Service de Neurologie, CHU Montpellier, Hôpital Gui de Chauliac, 34295, Montpellier, France.
Muscle Nerve. 2013 Nov;48(5):727-32. doi: 10.1002/mus.23821. Epub 2013 Aug 30.
Sensory chronic inflammatory demyelinating polyneuropathy (CIDP) can be difficult to diagnose.
We report 22 patients with chronic sensory polyneuropathy with ≥1 clinical sign atypical for chronic idiopathic axonal polyneuropathy (CIAP) but no electrodiagnostic criteria for CIDP.
Clinical signs atypical for CIAP were: sensory ataxia (59%), generalized areflexia (36%), cranial nerve involvement (32%), rapid upper limb involvement (40%), and age at onset ≤55 years (50%). Additional features were: normal sensory nerve action potentials (36%), abnormal radial/normal sural pattern (23%), abnormal somatosensory evoked potentials (SSEPs) (100%), elevated cerebrospinal fluid (CSF) protein (73%), and demyelinating features in 5/7 nerve biopsies. Over 90% of patients responded to immunotherapy. We conclude that all patients had sensory CIDP.
Sensory CIDP patients can be misdiagnosed as having CIAP. If atypical clinical/electrophysiologic features are present, we recommend performing SSEPs and CSF examination. Nerve biopsy should be restricted to disabled patients if other examinations are inconclusive.
感觉性慢性炎性脱髓鞘性多发性神经病（CIDP）的诊断可能较为困难。
我们报告了 22 例慢性感觉性多发性神经病患者，其具有≥1 项不符合慢性特发性轴索性多发性神经病（CIAP）的临床特征，但无 CIDP 的电诊断标准。
不符合 CIAP 的临床特征为：感觉性共济失调（59%）、全身反射消失（36%）、颅神经受累（32%）、上肢快速受累（40%）和发病年龄≤55 岁（50%）。其他特征包括：感觉神经动作电位正常（36%）、桡神经异常/腓肠神经正常（23%）、体感诱发电位异常（100%）、脑脊液（CSF）蛋白升高（73%）和 5/7 例神经活检有脱髓鞘表现。超过 90%的患者对免疫治疗有反应。我们得出结论，所有患者均患有感觉性 CIDP。
感觉性 CIDP 患者可能被误诊为患有 CIAP。如果存在不典型的临床/电生理特征，我们建议进行体感诱发电位和 CSF 检查。如果其他检查结果不确定，应仅对有残疾的患者进行神经活检。
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