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Stroke-Like Migraine after Radiation Treatment Syndrome in Children with CancerFunding This study was supported by Cancer Center Support Grant [CA21765] from the National Cancer Institute, and by the American Libyan Syrian Associated Charities.
Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a symptom complex of transient neurological deficits, headache, and abnormal cortical contrast enhancement on brain MRI. Pathophysiology is unclear, but exposure to cranial radiation (RT) is a sine qua non. We report five children with SMART syndrome treated with RT therapy for medulloblastoma (n = 3), atypical teratoid rhabdoid tumor (n = 1), and pleomorphic xanthoastrocytoma (n = 1). Median age at tumor diagnosis was 9.4 years (range 5.1–14.7). Median follow-up from cancer diagnosis was 3.1 years (range 1.4–12.9). All patients had 54 Gy focal RT treatment and medulloblastoma children had additional 36 Gy craniospinal irradiation. Median time from the end of RT to first transient neurological deficit was 1 year (range 0.7–12.1). The median follow-up since first SMART episode was 0.6 years (range 0.3–2.6). Presenting symptoms included the gradual development of unilateral weakness (n = 4), non-fluent dysphasia (n = 1), somnolence (n = 1), and headaches (n = 3). Neurological deficits resolved within 30 minutes to 10 days. Transient cortical enhancement on magnetic resonance imaging (MRI) was confirmed in two children and was absent in the other three. Two children had a single and three had multiple episodes over the next few months. Two children with protracted symptoms responded to 3 days treatment with high dose intravenous methylprednisolone. Symptoms ultimately resolved in all patients. SMART syndrome is a rare disorder characterized by slow evolution of neurological deficits with variable abnormal cortical contrast enhancement. The use of steroids may improve symptoms and speed resolution.
Q.S.A. collected the data and wrote the first draft. R.B.K. reviewed imaging and edited the manuscript. All other authors read and edited the manuscript.
The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
Received: 14 July 2021
Accepted: 03 November 2021
Article published online:
20 December 2021
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