From Assistance Publique-Hôpitaux de Paris (K.D., H.M., M.T.), Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Neuro-Inflammatory Diseases in Children, and University Paris Sud, Le Kremlin-Bicêtre, France; Children's Neurosciences (M.A., Y.H., M.L.), Evelina Children's Hospital at Guy's & St Thomas' NHS Foundation Trust, Kings Health Partners Academic Health Science Centre, London; the Department of Paediatric Neurology (C.H., G.N.), Great Ormond Street Hospital for Children, London, UK; Assistance Publique-Hôpitaux de Paris (B.H.), Hôpital Bicêtre, Pediatric Radiology Department, Le Kremlin-Bicêtre, France; and the Department of Paediatric Neurology (E.W.), Birmingham Children's Hospital, UK.
Neurology. 2015 Jan 27;84(4):341-9. doi: 10.1212/WNL.0000000000001179. Epub 2014 Dec 24.
To identify early prognostic factors of relapse and disability in children presenting with an acute idiopathic transverse myelitis (TM).
Ninety-five children with acute idiopathic TM from 2 national European cohorts (France and United Kingdom) of CNS demyelinating diseases in children were identified and studied for early factors that predict relapse and disability using logistic regression models.
Sixteen (17%) relapsed, with a diagnosis of multiple sclerosis in 13 (14%) and neuromyelitis optica in 3 (3%). Logistic regression revealed 2 main criteria as risk factors for relapse: female sex (odds ratio [OR] 3.21, 95% confidence interval [CI] 0.88-11.78) and presence of associated brain lesions (OR 14.0, 95% CI 2.8-69.3). Twenty-eight (30%) children had a poor outcome defined by a Kurtzke Expanded Disability Status Scale score ≥4 or an American Spinal Injury Association impairment (ASIA) scale <D at last follow-up. Five factors were associated with poor outcome: female sex (OR 5.8, 95% CI 0.99-32.7), severe ASIA scale at onset (OR 33.5, 95% CI 1.8-618), gadolinium enhancement on spinal MRI (OR 24.1, 95% CI 3.78-153.88), absence of pleocytosis (OR 4.6, 95% CI 0.87-26.0), and absence of cervical or cervico-thoracic lesion on spinal MRI (OR 3.9, 95% CI 0.78-19.6).
Early predictors of relapse and disability identified in this large childhood study of acute idiopathic TM have important utility for clinical management. Larger prospective collaborative studies are required to validate these findings, which may improve the design of clinical trials.
确定儿童急性特发性横贯性脊髓炎(TM)患者复发和残疾的早期预后因素。
从法国和英国的 2 个国家中枢神经系统脱髓鞘疾病儿童的欧洲队列研究(CNS)中,确定了 95 例急性特发性 TM 患儿,并使用逻辑回归模型研究了早期预测复发和残疾的因素。
16 例(17%)复发,其中 13 例(14%)诊断为多发性硬化症,3 例(3%)诊断为视神经脊髓炎。逻辑回归显示 2 个主要标准为复发的危险因素:女性(比值比[OR]3.21,95%置信区间[CI]0.88-11.78)和存在伴发脑病变(OR 14.0,95%CI 2.8-69.3)。28 例(30%)患儿预后不良,定义为 Kurtzke 扩展残疾状态量表评分≥4 分或美国脊髓损伤协会损伤(ASIA)评分<D。5 个因素与不良预后相关:女性(OR 5.8,95%CI 0.99-32.7)、发病时严重的 ASIA 量表评分(OR 33.5,95%CI 1.8-618)、脊髓 MRI 钆增强(OR 24.1,95%CI 3.78-153.88)、无细胞增多症(OR 4.6,95%CI 0.87-26.0)和脊髓 MRI 无颈或颈胸病变(OR 3.9,95%CI 0.78-19.6)。
本研究对儿童急性特发性 TM 进行了大型研究,确定了早期复发和残疾的预测因素,对临床管理具有重要的实用价值。需要更大规模的前瞻性协作研究来验证这些发现,这可能会改善临床试验的设计。