Antoine Jean-Christophe, Robert-Varvat Florence, Maisonobe Thierry, Créange Alain, Franques Jérôme, Mathis Stéphane, Delmont Emilien, Kuntzer Thierry, Lefaucheur Jean-Pascal, Pouget Jean, Viala Karine, Desnuelle Claude, Echaniz-Laguna Andoni, Rotolo Francesco, Camdessanché Jean-Philippe
Centre de Référence Maladies Neuromusculaires Rares Rhône-Alpes, CHU de Saint-Etienne, France; Université de Lyon, Saint-Etienne, France; INSERM U1028, Centre des Neurosciences de Lyon, Lyon, France.
Centre de Référence Maladies Neuromusculaires Rares Rhône-Alpes, CHU de Saint-Etienne, France.
J Neurol Sci. 2016 Feb 15;361:187-91. doi: 10.1016/j.jns.2015.12.044. Epub 2015 Dec 29.
Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration.
Eighty-six patients with acute/subacute inflammatory SNN (paraneoplastic, associated with dysimmune diseases, or idiopathic) were retrospectively studied. The monthly SNAP reduction was determined and normalized with the lower limit of normal. Disability progression was expressed by the modified Rankin score and correlated with SNAP reduction.
The monthly SNAP reduction was similar in the four limbs although the median nerve was less severely affected. The monthly SNAP reduction was very severe within the first two months of evolution, began to slow down after seven months, and stabilized after ten months. It was tightly correlated with disability progression. Kaplan-Meier analysis showed that the median time until matching the diagnostic criteria of SNN was 8.5 months. Within this period, 42% of nerves remained excitable.
Developing treatment aiming at the stabilization of SNN is possible within the first 8 months of evolution. An improvement of the disease is possible if patients are treated within two months, which needs an early referral to an expert center and ENMG testing of the radial and ulnar nerves, which are most sensitive to changes.
炎性感觉神经元病（SNN）患者若能及时接受免疫调节或免疫抑制治疗，可能会从中受益。了解背根神经节中神经元丢失的时间特征，将有助于确定在不可逆的神经元损伤发生之前是否能够做出最终诊断。因此，我们通过使用感觉神经动作电位（SNAPs）作为神经元变性的替代标志物，来研究SNN中神经元丢失的演变情况。
对86例急性/亚急性炎性SNN患者（副肿瘤性、与免疫失调疾病相关或特发性）进行回顾性研究。确定每月SNAP的降低情况，并以正常下限进行标准化。残疾进展用改良Rankin评分表示，并与SNAP降低情况相关联。
尽管正中神经受影响程度较轻，但四肢的每月SNAP降低情况相似。在病程的前两个月内，每月SNAP降低非常严重，七个月后开始放缓，十个月后趋于稳定。它与残疾进展密切相关。Kaplan-Meier分析显示，达到SNN诊断标准的中位时间为8.5个月。在此期间，42%的神经仍可兴奋。
在病程的前8个月内，有可能制定出旨在稳定SNN的治疗方案。如果患者在两个月内接受治疗，病情有可能得到改善，这需要尽早转诊至专家中心，并对桡神经和尺神经进行ENMG检测，这两条神经对变化最为敏感。
