Marchioni E, Ravaglia S, Piccolo G, Furione M, Zardini E, Franciotta D, Alfonsi E, Minoli L, Romani A, Todeschini A, Uggetti C, Tavazzi E, Ceroni M
Institute of Neurology IRCCS C. Mondino, University of Pavia, Via Mondino 2, 27100 Pavia, Italy.
Neurology. 2005 Oct 11;65(7):1057-65. doi: 10.1212/01.wnl.0000179302.93960.ad.
Acute disseminated encephalomyelitis (ADEM) refers to a monophasic acute multifocal inflammatory CNS disease. However, both relapsing and site-restricted variants, possibly associated with peripheral nervous system (PNS) involvement, are also observed, and a systematic classification is lacking.
To describe a cohort of postinfectious ADEM patients, to propose a classification based on clinical and instrumental features, and to identify subgroups of patients with different prognostic factors.
Inpatients of a Neurologic and Infectious Disease Clinic affected by postinfectious CNS syndrome consecutively admitted over 5 years were studied.
Of 75 patients enrolled, 60 fulfilled criteria for ADEM after follow-up lasting from 24 months to 7 years. Based on lesion distribution, patients were classified as encephalitis (20%), myelitis (23.3%), encephalomyelitis (13.3%), encephalomyeloradiculoneuritis (26.7%), and myeloradiculoneuritis (16.7%). Thirty patients (50%) had a favorable outcome. Fifteen patients (25%) showed a relapsing course. Poor outcome was related with older age at onset, female gender, elevated CSF proteins, and spinal cord and PNS involvement. All but two patients received high-dose steroids as first-line treatment, with a positive response in 39 (67%). Ten of 19 nonresponders (53%) benefited from high-dose IV immunoglobulin; 9 of 10 had PNS involvement. The data were not controlled.
A high prevalence of "atypical variants" was found in this series, with site-restricted damage or additional peripheral nervous system (PNS) involvement. Prognosis and response to steroids were generally good, except for some patient subgroups. In patients with PNS involvement and steroid failure, a favorable effect of IV immunoglobulin was observed.
急性播散性脑脊髓炎（ADEM）是一种单相性急性多灶性炎症性中枢神经系统疾病。然而，也观察到了可能与外周神经系统（PNS）受累相关的复发型和部位受限型变异，且缺乏系统的分类。
描述一组感染后ADEM患者，基于临床和影像学特征提出一种分类方法，并识别具有不同预后因素的患者亚组。
对一家神经科和传染病诊所5年内连续收治的感染后中枢神经系统综合征住院患者进行研究。
在纳入的75例患者中，60例在随访24个月至7年后符合ADEM标准。根据病变分布，患者分为脑炎型（20%）、脊髓炎型（23.3%）、脑脊髓炎型（13.3%）、脑脊髓神经根神经炎型（26.7%）和脊髓神经根神经炎型（16.7%）。30例患者（50%）预后良好。15例患者（25%）呈复发病程。预后不良与发病时年龄较大、女性、脑脊液蛋白升高以及脊髓和PNS受累有关。除2例患者外，所有患者均接受大剂量类固醇作为一线治疗，39例（67%）有阳性反应。19例无反应者中有10例（53%）从大剂量静脉注射免疫球蛋白中获益；10例中有9例有PNS受累。数据未进行对照。
本系列研究发现 “非典型变异型” 的患病率较高，存在部位受限性损害或额外的外周神经系统（PNS）受累。除了一些患者亚组外，预后和对类固醇的反应总体良好。在PNS受累且类固醇治疗无效的患者中，观察到静脉注射免疫球蛋白有良好效果。
