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Objectives: Rates of cognitive, academic and behavioral comorbidities are elevated in children with epilepsy. The contribution of environmental and genetic influences to comorbidity risk is not fully understood. This study investigated children with epilepsy, their unaffected siblings, and controls to determine the presence and extent of risk associated with family relatedness across a range of epilepsy comorbidities. Methods: Participants were 346 children (8–18 years), n=180 with recent-onset epilepsy, their unaffected siblings (n=67), and healthy first-degree cousin controls (n=99). Assessments included: (1) Child Behavior Checklist/6-18 (CBCL), (2) Behavior Rating Inventory of Executive Function (BRIEF), (3) history of education and academic services, and (4) lifetime attention deficit hyperactivity disorder (ADHD) diagnosis. Analyses consisted of linear mixed effect models for continuous variables, and logistic mixed models for binary variables. Results: Differences were detected between the three groups of children across all measures (p<.001). For ADHD, academic problems, and executive dysfunction, children with epilepsy exhibited significantly more problems than unaffected siblings and controls; siblings and controls did not differ statistically significantly from each other. For social competence, children with epilepsy and their unaffected siblings displayed more abnormality compared with controls, with no statistically significant difference between children with epilepsy and unaffected siblings. For behavioral problems, children with epilepsy had more abnormality than siblings and controls, but unaffected siblings also exhibited more abnormalities than controls. Conclusions: The contribution of epilepsy and family relatedness varies across specific neurobehavioral comorbidities. Family relatedness was not significantly associated with rates of ADHD, academic problems and executive dysfunction, but was associated with competence and behavioral problems. (JINS, 2018, 24, 1–9)
Neurofibromatosis 1 (NF1) and neurofibromatosis 2 (NF2) are inherited tumor predisposition syndromes that have a major impact on the nervous system but are clinically and genetically distinct disorders. This chapter discusses the clinical characteristics and genetics of the neurofibromatoses and presents the current knowledge about their relationship with epilepsy. As neurofibromin acts as a tumor suppressor, NF1 individuals are at increased risk of developing benign and malignant tumors, particularly pilocytic astrocytomas, although pilomyxoid astrocytomas and glioblastoma multiforme can occur. The treatment of NF1 is based on assiduous monitoring for disease complications and treatment of the specific disease manifestations. NF2 should be distinguished from schwannomatosis, a rare condition characterized by the development of painful schwannomas involving the cutaneous, peripheral, and spinal nerves. The diagnosis of NF2 is made according a combination of skin, eye, and central and peripheral nervous system manifestations.
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