Helfferich Jelte, Bruijstens Arlette L, Wong Yu Yi M, Danielle van Pelt E, Boon Maartje, Neuteboom Rinze F
Department of Neurology, University Medical Center Groningen, PO Box 30001, 9700 RB Groningen, the Netherlands; Department of Neurology, Erasmus Medical Center, Room Ee-2230, PO Box 2040, 3000 CA Rotterdam, the Netherlands.
Department of Neurology, Erasmus Medical Center, Room Ee-2230, PO Box 2040, 3000 CA Rotterdam, the Netherlands.
Brain Dev. 2021 May;43(5):626-636. doi: 10.1016/j.braindev.2020.12.019. Epub 2021 Jan 26.
It may be difficult for clinicians to estimate the prognosis of pediatric acute transverse myelitis (ATM). The aim of this study was to define prognostic factors for relapsing disease and poor outcome in pediatric ATM.
This prospective cohort study included 49 children, 18 boys and 31 girls (median age 13.1 years, IQR 6.5-16.2) with a first episode of ATM. Factors associated with relapsing disease and poor outcome (Expanded Disability Status Scale (EDSS) ≥ 4) were assessed during a median follow-up of 37 months (IQR 18-75).
In total, 14 patients (29%) experienced ≥ 1 relapse(s) and nine patients (18%) had a poor outcome. Factors at onset associated with relapsing disease included higher age (16.1 vs. 11.6 years, p = 0.002), longer time to maximum severity of symptoms (5.5 vs. 3 days, p = 0.01), lower maximum EDSS score (4.0 vs. 6.5, p = 0.003), short lesion on spinal MRI (64 vs. 21%, p = 0.006), abnormalities on brain MRI (93 vs. 44%, p = 0.002) and presence of oligoclonal bands in cerebrospinal fluid (67 vs. 14%, p = 0.004). The only factor associated with poor outcome was presence of a spinal cord lesion on MRI without cervical involvement (56 vs. 14%, p = 0.02).
Pediatric ATM patients presenting with clinical, radiological and laboratory features associated with multiple sclerosis (MS) are at risk for relapsing disease. In absence of these known MS risk factors at onset of disease these patients are at low risk for relapses. Only a minority of pediatric ATM patients in this cohort have a poor outcome.
临床医生可能难以评估小儿急性横贯性脊髓炎（ATM）的预后。本研究的目的是确定小儿ATM复发疾病和不良预后的预测因素。
这项前瞻性队列研究纳入了49例首次发作ATM的儿童，其中18例男孩和31例女孩（中位年龄13.1岁，四分位间距6.5 - 16.2岁）。在中位随访37个月（四分位间距18 - 75个月）期间，评估与复发疾病和不良预后（扩展残疾状态量表（EDSS）≥4）相关的因素。
总共14例患者（29%）经历了≥1次复发，9例患者（18%）预后不良。发病时与复发疾病相关的因素包括年龄较大（16.1岁对11.6岁，p = 0.002）、达到症状最大严重程度的时间较长（5.5天对3天，p = 0.01）、最大EDSS评分较低（4.0对6.5，p = 0.003）、脊髓MRI上病变较短（64%对21%，p = 0.006）、脑MRI异常（93%对44%，p = 0.002）以及脑脊液中存在寡克隆带（67%对14%，p = 0.004）。与不良预后相关的唯一因素是MRI上存在脊髓病变且无颈部受累（56%对14%，p = 0.02）。
表现出与多发性硬化（MS）相关的临床、放射学和实验室特征的小儿ATM患者有疾病复发的风险。在疾病发作时若无这些已知的MS风险因素，这些患者复发风险较低。该队列中只有少数小儿ATM患者预后不良。
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