Samile Noorbakhsh, Hassan Tonekaboni
Research Center of Pediatric Infectious Diseases, Iran University of Medical Sciences, Hazrat Rasool Hospital, Niayesh Ave, Satarkhan Street, Tehran 14455, Islamic Republic of Iran.
Saudi Med J. 2007 Mar;28(3):396-9.
To determine the frequency, etiology (viral infection or vaccination), presenting signs and symptoms, response to therapy, complication and course of acute disseminated encephalomyelitis (ADEM) in our hospitals.
A 2-year retrospective, descriptive, chart review of children with final diagnosis of ADEM in 2 hospitals (Hazrat Rasool and Mofid in Tehran, Iran during 2000-2002) were carried out. The diagnosis is based upon clinical presentation, physical examination and ruling out of other disease (imaging, laboratories and so forth) of expert pediatric neurologists. Acute disseminated encephalomyelitis was documented in all cases by characteristics MRI changes included inflammation and demyelination in subcortical or periventricular regions.
Acute disseminated encephalomyelitis were diagnosed in 15 patients. More than half of patients were between 9-14 years old. It was rare in 1-5 years old children. It had an abrupt onset, preceding infection/vaccination with no gender differences. Approximately 46.4% of cases had a recent upper respiratory tract illness. Varicella zoster virus infection, urinary tract infection, and mycoplasma pneumoniae were observed. Presentation signs included ataxia, decreased consciousness, fever plus nausea/vomiting, cranial nerve involvement, dysarthric speech, convulsion, hemiparesis, paresthesia, meningismus, and headache. We identified inflammation and demyelination in subcortical than periventricular lesions by magnetic resonance imaging. Prognosis was excellent with low mortality rate (6.6%).
Acute disseminated encephalomyelitis is common in our children, possibly because of the high prevalence of causative infections. Due to advances in control of traditional exanthematous diseases such as measle, rubella and so forth, most cases of ADEM in this study followed non-specific upper respiratory infections. Differentiation of ADEM from a single episode of multiple sclerosis is difficult. Diagnosis of multiple sclerosis should be carried out if new symptoms and signs or imaging abnormalities appear, more than 3 months after the onset of clinical symptoms in ADEM cases.
确定我院急性播散性脑脊髓炎（ADEM）的发病率、病因（病毒感染或疫苗接种）、临床表现、治疗反应、并发症及病程。
对2家医院（2000 - 2002年期间伊朗德黑兰的哈兹拉特·拉苏尔医院和莫菲德医院）最终诊断为ADEM的儿童进行了为期2年的回顾性、描述性病历审查。诊断基于临床表现、体格检查以及排除其他疾病（影像学、实验室检查等），由专业儿科神经科医生进行。所有病例均通过特征性MRI改变确诊为急性播散性脑脊髓炎，包括皮质下或脑室周围区域的炎症和脱髓鞘。
15例患者被诊断为急性播散性脑脊髓炎。超过半数患者年龄在9 - 14岁之间。1 - 5岁儿童中较为罕见。起病急，发病前有感染/疫苗接种史，无性别差异。约46.4%的病例近期有上呼吸道疾病史。观察到水痘带状疱疹病毒感染、尿路感染和肺炎支原体感染。临床表现包括共济失调、意识减退、发热伴恶心/呕吐、脑神经受累情况、构音障碍、惊厥、偏瘫、感觉异常、颈项强直和头痛。通过磁共振成像，我们发现皮质下病变比脑室周围病变的炎症和脱髓鞘更明显。预后良好，死亡率低（6.6%）。
急性播散性脑脊髓炎在我国儿童中较为常见，可能是由于致病感染的高发病率。由于麻疹、风疹等传统出疹性疾病防控取得进展，本研究中大多数ADEM病例继发于非特异性上呼吸道感染。ADEM与多发性硬化单次发作的鉴别困难。对于ADEM病例，如果在临床症状出现3个月后出现新的症状、体征或影像学异常，则应进行多发性硬化的诊断。
