周围神经病理检查在慢性炎性脱髓鞘性多发性神经根神经病诊断中的价值

Diagnostic value of sural nerve biopsy in chronic inflammatory demyelinating poly-radiculoneuropathy

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 explore the diagnostic value of sural nerve biopsy for atypical chronic inflammatory demyelinating polyradiculoneuropathy （ CIDP ）. Methods All 12 patients with CIDP were studied. Retrospective analysis were performed on their clinical, electrophysiological data, and pathological characteristics. Results There were 9 cases misdiagnosed as subacute combined degeneration of the spinal cord, compressive myelopathy, cerebellar ataxia, neurofibromatosis, polymyositis, and progressive spinal muscular atrophy etc. Only 3 cases fulfilled the electrodiagnostic criteria of AAN （Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force ）, 6 cases had lower motor nerve conduction velocity （MCV）, but did not meet electrophysiological criteria for a diagnose of CIDP. 3 eases had normal MCV. Pathological examination found demyelination and decreased number of myelinated axons in all the 12 cases. Lymphocytes infdtration was also found in the endoneurium of 10 cases, supporting the diagnostic of C1DP. Conclusions CIDP should have heterogeneity in clinical manifestation, which might be responsible for its misdiagnosis. The sensitivity of AAN is limited, nerve biopsy is partially helpful in diagnosis, especially in atypical cases.