华氏巨球蛋白血症或意义未明的单克隆丙种球蛋白病(MGUS)的标志物。对1980年至1986年间评估的与单克隆IgM(IgM-MGUS,31例患者)、单克隆IgG(IgG-MGUS,24例患者)和单克隆IgA(IgA-MGUS,10例患者)相关的神经病变的临床特征、病程及肌电图特征进行了比较。有四个具有统计学意义的差异将IgM-MGUS神经病变与IgG-MGUS和IgA-MGUS神经病变区分开来:(1)感觉丧失和共济失调的发生率更高;(2)神经传导异常的发生率更高——10项指标明显更差(无明显更好的指标);(3)复合肌肉动作电位离散的发生率更高;(4)在MGUS神经病变队列中,IgM-MGUS的发生率高于我们机构所见的无神经病变的MGUS特征或高于流行病学调查中遇到的情况。这些差异被认为并非由于选择或严重程度偏倚所致。IgM的量或单克隆峰的估计大小均与神经病变的严重程度无关。伴有抗髓鞘相关糖蛋白抗体的IgM-MGUS神经病变的类型和严重程度与不伴有抗髓鞘相关糖蛋白抗体的神经病变无显著差异。不能假定IgM-MGUS或抗髓鞘相关糖蛋白抗体的存在及量与神经病变之间存在简单关系。

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