Verschueren A
Service de neurologie et des maladies neuromusculaires, Hôpital de la Timone, 265, rue Saint-Pierre, 13005 Marseille, France.
Rev Neurol (Paris). 2007 Sep;163 Spec No 1:3S58-60.
Diagnostic value of nerve biopsy in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been widely discussed and indication has markedly decreased since 20 years. Diagnosis of CIDP is usually based on clinical features (progressive or relapsing course, symmetrical motor and sensory neuropathy in arms and legs, hypo or areflexia usually involve all four limbs), raised CSF protein concentration and nerve conduction studies consistent with demyelination. Several electrophysiological criteria for CIDP have been proposed. We presented a case of sensory-motor neuropathy with examination compatible with CIDP. Cerebrospinal fluid protein content has been slightly elevated. Electrophysiological criteria have been lacking due to absence of recorded compound motor action potential or sensory nerve action potential in lower limbs and absence of demyelination features in upper limbs. Sural nerve biopsy has allowed to show significant demyelination on microscopic and teased nerve fibers analysis. A diagnosis of CIDP has been kept and immunomodulatory treatment has been discussed. Intravenous immune globulin treatment has been effective in this patient. Nerve biopsy stay useful for confirmation of CIDP in front of clinical features compatible with the diagnosis but without usual biological and electrophysiological criteria. The confirmation of diagnosis is significant and can lead to effective treatment.
神经活检在慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)诊断中的价值已得到广泛讨论,自20年来其适应证已显著减少。CIDP的诊断通常基于临床特征(进行性或复发性病程、四肢对称性运动和感觉神经病变、通常累及四肢的低反射或无反射)、脑脊液蛋白浓度升高以及与脱髓鞘相符的神经传导研究。已经提出了几种CIDP的电生理标准。我们报告了一例感觉运动性神经病变病例,其检查结果与CIDP相符。脑脊液蛋白含量略有升高。由于下肢未记录到复合运动动作电位或感觉神经动作电位,且上肢无脱髓鞘特征,因此缺乏电生理标准。腓肠神经活检在显微镜检查和神经纤维分离分析中显示出明显的脱髓鞘。维持了CIDP的诊断并讨论了免疫调节治疗。静脉注射免疫球蛋白治疗对该患者有效。在临床特征与诊断相符但无常规生物学和电生理标准的情况下,神经活检对于CIDP的确诊仍然有用。诊断的确认很重要,可导致有效的治疗。