背景:中枢神经系统(CNS)的脱髓鞘疾病是罕见的疾病,其特征是炎症和CNS髓鞘的选择性破坏。在发达国家,这种疾病的发病率正在增加。尼日利亚关于儿科人群的研究非常稀缺。
目的:本研究的目的是记录流行病学,临床资料,以及晚期表现对儿科患者中枢神经系统脱髓鞘疾病治疗结果的影响。
方法:回顾性回顾了2018年1月至2022年12月在三级医院收治的年龄为1-15岁的患者,这些患者有各种症状提示脱髓鞘性中枢神经系统疾病。诊断得到临床和放射学证实。从病例记录中检索到的信息包括患者的人口统计学,临床症状和体征,在医院出现症状的天数,磁共振成像(MRI)的结果,治疗,和治疗结果。数据输入到Excel表格中,结果以表格和百分比呈现。
结果:在此期间脱髓鞘疾病的发生率为0.013%(在此期间769例患者中有10例)。急性脱髓鞘性脑脊髓炎(ADEM)是研究人群中最常见的疾病(60%,n=6),其次是横贯性脊髓炎和两个(20%)有视神经炎(ON)。大多数ADEM患者在1-5岁年龄组。男女比例为2.3:1。截瘫,视力障碍,共济失调是研究人群中最常见的临床表现.其中一名患者在随访期间符合多发性硬化症的诊断标准。在一例中,人类免疫缺陷病毒(HIV)被确定为脱髓鞘的原因。大多数患者使用类固醇改善。
结论:ADEM是本研究中最常见的临床表型。ADEM和ON患者的预后优于横贯性脊髓炎。晚期表现也被确定为不良预后因素。病例随访对于监测多发性硬化症的疾病进展非常重要。
BACKGROUND: Demyelinating disorders of the central nervous system (CNS) are rare disorders characterized by inflammation and the selective destruction of CNS myelin. The incidence of this disorder is increasing in developed countries. Nigerian studies on the pediatric population on the subject are very scarce.
OBJECTIVE: The aim of the study was to document the epidemiology, clinical profile, and impact of late presentation on the treatment outcome of demyelinating diseases of the CNS in pediatric patients.
METHODS: The retrospective review of patients aged 1-15 years admitted in a tertiary hospital from January 2018 to December 2022 with various symptoms suggestive of demyelinating CNS disorders. The diagnosis was clinically and radiologically confirmed. Information retrieved from the
case notes included patients\' demographics, clinical symptoms and signs, number of days with symptoms to presentation in the hospital, results of the magnetic resonance imaging (MRI), treatment, and treatment outcomes. Data were entered in Excel sheet and results were presented in tables and percentages.
RESULTS: The incidence of demyelinating disorders over the period was 0.013% (10 out of 769 patients admitted over the period). Acute demyelinating encephalomyelitis (ADEM) was the most common disorder seen in the study population (60%, n = 6), followed by transverse myelitis and two (20%) had optic neuritis (ON). Most of the patients with ADEM were in the 1-5-year age group. The female-to-male ratio was 2.3:1. Paraplegia, visual impairment, and ataxia were the most common clinical presentations in the study population. One of the patients met the criteria for the diagnosis of multiple sclerosis during follow-up. Human immunodeficiency virus (HIV) was identified as the cause of demyelination in one
case. Most of the patients improved with steroids.
CONCLUSIONS: ADEM was the most common clinical phenotype seen in this study. Patients with ADEM and ON had a better prognosis than transverse myelitis. Late presentation was also identified as a poor prognostic factor. Follow-up of cases is very important to monitor disease progression to multiple sclerosis.