From the Section of Pediatric Neurology (M.F.R.), Department of Pediatrics and Child Health, University of Manitoba, Children's Hospital Research Institute of Manitoba, Winnipeg, Canada; Department of Neurology and Pediatrics (K.A.S., H.J.F.), University of California, San Francisco; University of Toronto (A.-M.S.); Division of Neurology (G.A.d.V., N.D., A.L.), The Hospital for Sick Children, University of Toronto, Ontario; Department of Pediatrics and Clinical Neurosciences (A.K.), Cumming School of Medicine, University of Calgary, Alberta, Canada; Department of Neurology (C.A.-L.), University of Washington, Seattle; Department of Neuropediatrics (B.W.), Charité University Medicine Berlin, Germany; Department of Neurology (J.L.C.), George Washington University, Washington, DC; Department of Neurology (M.T.M.), Royal Children's Hospital Melbourne, Murdoch Children's Research Institute and University of Melbourne, Australia; Boston Children's Hospital (M.R.), Harvard Medical School, Boston, MA; and Division of Child Neurology (T.J.B.), Department of Pediatrics and the Hemophilia and Thrombosis Center, University of Colorado, Denver.
Neurology. 2020 Jun 9;94(23):e2479-e2490. doi: 10.1212/WNL.0000000000009557. Epub 2020 May 26.
To determine that children with arterial ischemic stroke (AIS) due to an identifiable arteriopathy are distinct from those without arteriopathy and that each arteriopathy subtype has unique and recognizable clinical features.
We report a large, observational, multicenter cohort of children with AIS, age 1 month to 18 years, enrolled in the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared by use of the Fisher exact test, with linear step-up permutation min- adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between cases of AIS with and without arteriopathy and between arteriopathy subtypes.
Of 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy-inflammatory subtype (FCA-i; 15%), diffuse cerebral vasculitis (15%), and nonspecific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6 and 9 years of age (odds ratio [OR] 1.93, = 0.029) with headache (OR 1.55, = 0.023), multiple infarctions (OR 2.05, < 0.001), sickle cell anemia (OR 2.9, = 0.007), and head/neck trauma (OR 1.93, = 0.018). Antithrombotic use and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, dissection was associated with male sex, older age, headache, and anticoagulant use; FCA-i was associated with hemiparesis and single infarcts; moyamoya was associated with seizures and recurrent strokes; and vasculitis was associated with bilateral infarctions.
Specific clinical profiles are associated with cerebral arteriopathies in children with AIS. These observations may be helpful indicators in guiding early diagnosis and defining subgroups who may benefit most from future therapeutic trials.
确定由可识别的血管病变引起的儿童动脉缺血性卒中(AIS)与无血管病变的儿童明显不同,并且每种血管病变亚型都具有独特且可识别的临床特征。
我们报告了一项大型、观察性、多中心队列研究,该研究纳入了 2003 年至 2014 年期间参加国际儿科卒中研究的年龄在 1 个月至 18 岁的患有 AIS 的儿童。使用 Fisher 精确检验比较临床和人口统计学差异,采用线性逐步置换最小化调整进行多重比较。进行探索性分析以评估有和无血管病变的 AIS 病例之间以及血管病变亚型之间的差异。
在 2127 例 AIS 患儿中,725 例(34%)存在血管病变(中位年龄 7.45 岁)。血管病变亚型包括夹层(27%)、烟雾病(24.5%)、局灶性脑动脉炎-炎症亚型(FCA-i;15%)、弥漫性脑血管炎(15%)和非特异性血管病变(18.5%)。有血管病变的 AIS 患儿更可能在 6 至 9 岁之间出现(比值比 [OR] 1.93, = 0.029),伴有头痛(OR 1.55, = 0.023)、多发性梗死(OR 2.05, < 0.001)、镰状细胞贫血(OR 2.9, = 0.007)和头/颈部外伤(OR 1.93, = 0.018)。血管病变患儿的抗血栓药物使用和卒中复发率更高。在血管病变亚型中,夹层与男性、年龄较大、头痛和抗凝药物使用有关;FCA-i 与偏瘫和单发梗死有关;烟雾病与癫痫发作和复发性卒中有关;血管炎与双侧梗死有关。
特定的临床特征与儿童 AIS 中的脑动脉病变有关。这些观察结果可能有助于指导早期诊断,并确定最有可能从未来治疗试验中获益的亚组。