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Diagnosis of paraproteinemic neuropathy: Room for improvement
Journal of the Neurological Sciences ( IF 4.4 ) Pub Date : 2020-08-01 , DOI: 10.1016/j.jns.2020.116902
Chafic Karam 1 , Orly Moshe-Lilie 1 , Nizar Chahin 1 , Thomas Ragole 1 , Eva Medvedova 2 , Rebecca Silbermann 2
Affiliation  

OBJECTIVE To report our institutional experience with paraproteinemic neuropathy. METHODS We reviewed the charts of patients evaluated at our tertiary, academic neuromuscular clinic for neuropathy between 2017 and 2019 and selected those with a serum monoclonal protein. We collected patients' characteristics and reviewed their initial diagnoses and eventual outcomes. RESULTS Fifty-four of 410 patients with neuropathy (13%) had a monoclonal protein. Of these patients, 25% had not had SPEP or IFE checked prior to referral. FLC was not checked in any of the patients prior to referral. The neuropathy was felt to be related to the monoclonal protein in 24 patients (44%). Ten patients (19%), had been misdiagnosed either because they were not screened for monoclonal protein or the monoclonal protein was considered a MGUS. AL amyloid and POEMS syndrome were the most frequently missed diagnoses. CONCLUSION The diagnosis of paraproteinemic neuropathy was missed in nearly one in five patients in our cohort. Failure to accurately characterize a paraproteinemic neuropathy can have devastating effect on patients as some have underlying malignancies. We propose that testing serum free light chains in patients with peripheral neuropathy of unknow etiology, when SPEP/IFE are normal, may reduce the rate of misdiagnosis. Furthermore, patients with refractory CIDP should be carefully screened for POEMS syndrome.

中文翻译:

副蛋白血症性神经病的诊断:改进空间

目的报告我们在副蛋白血症性神经病方面的机构经验。方法 我们回顾了 2017 年至 2019 年在我们的三级学术神经肌肉诊所评估的神经病患者图表,并选择了具有血清单克隆蛋白的患者。我们收集了患者的特征并回顾了他们的初步诊断和最终结果。结果 410 名神经病变患者中有 54 名 (13%) 含有单克隆蛋白。在这些患者中,25% 的患者在转诊前未进行 SPEP 或 IFE 检查。在转诊之前没有检查任何患者的 FLC。24 名患者 (44%) 的神经病变被认为与单克隆蛋白有关。10 名患者 (19%) 被误诊,要么是因为他们没有进行单克隆蛋白筛查,要么是因为单克隆蛋白被认为是 MGUS。AL 淀粉样蛋白和 POEMS 综合征是最常漏诊的。结论 在我们的队列中,近五分之一的患者漏诊了副蛋白血症性神经病的诊断。未能准确描述副蛋白血症性神经病变可能对患者产生破坏性影响,因为有些患者具有潜在的恶性肿瘤。我们建议,当 SPEP/IFE 正常时,检测病因不明的周围神经病变患者的血清游离轻链可能会降低误诊率。此外,难治性 CIDP 患者应仔细筛查 POEMS 综合征。未能准确描述副蛋白血症性神经病变可能对患者产生破坏性影响,因为有些患者具有潜在的恶性肿瘤。我们建议,当 SPEP/IFE 正常时,检测病因不明的周围神经病变患者的血清游离轻链可能会降低误诊率。此外,难治性 CIDP 患者应仔细筛查 POEMS 综合征。未能准确描述副蛋白血症性神经病变可能对患者产生破坏性影响,因为有些患者具有潜在的恶性肿瘤。我们建议,当 SPEP/IFE 正常时,检测病因不明的周围神经病变患者的血清游离轻链可能会降低误诊率。此外,难治性 CIDP 患者应仔细筛查 POEMS 综合征。
更新日期:2020-08-01
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