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目的探讨周围神经病理检查在不典型慢性炎性脱髓鞘性多发性神经根神经病(CIDP)诊断中的价值。方法收集12例CIDP患者的临床和电生理资料,结合病理检查结果,评价周围神经活检在诊断CIDP中的价值。结果12例患者中有9例分别误诊为亚急性联合变性2例,颈、腰椎间盘突出,小脑性共济失调,多发性神经纤维瘤,肌炎,进行性脊肌萎缩症和末梢神经炎各1例。电生理检查12例患者中有9例未达到CIDP的诊断标准,但全部患者的病理检查均有不同程度的大、中型有髓纤维减少和髓鞘脱失,其中10例有淋巴细胞浸润,支持炎性脱髓鞘性神经病的诊断。结论CIDP的电生理诊断标准有可能会导致误诊;临床疑为CIDP而电生理不支持时,周围神经活检可提供一定的帮助。
Objective To investigate the value of peripheral neuropathology in the diagnosis of atypical chronic inflammatory demyelinating polyarticular nerve root disease (CIDP). Methods The clinical and electrophysiological data of 12 patients with CIDP were collected. Combined with the pathological findings, the value of peripheral nerve biopsy in the diagnosis of CIDP was evaluated. Results Of the 12 patients, 9 cases were misdiagnosed as subacute combined degeneration in 2 cases, cervical and lumbar disc herniation, cerebellar ataxia, multiple neurofibromatosis, myositis, progressive spinal muscular atrophy and peripheral neuritis example. Electrophysiological examination, 9 of 12 patients did not meet the diagnostic criteria of CIDP, but all patients had varying degrees of large and medium-sized myelinated fibers and demyelination, of which 10 cases of lymphocyte infiltration, support Diagnosis of inflammatory demyelinating neuropathy. Conclusion Electrophysiological diagnostic criteria of CIDP may lead to misdiagnosis. When clinical suspicion of CIDP is not supported by electrophysiology, peripheral nerve biopsy may provide some help.