神经周围绕着周围神经的每个束,形成血液神经屏障的一部分。我们描述了它的正常解剖结构和功能。“神经膜炎”是指非特异性组织病理学发现和更具体的临床病理实体,原发性神经周炎(PP)。在进行神经活检之前,PP患者通常被认为患有非系统性血管性神经病。我们系统地回顾了有关PP的文献,并开发了组织病理学定义的神经周炎的鉴别诊断。我们搜索了PubMed,Embase,Scopus,和WebofScience的“神经周炎”。“我们确定了20例(11M/9F)PP:进行性,不明原因的神经病,活检显示神经周炎,无血管炎或其他已知的易感疾病。患者年龄在18至75岁之间(平均53.7岁),并且在诊断前有2-24个症状(中位数4.5个月)。神经病通常是感觉运动(15/20),痛苦(18/19)多焦(16/20),远端占优势(16/17),腿部比手臂更受影响。截头麻木发生在6/17;10/18脑脊液(CSF)蛋白升高。肌电图(EMG)和神经传导研究(NCS)主要显示轴突变化。神经活检显示T细胞为主的炎症,加宽,神经周纤维化;神经外膜浸润10/20,神经内膜浸润7/20;轴突变性不均匀。六个有上皮样细胞。19/20接受皮质类固醇,8与额外的免疫调节剂;18/19改善。两名患者对静脉注射免疫球蛋白(IVIg)无反应。在最后的后续行动中,13/16患者有轻度和2/16中度残疾;1/16死亡。神经膜炎的次要原因包括麻风病,血管炎,神经结节病,神经性伯利松病,神经淋巴瘤病,有毒油综合征,嗜酸性粒细胞增多-肌痛综合征,和罕见的条件。PP似乎是一种免疫介导的,皮质类固醇反应性障碍。它模仿非系统性血管神经病。有上皮样细胞的病例可能代表周围神经系统(PNS)限制形式的结节病。
The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood-nerve barrier. We describe its normal anatomy and function. \"
Perineuritis\" refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary
perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined
perineuritis. We searched PubMed, Embase, Scopus, and Web of Science for \"
perineuritis.\" We identified 20 cases (11 M/9F) of PP: progressive, unexplained neuropathy with biopsy showing
perineuritis without vasculitis or other known predisposing condition. Patients ranged in age from 18 to 75 (mean 53.7) y and had symptoms 2-24 (median 4.5) mo before diagnosis. Neuropathy was usually sensory-motor (15/20), painful (18/19), multifocal (16/20), and distal-predominant (16/17) with legs more affected than arms. Truncal numbness occurred in 6/17; 10/18 had elevated cerebrospinal fluid (CSF) protein. Electromyography (EMG) and nerve conduction studies (NCS) demonstrated primarily axonal changes. Nerve biopsies showed T-cell-predominant inflammation, widening, and fibrosis of perineurium; infiltrates in epineurium in 10/20 and endoneurium in 7/20; and non-uniform axonal degeneration. Six had epithelioid cells. 19/20 received corticosteroids, 8 with additional immunomodulators; 18/19 improved. Two patients did not respond to intravenous immunoglobulin (IVIg). At final follow-up, 13/16 patients had mild and 2/16 moderate disability; 1/16 died. Secondary causes of perineuritis include leprosy, vasculitis, neurosarcoidosis, neuroborreliosis, neurolymphomatosis, toxic oil syndrome, eosinophilia-myalgia syndrome, and rarer conditions. PP appears to be an immune-mediated, corticosteroid-responsive disorder. It mimics nonsystemic vasculitic neuropathy. Cases with epithelioid cells might represent peripheral nervous system (PNS)-restricted forms of sarcoidosis.