Lozano Christopher S, Nesvick Cody L, Sequeira Alexandra De, Vogt Lindsey M, Laughlin Suzane, Jain Puneet, Kulkarni Abhaya V, Rutka James T, Ibrahim George M, Drake James
Division of Neurosurgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
School of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland.
J Neurosurg Case Lessons. 2025 May 26;9(21). doi: 10.3171/CASE25189.
Stroke-like migraine attack after radiation therapy (SMART) syndrome is a rare, episodic neurological condition characterized by headache and unilateral cortical deficits following remote cranial radiation therapy. It is more frequently reported in adults, while pediatric cases remain exceedingly rare due to the latency period between radiation therapy and onset, which can span years to decades. Diagnosis is challenging due to overlapping symptoms with conditions like ventriculoperitoneal shunt (VPS) malfunction.
The authors report the case of an 11-year-old boy with SMART syndrome following radiation treatment for a resected pineal region nongerminomatous germ cell tumor and VPS placement. The patient presented with headache, vomiting, lethargy, and seizures, raising initial concern for shunt malfunction. MRI revealed left occipital and posterior temporal cortical FLAIR hyperintensity and abnormal leptomeningeal enhancement, consistent with SMART syndrome. Multidisciplinary evaluation ruled out shunt failure and tumor recurrence. A course of corticosteroids led to symptom resolution. A review of 15 pediatric cases highlights variability in latency, symptoms, and treatment, with most patients responding favorably.
SMART syndrome, although rare, should be considered in pediatric patients with postradiation episodic neurological symptoms to avoid unnecessary interventions. Multidisciplinary collaboration is essential. Further studies are needed to establish standardized diagnostic and treatment protocols. https://thejns.org/doi/10.3171/CASE25189.
放疗后类卒中偏头痛发作（SMART）综合征是一种罕见的发作性神经系统疾病，其特征为在既往头颅放疗后出现头痛和单侧皮质功能缺损。该综合征在成人中报道更为频繁，而儿科病例由于放疗与发病之间的潜伏期（可长达数年至数十年）仍然极为罕见。由于与脑室腹腔分流术（VPS）故障等疾病症状重叠，诊断具有挑战性。
作者报告了一例11岁男孩的病例，该男孩在接受松果体区非生殖细胞性生殖细胞瘤切除及VPS置入放疗后出现SMART综合征。患者出现头痛、呕吐、嗜睡和癫痫发作，最初引起对分流故障的担忧。MRI显示左侧枕叶和颞叶后部皮质FLAIR高信号及软脑膜异常强化，符合SMART综合征。多学科评估排除了分流失败和肿瘤复发。一个疗程的皮质类固醇治疗使症状得到缓解。对15例儿科病例的回顾突出了潜伏期、症状和治疗的变异性，大多数患者反应良好。
尽管SMART综合征罕见，但对于有放疗后发作性神经症状的儿科患者应予以考虑，以避免不必要的干预。多学科协作至关重要。需要进一步研究以建立标准化的诊断和治疗方案。https://thejns.org/doi/10.3171/CASE25189 。
