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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. [ABSTRACT FROM AUTHOR]

Background: The objective of this study was to determine the impact of seizure‐related factors on neurocognitive, health‐related quality of life (HRQOL), and social outcomes in survivors of childhood cancer. Methods: Survivors of childhood cancer treated at St. Jude Children's Hospital (n = 2022; 48.3% female; median age, 31.5 years; median time since diagnosis, 23.6 years) completed neurocognitive testing and questionnaires. The presence, severity, resolution, and treatment history of seizures were abstracted from medical records. Adjusting for the age at diagnosis, sex, and prior cancer therapy, multivariable models examined the impact of seizures on neurocognitive and HRQOL outcomes. Mediation analyses were conducted for social outcomes. Results: Seizures were identified in 232 survivors (11.5%; 29.9% of survivors with central nervous system [CNS] tumors and 9.0% of those without CNS tumors). In CNS tumor survivors, seizures were associated with poorer executive function and processing speed (P <.02); in non‐CNS tumor survivors, seizures were associated with worse function in every domain (P <.05). Among non‐CNS survivors, seizure severity was associated with worse processing speed (P =.023), and resolution was associated with better executive function (P =.028) and attention (P =.044). In CNS survivors, seizure resolution was associated with improved attention (P =.047) and memory (P <.02). Mediation analysis revealed that the impact of seizures on social outcomes was mediated by neurocognitive function. Conclusions: Seizures in cancer survivors adversely affect long‐term functional and psychosocial outcomes independently of cancer therapy. The resolution of seizure occurrence is associated with better outcomes. Seizure severity is associated with poorer outcomes and should be a focus of clinical management and patient education. Adult survivors of childhood cancer are at risk for poorer cognitive, health‐related quality of life, and social outcomes. Seizure frequency, independently of the underlying etiology, is associated with poorer outcomes in non–brain tumor survivors, and this implies that they may be a more vulnerable population with a lower threshold for impact from seizures of any severity. [ABSTRACT FROM AUTHOR]

Daytime sleepiness is recognized in childhood brain tumor survivors. Our objective was to determine prevalence, risk factors for PSG/MLST proven hypersomnia/narcolepsy, and response to stimulants in childhood brain tumor survivors. Standard PSG/MSLT criteria were used to diagnose hypersomnia/narcolepsy. Medical records of brain tumor survivors having undergone a PSG/MSLT were reviewed for the diagnostic code of hypersomnia/narcolepsy. Survivors with hypersomnia/narcolepsy were matched with 2-3 survivors without reported hypersomnia/narcolepsy by age at tumor diagnosis, gender, and time from tumor diagnosis. Between January 2000 to April 2015, 39 of the 2336 brain tumor patients treated at our institution were diagnosed with hypersomnia/narcolepsy for a prevalence rate of 1670/100,000. Hypersomnia/narcolepsy was diagnosed at a median of 6.1 years (range 0.4-13.2) from tumor diagnosis and 4.7 years (range − 1.5 to 10.4) from cranial radiation. Midline tumor location (OR 4.6, CI 1.7-12.2, p = 0.002) and anti-epilepsy drug (AED) use (OR 11, CI 2.4-54) correlated with hypersomnia/narcolepsy while radiation dose > 30 Gray trended towards significance (OR 1.8, CI 0.9-3.6); posterior fossa tumor location reduced the risk (OR 0.1, CI 0.04-0.5, p = 0.002). AED use also correlated with midline tumor location. Thirty-seven survivors were treated with stimulants and reported improved wakefulness and school performance [response rate CI 0.97 (0.86-0.99) and 0.83 (0.65-0.94)]. Prevalence of hypersomnia/narcolepsy among childhood brain tumor survivors was higher than the general population. Tumor location and radiation dose were possible risk factors, and stimulants were reported to be beneficial. [ABSTRACT FROM AUTHOR]