From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia.
Neurology. 2015 Sep 29;85(13):1146-53. doi: 10.1212/WNL.0000000000001969. Epub 2015 Aug 28.
To describe characteristics of young children with arterial ischemic stroke (AIS) and bilateral cerebral arteriopathies.
Retrospective review of clinical features, course, and outcome. Neuroimaging was analyzed for infarct pattern, cerebrovascular diagnosis (anatomic/Childhood Arterial Ischemic Stroke Standardized Classification and Diagnostic Evaluation [CASCADE] criteria), and disease progression.
In the 31 children (median age, 18 months), presentations included acute hemiparesis (n=23) and focal seizures (n=12). Seven had systemic arterial disease; 13 had cardiac abnormalities. Twenty had recurrent AIS or transient ischemic attack (after median of 3 months); 16 had >1 recurrence. Median modified Rankin Scale score was 3, with motor impairments in 20, cognitive impairments in 11, and seizures in 7. At presentation, 17 had old and acute infarcts. Twenty-five had high signal in white matter. A total of 13/23 reimaged patients accrued further infarcts over a median of 39 months. Arteriopathy involved the carotid circulation bilaterally in all; 6 had posterior circulation and 11 had extracranial involvement. Arteriopathy distribution was symmetric in 24/31. CASCADE categories were 3A in 19, 3B in 5, 3C in 5, and 7 in 2. After a median of 35 months, 14 had had progression of arteriopathy. Patients categorized as CASCADE 3A (moyamoya) had significantly shorter time to recurrence than other groups.
Young children with bilateral cerebral arteriopathies (particularly meeting criteria for CASCADE 3A) have a malignant course, with frequent recurrent events, progressive disease, and poor outcomes. Current classifications are limited in characterizing disease in many cases. Symmetric involvement suggests these arteriopathies may be developmentally determined, while systemic involvement suggests potential genetic etiology.
描述动脉缺血性脑卒中（AIS）和双侧脑动脉疾病患儿的特点。
回顾性分析临床特征、病程和结局。对神经影像学进行分析，以确定梗死模式、脑血管诊断（解剖/儿童动脉缺血性脑卒中标准化分类和诊断评估[CASCADE]标准）和疾病进展。
31 名患儿（中位年龄 18 个月）的表现包括急性偏瘫（n=23）和局灶性癫痫发作（n=12）。7 例存在系统性动脉疾病；13 例存在心脏异常。20 例有复发性 AIS 或短暂性脑缺血发作（中位时间 3 个月后）；16 例有>1 次复发。中位改良 Rankin 量表评分为 3 分，20 例存在运动障碍，11 例存在认知障碍，7 例存在癫痫。在发病时，17 例存在陈旧性和急性梗死。25 例患儿的脑白质有高信号。在中位时间 39 个月的时间内，23 例重新成像的患者中有 13 例发生了进一步的梗死。动脉病变双侧累及颈内动脉循环；6 例累及后循环，11 例有颅外受累。31 例患儿中有 24 例的动脉病变分布对称。25 例患儿的动脉病变分布对称。CASCADE 分类为 19 例 3A，5 例 3B，5 例 3C，2 例 7。中位随访时间 35 个月后，14 例患儿的动脉病变出现进展。归类为 CASCADE 3A（烟雾病）的患儿复发时间明显短于其他组。
双侧脑动脉疾病（特别是符合 CASCADE 3A 标准的患儿）具有恶性病程，常反复发作，疾病进展，预后不良。目前的分类方法在许多情况下都无法很好地描述疾病。对称受累提示这些动脉疾病可能与发育有关，而系统性受累提示可能存在遗传病因。
