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Abstract
Neurofibromatosis type-1 (NF1) is a monogenic tumor-predisposition syndrome creating a wide variety of cognitive and behavioral abnormalities, such as decrease in cognitive functioning, deficits in visuospatial processing, attention, and social functioning. NF1 patients are at risk to develop neurofibromas and other tumors, such as optic pathway gliomas and other tumors of the central nervous system. Few studies have investigated the impact of an additional diagnosis of brain tumor on the cognitive outcome of children with NF1, showing unclear results and without controlling by the effect of surgery, radio- or chemotherapy. In the present mono-institutional study, we compared the behavioral and cognitive outcomes of 26 children with neurofibromatosis alone (NF1) with two age-matched groups of 26 children diagnosed with NF1 and untreated optic pathway glioma (NF1 + OPG) and 19 children with NF1 and untreated other central nervous system tumors (NF1 + CT). NF1 + CT and NF1 + OPG showed significantly impaired cognitive abilities compared to NF1 group, with weaknesses in visuo-spatial abilities, visual scanning and verbal working memory, while general verbal abilities are preserved. Moreover, NF1 + OPG patients present more frequent internalizing problems and increased oppositional-deviant behaviors. These results suggest that the co-diagnosis of a brain tumor in NF1 children may partially worsen the cognitive and emotional outcome.
Highlights
Neurofibromatosis type-1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 2700 individuals [1]
In the present monoinstitutional study, we examined the influence of brain tumors on the cognitive and behavioral outcome of 71 children with NF1, comparing children with neurofibromatosis alone with two age-matched groups of children diagnosed with neurofibromatosis and untreated optic pathway glioma and children with neurofibromatosis and non-treated other central nervous system (CNS) tumors
We hypothesized that the children with NF1 plus a brain tumor would present more pronounced cognitive and emotional-behavior impairment compared to children with NF1 without an additional diagnosis of brain tumor; we hypothesized that tumors involving different central nervous system structures (i.e., optic pathway gliomas (OPGs) vs. other CNS tumors) may differently influence cognitive and emotional-behavioral outcome
Summary
Neurofibromatosis type-1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 2700 individuals [1]. Affective and behavioral dysregulation have been extensively described, such as attention problems, problems reflecting anxiety, depression, or withdrawal and social problems [13] These cognitive-behavioural impairments have a substantial impact on the quality of life and are a major concern for parents and teachers [14]. No studies investigated the impact of central nervous system tumors on behavioral, cognitive and emotional features of NF1 children who never received any kind of treatment. In the present monoinstitutional study, we examined the influence of brain tumors on the cognitive and behavioral outcome of 71 children with NF1, comparing children with neurofibromatosis alone with two age-matched groups of children diagnosed with neurofibromatosis and untreated optic pathway glioma and children with neurofibromatosis and non-treated other central nervous system (CNS) tumors. We hypothesized that the children with NF1 plus a brain tumor would present more pronounced cognitive and emotional-behavior impairment compared to children with NF1 without an additional diagnosis of brain tumor; we hypothesized that tumors involving different central nervous system structures (i.e., OPG vs. other CNS tumors) may differently influence cognitive and emotional-behavioral outcome
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