Mani Arun Mathai, Devasia Anup Joseph, Nair Aditya, Benjamin Rohit Ninan, Prabhakar Appaswamy Thirumal, Sivadasan Ajith, Mathew Vivek, Aaron Sanjith, George Biju, Alexander Mathew
Neurology Unit, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.
Department of Haematology, Christian Medical College, Vellore, Tamil Nadu, India.
Can J Neurol Sci. 2021 Sep;48(5):616-625. doi: 10.1017/cjn.2020.278. Epub 2021 Jan 5.
To study the clinical profile and outcomes of patients with paraproteinemic neuropathy (PPN) and to explore the utility of nerve conduction studies (NCSs) to differentiate between the demyelinating subtypes.
We did a retrospective analysis of patients diagnosed with PPN between January 2010 and December 2019 in an inpatient setting. The study population consisted of patients above 16 years of age presenting with clinical features suggestive of chronic peripheral neuropathy and on evaluation was found to have PPN.
A total of 74 patients were identified. The patients were predominantly in the 6th decade, and the majority were males. The subtypes of PPN were monoclonal gammopathy of undetermined significance (MGUS) (45.9%), POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) (24.3%), solitary plasmacytoma (17.6%), multiple myeloma (8.1%), and AL amyloidosis (4.1%). There are specific features on NCS which can help in identifying POEMS syndrome and IgM MGUS. The majority of patients with PPN tend to stabilize or improve with treatment; however, many have a severe residual disability. New terminology and classification of these entities as 'monoclonal gammopathies of neurological significance' can aid in early diagnosis and the development of effective treatment, to prevent residual disability.
PPN has a heterogeneous spectrum of clinical, biochemical, and electrophysiological features. NCS can help distinguish POEMS syndrome and IgM MGUS from other demyelinating subtypes.
研究副蛋白血症性神经病（PPN）患者的临床特征及预后，并探讨神经传导研究（NCS）在区分脱髓鞘亚型方面的作用。
我们对2010年1月至2019年12月在住院环境中诊断为PPN的患者进行了回顾性分析。研究人群包括16岁以上出现提示慢性周围神经病临床特征且经评估确诊为PPN的患者。
共确定74例患者。患者主要处于第六个十年，大多数为男性。PPN的亚型包括意义未明的单克隆丙种球蛋白病（MGUS）（45.9%）、POEMS综合征（多发性神经病、器官肿大、内分泌病、单克隆浆细胞病和皮肤改变）（24.3%）、孤立性浆细胞瘤（17.6%）、多发性骨髓瘤（8.1%）和AL淀粉样变性（4.1%）。NCS有特定特征可帮助识别POEMS综合征和IgM MGUS。大多数PPN患者经治疗后倾向于稳定或改善；然而，许多患者仍有严重的残留残疾。将这些实体重新命名和分类为“具有神经学意义的单克隆丙种球蛋白病”有助于早期诊断和制定有效治疗方案，以预防残留残疾。
PPN具有临床、生化和电生理特征的异质性谱。NCS有助于将POEMS综合征和IgM MGUS与其他脱髓鞘亚型区分开来。
