Department of Neurology, Division of Neuromuscular Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
Muscle Nerve. 2021 Jun;63(6):881-884. doi: 10.1002/mus.27235. Epub 2021 Apr 8.
It is unknown how often patients with electrodiagnostic evidence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a potentially treatable condition, present with a distal symmetric polyneuropathy (DSP) phenotype.
We reviewed the records of patients who presented to our electrodiagnostic laboratory between January 1, 2011, to December 31, 2019, and fulfilled electrodiagnostic criteria for CIDP to identify those who presented with a sensory predominant DSP phenotype.
One hundred sixty-two patients had a chronic acquired demyelinating neuropathy, of whom 138 met criteria for typical or atypical CIDP. Nine of these patients presented with a sensory predominant DSP phenotype, among whom six were eventually diagnosed with distal acquired demyelinating symmetric (DADS) neuropathy; one with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes (POEMS) syndrome; and two with idiopathic DSP. The prevalence of acquired chronic demyelinating neuropathies among all patients presenting with a DSP phenotype was estimated to be 0.34%.
Patients who meet electrodiagnostic criteria for CIDP rarely present with a sensory predominant DSP phenotype, and electrodiagnostic testing rarely identifies treatable demyelinating neuropathies in patients who present with a DSP phenotype.
患有电诊断证据显示的慢性炎症性脱髓鞘性多发性神经病（CIDP）的患者，一种潜在可治疗的疾病，其表现为远端对称性多发性神经病（DSP）表型的频率尚不清楚。
我们回顾了 2011 年 1 月 1 日至 2019 年 12 月 31 日期间在我们的电诊断实验室就诊的患者的记录，这些患者符合 CIDP 的电诊断标准，以确定那些表现出感觉为主的 DSP 表型的患者。
162 例患者患有慢性获得性脱髓鞘神经病，其中 138 例符合典型或非典型 CIDP 的标准。其中 9 例患者表现为感觉为主的 DSP 表型，其中 6 例最终被诊断为远端获得性脱髓鞘对称性（DADS）神经病；1 例为 Polyneuropathy、Organomegaly、Endocrinopathy、Monoclonal protein、Skin changes (POEMS) 综合征；2 例为特发性 DSP。在所有表现为 DSP 表型的患者中，获得性慢性脱髓鞘神经病的患病率估计为 0.34%。
符合 CIDP 电诊断标准的患者很少表现出以感觉为主的 DSP 表型，电诊断测试很少在表现为 DSP 表型的患者中发现可治疗的脱髓鞘神经病。
