Chaudhary Umair J, Rajabally Yusuf A
Inflammatory Neuropathy Clinic, Department of Neurology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, B15 2TH, UK.
Aston Medical School, Aston University, Birmingham, UK.
J Neurol. 2021 Apr;268(4):1366-1373. doi: 10.1007/s00415-020-10287-7. Epub 2020 Nov 10.
The frequency and causes of underdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) are uncertain. We aimed to assess the frequency and electroclinical features of pre-referral CIDP underdiagnosis and the duration of delay prior to diagnosis and treatment initiation in a tertiary specialist clinic.
We retrospectively investigated 60 consecutive patients attending our Inflammatory Neuropathy Service, between 2015 and 2019, with a final diagnosis of treatment-responsive definite/probable CIDP. We reviewed the clinical and electrophysiological data in light of European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines and determined the frequency, causes and delay in diagnosis of CIDP.
An initial alternative diagnosis to that of CIDP had been made in 68.3% (41/60) of patients. The commonest alternative diagnosis was of Guillain-Barré syndrome (GBS) in 23.3% (14/60) patients. Non-GBS underdiagnoses (27/60; 45%) mainly consisted of genetic neuropathy (8/27; 29.6%), diabetic neuropathy (5/27; 18.5%) and chronic idiopathic axonal polyneuropathy (4/27; 14.8%). Non-GBS underdiagnoses were predominantly due to non-recognition of proximal weakness (70.4%), multifocal deficits (18.5%) or proprioceptive loss (7.4%). Electrophysiological misinterpretation was contributory to pre-referral non-GBS underdiagnoses of CIDP in 85% of patients. Mean diagnostic delay in patients with non-GBS underdiagnoses of CIDP was of 21.3 months (range 2-132 months).
Underdiagnosis of CIDP is frequent and may lead to significant diagnostic and treatment delay. We suggest that lack of comprehensive and precise attention to typical electroclinical features of CIDP and its diagnostic criteria at the time of initial evaluation are equally contributory to underdiagnoses.
慢性炎症性脱髓鞘性多发性神经病（CIDP）漏诊的频率及原因尚不确定。我们旨在评估在三级专科诊所中，转诊前CIDP漏诊的频率、电临床特征以及诊断和开始治疗前的延迟时间。
我们回顾性研究了2015年至2019年间连续就诊于我们的炎症性神经病服务中心的60例患者，最终诊断为对治疗有反应的明确/可能的CIDP。我们根据欧洲神经病学学会联合会/周围神经学会（EFNS/PNS）指南回顾了临床和电生理数据，并确定了CIDP的诊断频率、原因和延迟情况。
68.3%（41/60）的患者最初被诊断为非CIDP。最常见的误诊为吉兰-巴雷综合征（GBS），占23.3%（14/60）。非GBS漏诊（27/60；45%）主要包括遗传性神经病（8/27；29.6%）、糖尿病性神经病（5/27；18.5%）和慢性特发性轴索性多发性神经病（4/27；14.8%）。非GBS漏诊主要是由于未认识到近端肌无力（70.4%）、多灶性缺损（18.5%）或本体感觉丧失（7.4%）。电生理解读错误导致85%的患者在转诊前出现非GBS的CIDP漏诊。非GBS漏诊的CIDP患者平均诊断延迟为21.3个月（范围2 - 132个月）。
CIDP漏诊很常见，可能导致显著的诊断和治疗延迟。我们认为，在初次评估时缺乏对CIDP典型电临床特征及其诊断标准的全面、精确关注同样会导致漏诊。
