Ben Achour N, Ben Waddey O, Kraoua I, Benrhouma H, Klaa H, Rouissi A, Ben Youssef-Turki I
UR12SP24 et service de neurologie de l'enfant et de l'adolescent, Institut national Mongi Ben Hmida de neurologie, La Rabta, 1007 Tunis, Tunisie.
UR12SP24 et service de neurologie de l'enfant et de l'adolescent, Institut national Mongi Ben Hmida de neurologie, La Rabta, 1007 Tunis, Tunisie.
Rev Neurol (Paris). 2015 Dec;171(12):882-90. doi: 10.1016/j.neurol.2015.09.011. Epub 2015 Nov 11.
Acute disseminated encephalomyelitis (ADEM) is an inflammatory, demyelinating disorder of the central nervous system whose clinical features, management and outcome are incompletely understood in Tunisian population.
To describe clinical, neuroimaging and laboratory features; treatment and outcome in a cohort of Tunisian children with ADEM.
We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with ADEM between 2005 and 2015. Clinical, neuroimaging and laboratory features, therapeutic data and outcome were analyzed.
There were 15 children (7 males and 8 females). The mean age at onset was 6.9 years. Thirteen (86.6%) patients had a prodromal event. The onset of neurological symptoms occurred within 17.6 days (4-30). Limb weakness was the most common presenting symptom (53.3%). Extrapyramidal syndrome was noticed in 6 patients (40%). Initial MRI showed a deep gray matter involvement in 7 cases (46.6%). Gadolinium enhancement at acute stage was observed in only 2 patients (13%). Cerebrospinal fluid findings did not show intrathecal oligoclonal bands. The use of high-dose IV methylprednisolone followed by oral steroid taper was associated with rapid recovery. Additional treatment with intravenous immunoglobulin was necessary in 2 patients. Complete recovery was obtained in 11 patients (73.3%). A monophasic course was noticed in 14 cases. Only one patient (5%) developed multiple sclerosis.
The high frequency of prodromal events and extrapyramidal syndrome in addition to the low rate of gadolinium enhancement at acute stage seem to be the main features in our patients. Larger ADEM multicenter cohort studies in Tunisia and North Africa could provide more detailed information about this entity.
急性播散性脑脊髓炎（ADEM）是一种中枢神经系统的炎症性脱髓鞘疾病，其临床特征、治疗方法及预后在突尼斯人群中尚未完全明确。
描述一组突尼斯患ADEM儿童的临床、神经影像学及实验室特征、治疗方法及预后情况。
我们对2005年至2015年间在突尼斯儿童与青少年神经科就诊的所有患ADEM儿童的病历进行了回顾性分析。分析了临床、神经影像学及实验室特征、治疗数据及预后情况。
共15名儿童（7名男性，8名女性）。发病时的平均年龄为6.9岁。13名（86.6%）患者有前驱事件。神经症状在17.6天内（4 - 30天）出现。肢体无力是最常见的首发症状（53.3%）。6名患者（40%）出现锥体外系综合征。初始MRI显示7例（46.6%）有深部灰质受累。急性期仅2例（13%）观察到钆增强。脑脊液检查未发现鞘内寡克隆带。使用大剂量静脉注射甲泼尼龙后逐渐减量口服类固醇与快速恢复相关。2例患者需要额外静脉注射免疫球蛋白治疗。11例患者（73.3%）完全康复。14例呈单相病程。仅1例患者（5%）发展为多发性硬化。
前驱事件和锥体外系综合征的高发生率以及急性期钆增强率低似乎是我们患者的主要特征。在突尼斯和北非开展更大规模的ADEM多中心队列研究可以提供有关该疾病实体的更详细信息。
