Child Neuropsychiatry Unit, Department of Medical, Surgical and Experimental Sciences, University Hospital of Sassari, Sassari, Italy.
Multiple Sclerosis Centre, Clinical Neurology Unit, Department of Medical, Surgical and Experimental Sciences, University Hospital of Sassari, Sassari, Italy.
Dev Med Child Neurol. 2021 Sep;63(9):1059-1065. doi: 10.1111/dmcn.14912. Epub 2021 May 3.
We assessed the frequency, characteristics, and future trajectory of monophasic acquired demyelinating syndromes (ADS) associated with conversion to paediatric multiple sclerosis.
This was a retrospective observational study of Sardinian children (<18y of age) with onset of ADS between 2001 and 2018.
We identified 44 children with ADS (21 males, 23 females; median age at onset 16y, range 4mo-18y), 21 of whom were already presenting with criteria for paediatric multiple sclerosis. The mean crude prevalence of ADS in Sardinian children was 59.2 per 100 000, while incidence was 3.1 per 100 000 per year (1.3 in children aged ≤10y and 11.9 in those aged 10-17y). After a mean (SD) follow-up of 8 years 5 months (5y 4mo), the most common (n=32) trajectory was conversion to paediatric multiple sclerosis. At onset, the total prevalence and mean annual incidence of paediatric multiple sclerosis were 35.6 per 100 000 and 2.3 per 100 000 respectively (0.5 in individuals aged ≤10y, 10.0 in the older group).
Sardinia is a very high risk area for ADS in children. Nearly half of this population can already be diagnosed with paediatric multiple sclerosis at onset. Overall, 72% of those with ADS will have paediatric multiple sclerosis after a mean of 8 years. What this paper adds Sardinia is a very high risk area for paediatric acquired demyelinating syndromes (ADS). A high proportion of those with paediatric multiple sclerosis are diagnosed at onset of ADS. After an average 8 years from onset of paediatric ADS, three-quarters of patients are diagnosed with paediatric multiple sclerosis.
我们评估了单相获得性脱髓鞘综合征（ADS）与儿童多发性硬化症转化相关的频率、特征和未来轨迹。
这是一项对 2001 年至 2018 年间在撒丁岛发病的 ADS 儿童（<18 岁）进行的回顾性观察研究。
我们确定了 44 名 ADS 儿童（21 名男性，23 名女性；发病时的中位年龄为 16 岁，范围为 4 个月至 18 岁），其中 21 名已经符合儿童多发性硬化症的标准。撒丁岛儿童中 ADS 的粗患病率为 59.2/100000，年发病率为 3.1/100000（<10 岁儿童为 1.3，10-17 岁儿童为 11.9）。平均（SD）随访 8 年 5 个月（5 年 4 个月）后，最常见（n=32）的轨迹是转化为儿童多发性硬化症。发病时，儿童多发性硬化症的总患病率和平均年发病率分别为 35.6/100000 和 2.3/100000（<10 岁儿童为 0.5，年龄较大的儿童为 10.0）。
撒丁岛是儿童 ADS 的高风险地区。近一半的患者在发病时即可被诊断为儿童多发性硬化症。总体而言，在平均 8 年后，72%的 ADS 患者将被诊断为儿童多发性硬化症。
撒丁岛是儿童获得性脱髓鞘综合征（ADS）的高风险地区。相当一部分多发性硬化症患者在 ADS 发病时即被诊断。从儿童 ADS 发病平均 8 年后，四分之三的患者被诊断为儿童多发性硬化症。
