Vallat Jean-Michel, Mathis Stéphane, Ghorab Karima, Milor Michel-André, Richard Laurence, Magy Laurent
Department of Neurology, National Referral Center for 'Rare Peripheral Neuropathies', University Hospital Limoges, Limoges, France.
Eur Neurol. 2015;73(5-6):294-302. doi: 10.1159/000381767. Epub 2015 Apr 29.
Several treatments are available to treat the immune-mediated chronic inflammatory demyelinating polyneuropathy (CIDP). Among these treatments, intravenous immunoglobulins, corticosteroids and plasma exchanges are validated and widely used. A few immunosuppressive drugs have been tried, but they had little efficiency.
We describe three CIDP patients treated by Natalizumab (acting against cellular adhesion and T-cell migration) after a failure of the validated treatments.
We observed a long-term improvement in one patient, a dramatic improvement over a significant duration in another patient and stabilization in the last one.
This open label study provides evidence for the value of Natalizumab as second-line treatment for individual patients with a high dependency on waning efficacy of first-line therapies. CIDP is characterized by heterogeneity of clinical phenotypes, electrophysiological and pathological features, and various variable courses types of evolution. The different responses to drugs of our patients are consistent with some reported cases and may reflect the spectrum of lesional mechanisms and the molecular dysfunctions in CIDP.
有多种治疗方法可用于治疗免疫介导的慢性炎性脱髓鞘性多发性神经病（CIDP）。在这些治疗方法中，静脉注射免疫球蛋白、皮质类固醇和血浆置换已得到验证并被广泛使用。已经尝试了一些免疫抑制药物，但效果不佳。
我们描述了3例在经过验证的治疗失败后接受那他珠单抗（作用于细胞黏附和T细胞迁移）治疗的CIDP患者。
我们观察到1例患者长期改善，另1例患者在相当长的一段时间内显著改善，最后1例患者病情稳定。
这项开放标签研究为那他珠单抗作为一线治疗疗效逐渐减弱且高度依赖的个体患者的二线治疗价值提供了证据。CIDP的特征是临床表型、电生理和病理特征的异质性以及各种不同的病程演变类型。我们患者对药物的不同反应与一些报道的病例一致，可能反映了CIDP中病变机制和分子功能障碍的范围。
