Boesen Magnus Spangsberg, Blinkenberg Morten, Born Alfred Peter, Magyari Melinda, Chitnis Tanuja, Thygesen Lau Caspar, Langkilde Annika Reynberg
Department of Pediatrics, Rigshospitalet, University of Copenhagen, Denmark; Department of Neurology, Herlev Hospital, Denmark.
Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet, University of Copenhagen, Denmark.
Mult Scler Relat Disord. 2020 Nov;46:102590. doi: 10.1016/j.msard.2020.102590. Epub 2020 Oct 21.
It is essential to distinguish acute disseminated encephalomyelitis (ADEM) from MS early. Our aim was to determine MRI features at baseline and follow-up to distinguish pediatric ADEM from MS stratified according to age at onset.
Using hospital ICD-10 codes for acquired demyelinating syndromes from a nationwide register and subsequent chart review, we identified 52 children (<18 years) with ADEM and 66 children with MS. We undertook a retrospective analysis of MRI scans at onset and at follow-up. The MRI rater was a senior neuroradiologist blinded to clinical characteristics.
At baseline, children with ADEM had more diffuse poorly demarcated lesions, particularly in the basal ganglia/thalamus (p = 0.001) and cerebellar peduncles (p < 0.0001). Further, longitudinal extensive transverse myelitis was strongly associated with ADEM (p<0.0001). Children with ADEM had fewer contrast-enhancing lesions (p = 0.0004), occipital lesions (p = 0.01), optic nerve lesions (p = 0.01), periventricular lesions, well-defined lesions only (p<0.0001), and fewer fulfilled dissemination in time according to the McDonald 2017 criteria (p = 0.005). On baseline MRI, dissemination in space and time was fulfilled in 17% of children with ADEM and in 34% of children with MS (p = 0.06), and 60% of children with ADEM fulfilled the criterion for dissemination in space. The mean time from baseline MRI to follow-up MRI was 1.0 year for children with ADEM and 2.1 years for children with MS. On follow-up MRI, 85% of children with ADEM had partial or complete T2 lesion resolution, but in the 58% without complete resolution lesions were predominantly frontal. Only 47% of children with MS had partial or complete T2 lesion resolution, and therefore more MRI features differed between children with ADEM and MS on follow-up. MRI had the greatest distinguishing value after age 11 years because MS is exceptional in the first decade of life.
Age at onset and the timing of MRI in relation to disease onset are critical in the interpretation of MRI to distinguish between ADEM and MS.
早期区分急性播散性脑脊髓炎（ADEM）和多发性硬化症（MS）至关重要。我们的目的是确定基线和随访时的MRI特征，以区分根据发病年龄分层的儿童ADEM和MS。
利用全国登记处获取的脱髓鞘综合征的医院ICD - 10编码以及随后的病历审查，我们确定了52名患有ADEM的儿童（<18岁）和66名患有MS的儿童。我们对发病时和随访时的MRI扫描进行了回顾性分析。MRI评估者是一位对临床特征不知情的资深神经放射科医生。
在基线时，患有ADEM的儿童有更多弥漫性边界不清的病变，特别是在基底节/丘脑（p = 0.001）和小脑脚（p < 0.0001）。此外，纵向广泛横贯性脊髓炎与ADEM密切相关（p<0.0001）。患有ADEM的儿童有较少的强化病变（p = 0.0004）、枕叶病变（p = 0.01）、视神经病变（p = 0.01）、脑室周围病变、仅边界清晰的病变（p<0.0001），并且根据2017年麦克唐纳标准满足时间上播散的患儿较少（p = 0.005）。在基线MRI上，17%的ADEM患儿和34%的MS患儿满足时间和空间上的播散（p = 0.06），60%的ADEM患儿满足空间上播散的标准。患有ADEM的儿童从基线MRI到随访MRI的平均时间为1.0年，患有MS的儿童为2.1年。在随访MRI上，85%的ADEM患儿有部分或完全的T2病变消退，但在未完全消退的患儿中，病变主要位于额叶。只有47%的MS患儿有部分或完全的T2病变消退，因此在随访时，ADEM患儿和MS患儿之间更多的MRI特征存在差异。由于MS在生命的第一个十年中较为罕见，所以11岁以后MRI具有最大的鉴别价值。
发病年龄以及MRI与疾病发病的时间关系对于解释MRI以区分ADEM和MS至关重要。
