Clinical Spectrum and Evolution of Monoclonal Gammopathy-associated Neuropathy An Observational Study
Clinical Spectrum and Evolution of Monoclonal Gammopathy-associated Neuropathy An Observational Study
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DOI：
10.1097/nrl.0b013e31826a99e9
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发表时间：
2012-11-01
期刊：
影响因子：
1.2
通讯作者：
Padovani, Alessandro
中科院分区：
文献类型：
--
作者：
Filosto, Massimiliano;Cotelli, Mariasofia;Padovani, Alessandro
背景：副蛋白血症性神经病（PPN）由于其临床和电生理的变异性而常常被低估。神经病变的进展被认为是潜在的单克隆丙种球蛋白病（MG）可能恶性转化的警钟。目的：报告一组PPN患者的临床表现、病程和演变，以确定有助于实现诊断、怀疑进展和识别潜在血液学状况的结果。39例PPN患者接受了临床检查、电诊断研究、脑脊液分析和实验室检查。结果：本组病例中IgM型MG占51.4%，IgG型MG占33.3%，伊加MG占10.3%。PPN主要表现为感觉性、脱髓鞘性、轻度进行性神经病变，与峰型或轻链型无关。然而，轴突的结果存在于许多IgG型患者和部分IgM型患者中，少数IgG型患者可能在发病时出现运动症状。IgM型患者有明显的临床恶化趋势，IgG型患者的恶性肿瘤发生率更高。IgA相关的神经病变是罕见的，异质性，并具有较高的倾向，演变和malignancy.Conclusions：大多数的PPN往往提出了一个相对单形的临床图片，但他们可以在临床上异质性，必须怀疑，即使感觉障碍和脱髓鞘不是占主导地位的功能。恶性肿瘤的倾向似乎在全球范围内升高，需要加强随访。对周围神经病变患者的诊断方法应始终包括血清和尿液中单克隆免疫球蛋白的分型。相比之下，MG患者应接受神经传导研究/神经电图和神经系统评估。
Background: Paraproteinemic neuropathy (PPN) is often under-diagnosed because of its clinical and electrophysiological variability. Progression of neuropathy is considered an alarm bell for possible malignant conversion of underlying monoclonal gammopathy (MG).Objective: To report clinical presentation, course, and evolution in a group of patients with PPN in order to identify findings useful for achieving the diagnosis, suspecting progression, and recognizing the underlying hematological conditions.Patients and Methods: Thirty-nine patients with PPN underwent clinical examination, electrodiagnostic studies, cerebrospinal fluid analysis, and laboratory tests. These parameters were compared between the different peak groups.Results: IgM MG was found in 51.4%, IgG MG in 33.3%, and IgA MG in 10.3% of our cohort. PPN appeared as mainly sensory, demyelinating, mildly progressive neuropathy, regardless of the type of peak or light chain. However, axonal findings were present in many IgG patients and in part of the IgM patients and a small number of the IgG patients may have presented with motor symptoms at the onset. The IgM patients had a significant tendency toward clinical worsening and IgG subjects had a more elevated rate of malignancy. IgA-related neuropathies were rare, heterogenous, and with a high tendency to evolution and malignancy.Conclusions: Most of PPN often present a relatively monomorphic clinical picture but they can be clinically heterogenous and must be suspected even if sensory impairment and demyelination are not the dominant features. Tendency to malignancy seems globally elevated and needs intensive follow-up. Diagnostic approach to patients presenting with peripheral neuropathy should always include the typing of monoclonal immunoglobulins in serum and urine. In contrast, patients presenting with MG should be submitted to nerve conduction study/electroneurography and neurological evaluation.