ArticleFrontiers in neurology2024
Cognition and behavior in adults with neurofibromatosis type 1.
Article in Frontiers in neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Cognitive function in adult NF1 patients without other cognitive disorders: a cross-sectional study.Orphanet journal of rare diseases · 2026Article
- Clinical and Humanistic Burden Among Adults with Neurofibromatosis Type 1 and Symptomatic Plexiform Neurofibroma in the United States.Neurology and therapy · 2026Article
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8 authors.
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Abstract
Background: Neurofibromatosis Type 1 (NF1) is a congenital neurocutaneous disorder. As NF1 is incurable and presents with a wide range of physical and mental symptoms, knowledge of neurocognitive and behavioral functioning can be an important aid in understanding their functional impact, and developing treatment options. To date, studies in children with NF1 have shown dysfunction in several domains, but much less is known about cognition and behavior in adults with NF1. The present study describes the neuropsychological phenotype of adults with NF1 based on comprehensive clinical examination of cognition and behavior across multiple functions. Methods: Participants were 62 adults with NF1 (mean age 38.2 years; Results: Performance on the cognitive measures oral reading speed, visuospatial copying, visuospatial immediate recall, visual learning/imprinting, and visual memory immediate recall in the NF1 group were lower than normative means. The behavioral measures indicated higher levels of dysfunction, including psychopathology. The proportions of the NF1 study group with lower cognitive performance and higher levels of behavioral dysfunction were larger than in the normal population distributions. In addition, domain-level results revealed that intelligence, attention/speed, memory, and social cognition reflect cognitive dysfunction. Moreover, levels of emotion perception problems, experienced executive dysfunction, internalizing psychopathology (e.g., anxiety, depression), and severe fatigue were significantly higher compared to the simulated population sample. The mean level of emotion regulation (coping strategies) did not differ significantly from the population. Conclusion: Identified cognitive and behavioral dysfunction in multiple domains indicates high vulnerability in adults with NF1 and underscores the importance of individualized neuropsychological assessment and treatment. Further research on the relationships between cognition and behavior (including fatigue) in NF1 is warranted.
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