Background Diagnosis of posterior reversible encephalopathy syndrome (PRES) requires presence of headache, seizures, impaired vision, or altered mentation accompanied by specific imaging findings. We aimed to study long-term clinical and radiologic outcome of PRES in children with cancer to augment limited available data. Procedure Retrospective review of children with cancer who were diagnosed with PRES. Results We identified PRES in 21 males and 16 females amongst the 5,217 children treated during the study period. Median time from cancer diagnosis to PRES was 6.6 months in 25 leukemia (1.6%), 5 brain tumor (0.3%), and 7 other solid tumor (0.4%) patients; p=<0.0001 for leukemia versus all other tumors. Symptoms included seizures (97%), headaches (40%), altered mentation (68%), and vision impairment (27%). Hypertension in 97% and steroids use was seen in 78%. Headaches, visual disturbance, and mental status resolved within a median of <3 days, while epilepsy developed in 19%. T2 hyperintense signal was present in 100% of occipital, 47% of temporal, 75% of parietal, and 55% of frontal lobes, as well as 22% of cerebellum and 5% of basal ganglia. Follow-up MRI in 34 patients showed partial or complete T2 resolution in 79%, development of laminar necrosis in 5, microhemorrhages in 6, and focal atrophy in 3. Conclusion PRES in children is more common in hematological malignancy compared to other tumors and is associated with hypertension and steroid use. Seizure is the most common acute manifestation. Most MRI changes resolve, but persistent imaging abnormality and epilepsy may develop in a significant minority.

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.

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NEURO-ONCOLOGY • NOVEMBER 2017 course seizures, usually due to clinical progression, whereas IDH mutant gliomas occur more randomly and with higher refractoriness. This may help clinicians stratify seizure risk based on IDH status and seizure presentation history.