Vrancken Alexander F J E, Notermans Nicolette C, Jansen Gerard H, Wokke John H J, Said Gérard
Service de Neurologie et Laboratoire Louis Ranvier, Centre Hospitalier Universitaire de Bicêtre, Université Paris Sud XI, Le Kremlin-Bicêtre, France.
J Neurol. 2004 Mar;251(3):269-78. doi: 10.1007/s00415-004-0275-9.
Patients with a progressive disabling idiopathic axonal neuropathy could have a potentially treatable immune mediated neuropathy. To evaluate whether progressive idiopathic axonal neuropathy could be a pathologically difficult to prove vasculitic neuropathy pathologically difficult to prove or if it could be a separate clinical entity (i. e. with the axon as the primary immunological target), we performed a comparative clinical and histopathological study in 10 patients with progressive idiopathic axonal neuropathy, 10 patients with vasculitic neuropathy, and 12 patients with chronic idiopathic axonal polyneuropathy (CIAP). The clinical features and disease course in patients with progressive idiopathic axonal neuropathy and patients with vasculitic neuropathy were similar. Six patients with progressive idiopathic axonal neuropathy had been treated with prednisone and/or intravenous immunoglobulin. Disability decreased in all these six patients, but also in two of the four non-treated patients. Upon reviewing the sural nerve biopsy specimens, vasculitis was found in one patient with progressive idiopathic axonal neuropathy. Vasculitis-associated signs of ischemic injury or inflammation (most notably: large variation in fascicular axonal degeneration, perivascular inflammation, inflammation of the blood vessel wall without lumen obstruction) were found in four patients with progressive idiopathic axonal neuropathy, in all patients with vasculitic neuropathy, but were absent in patients with CIAP. The findings show that there is a small chance of finding sural nerve vasculitis upon scrutinising biopsy examination in progressive idiopathic axonal neuropathy. The presence of vasculitis-associated signs in progressive idiopathic axonal neuropathy suggests that some of these patients could have vasculitic neuropathy, even if vasculitic lesions cannot be demonstrated. However, if inflammatory changes cannot be demonstrated this does not preclude an immune-mediated origin.
患有进行性致残性特发性轴索性神经病的患者可能患有潜在可治疗的免疫介导性神经病。为了评估进行性特发性轴索性神经病是否在病理上难以证明为血管炎性神经病，或者它是否可能是一个独立的临床实体（即以轴突作为主要免疫靶点），我们对10例进行性特发性轴索性神经病患者、10例血管炎性神经病患者和12例慢性特发性轴索性多发性神经病（CIAP）患者进行了比较临床和组织病理学研究。进行性特发性轴索性神经病患者和血管炎性神经病患者的临床特征和病程相似。6例进行性特发性轴索性神经病患者接受了泼尼松和/或静脉注射免疫球蛋白治疗。所有这6例患者的残疾程度均有所减轻，但4例未治疗患者中的2例也有减轻。在复查腓肠神经活检标本时，在1例进行性特发性轴索性神经病患者中发现了血管炎。在4例进行性特发性轴索性神经病患者、所有血管炎性神经病患者中发现了与血管炎相关的缺血性损伤或炎症迹象（最显著的是：束状轴突变性差异大、血管周围炎症、血管壁炎症而无管腔阻塞），但CIAP患者中未发现。研究结果表明，在仔细检查进行性特发性轴索性神经病的活检检查时，发现腓肠神经血管炎的可能性很小。进行性特发性轴索性神经病中存在与血管炎相关的迹象表明，即使无法证明血管炎性病变，这些患者中的一些可能患有血管炎性神经病。然而，如果无法证明炎症变化，这并不排除免疫介导的起源。
Zotero 插件