ArticleAmerican journal of medical genetics. Part C, Seminars in medical genetics2011
Observations on intelligence and behavior in 15 patients with Legius syndrome.
Article in American journal of medical genetics. Part C, Seminars in medical genetics, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 29 citations in OpenAlex.
- Review
- MEK inhibition ameliorates social behavior phenotypes in a Spred1 knockout mouse model for RASopathy disorders.Molecular autism · 2021Article
- Central precocious puberty in a girl with LEGIUS syndrome: an accidental association?Italian journal of pediatrics · 2021Article
- Legius Syndrome and its Relationship with Neurofibromatosis Type 1.Acta dermato-venereologica · 2020Article
- Legius syndrome: case report and review of literature.Italian journal of pediatrics · 2015Review
- Modeling cognitive dysfunction in neurofibromatosis-1.Trends in neurosciences · 2013Review
- Lymphangiopathy in neurofibromatosis 1 manifesting with chylothorax, pericardial effusion, and leg edema.International journal of general medicine · 2013Article
- Neurofibromatosis type 1 (NF1) associated with tumor of the corpus callosum.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2012Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
Legius syndrome is a RAS-MAPK syndrome characterized by pigmentary findings similar to neurofibromatosis type 1 (NF1), but without tumor complications. Learning difficulties and behavioral problems have been reported to be associated with Legius syndrome, but have not been studied systematically. We investigated intelligence and behavior in 15 patients with Legius syndrome and 7 unaffected family members. We report a mean full-scale IQ of 101.57 in patients with Legius syndrome, which does not differ from the control group. We find a significantly lower Performance IQ in children with Legius syndrome compared to their unaffected family members. Few behavioral problems are present as assessed by the Child Behavior Checklist (CBCL) questionnaire. Our observations suggest that, akin to the milder somatic phenotype, the cognitive phenotype in Legius syndrome is less severe than that of NF1.
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