Banwell B, Kennedy J, Sadovnick D, Arnold D L, Magalhaes S, Wambera K, Connolly M B, Yager J, Mah J K, Shah N, Sebire G, Meaney B, Dilenge M-E, Lortie A, Whiting S, Doja A, Levin S, MacDonald E A, Meek D, Wood E, Lowry N, Buckley D, Yim C, Awuku M, Guimond C, Cooper P, Grand'Maison F, Baird J B, Bhan V, Bar-Or A
Research Institute, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, M5G 1X8 Canada.
Neurology. 2009 Jan 20;72(3):232-9. doi: 10.1212/01.wnl.0000339482.84392.bd.
The incidence of acquired demyelination of the CNS (acquired demyelinating syndromes [ADS]) in children is unknown. It is important that physicians recognize the features of ADS to facilitate care and to appreciate the future risk of multiple sclerosis (MS).
To determine the incidence, clinical features, familial autoimmune history, and acute management of Canadian children with ADS.
Incidence and case-specific data were obtained through the Canadian Pediatric Surveillance Program from April 1, 2004, to March 31, 2007. Before study initiation, a survey was sent to all pediatric health care providers to determine awareness of MS as a potential outcome of ADS in children.
Two hundred nineteen children with ADS (mean age 10.5 years, range 0.66-18.0 years; female to male ratio 1.09:1) were reported. The most common presentations were optic neuritis (ON; n = 51, 23%), acute disseminated encephalomyelitis (ADEM; n = 49, 22%), and transverse myelitis (TM; n = 48, 22%). Children with ADEM were more likely to be younger than 10 years, whereas children with monolesional ADS (ON, TM, other) were more likely to be older than 10 years (p < 0.001). There were 73 incident cases per year, leading to an annual incidence of 0.9 per 100,000 Canadian children. A family history of MS was reported in 8%. Before study initiation, 65% of physicians indicated that they considered MS as a possible outcome of ADS in children. This increased to 74% in year 1, 81% in year 2, and 87% in year 3.
The incidence of pediatric acquired demyelinating syndromes (ADS) is 0.9 per 100,000 Canadian children. ADS presentations are influenced by age.
儿童中枢神经系统获得性脱髓鞘疾病（获得性脱髓鞘综合征[ADS]）的发病率尚不清楚。医生认识到ADS的特征对于促进治疗以及了解多发性硬化症（MS）的未来风险非常重要。
确定加拿大患有ADS的儿童的发病率、临床特征、家族自身免疫病史及急性处理情况。
通过加拿大儿科监测项目获取2004年4月1日至2007年3月31日期间的发病率及病例特异性数据。在研究开始前，向所有儿科医疗服务提供者发送了一份调查问卷，以确定他们对MS作为儿童ADS潜在后果的认知情况。
共报告了219例患有ADS的儿童（平均年龄10.5岁，范围0.66 - 18.0岁；男女比例为1.09:1）。最常见的表现为视神经炎（ON；n = 51，23%）、急性播散性脑脊髓炎（ADEM；n = 49，22%）和横贯性脊髓炎（TM；n = 48，22%）。患有ADEM的儿童更可能小于10岁，而患有单病灶ADS（ON、TM、其他）的儿童更可能大于10岁（p < 0.001）。每年有73例新发病例，导致加拿大儿童的年发病率为每100,000人中有0.9例。8%的患儿有MS家族史。在研究开始前，65%的医生表示他们认为MS是儿童ADS的一种可能后果。这一比例在第1年升至74%，第2年升至81%，第3年升至87%。
加拿大儿童获得性脱髓鞘综合征（ADS）的发病率为每100,000人中有0.9例。ADS的表现受年龄影响。
