Prevalence, risk factors, and response to treatment for hypersomnia of central origin in survivors of childhood brain tumors.

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.

Clinical Characteristics and Long-Term Outcomes of Movement Disorders in Childhood Thalamic Tumors.

BACKGROUND: We studied the outcomes of movement disorders that were associated with childhood thalamic tumors. METHODS: We retrospectively reviewed 83 children with thalamic tumors treated at our institution from 1996 to 2013 to document the incidence and outcome of movement disorders. Magnetic resonance imaging was used to analyze the involvement of thalamic nuclei, and three instruments were used to rate the severity of the disorders. RESULTS: Nine (11%) patients had one or more of the following movement disorders: postural tremor, resting tremor, ballism, dystonia, myoclonus, and athetosis. Median age at tumor diagnosis was seven years (range, 0.25 to 11 years), and the average age at movement disorder onset was eight years (range, 1.5 to 11 years). Movement disorders developed at a median of 1.5 months (range, 0 to 4 months) after surgical resection. The severity of the disorders was either unchanged or slightly improved during follow-up. The red nuclei were the only thalamic structures that showed tumor involvement in all nine patients. CONCLUSIONS: No specific injury of the thalamic nuclei was associated with movement disorders in children with thalamic tumors, and the severity of these disorders did not change over time.

Imaging Patterns and Outcome of Posterior Reversible Encephalopathy Syndrome During Childhood Cancer Treatment.

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 among 5,217 children treated during the study period. Median time from cancer diagnosis to PRES was 6.6 months in 25 leukemia (1.6%), five brain tumor (0.3%), and seven 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 was seen in 97%, and steroids use was seen in 78%. Headaches, visual disturbance, and mental status resolved within a median of <3 days, whereas 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 magnetic resonance imaging (MRI) in 34 patients showed partial or complete T2 resolution in 79%, development of laminar necrosis in five, microhemorrhages in six, and focal atrophy in three. CONCLUSION: PRES in children is more common in hematological malignancy compared with 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.