Neurobehavioral profiles of children with neurofibromatosis 1 referred for learning disabilities are sex-specific
✍ Scribed by Chapman, Catherine A.; Waber, Deborah P.; Bassett, Nancy; Urion, David K.; Korf, Bruce R.
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 43 KB
- Volume
- 67
- Category
- Article
- ISSN
- 0148-7299
No coin nor oath required. For personal study only.
✦ Synopsis
We compared neurobehavioral profiles of 10 children with neurofibromatosis 1 ("-1) referred for evaluation of learning disabilities (NF/LD) to those of learning disabled children without known genetic disease (LD), matched for age, sex, and estimated I&. It was hypothesized that the NF/LD children wouId exhibit a neurobehavioral profile diagnostic of compromise of frontalkubcortical brain systems while those of the case controls would be heterogeneous.
Records from a clinic data base were reviewed retrospectively for the neurological and neuropsychological components of an interdisciplinary learning disabilities evaluation. Neurological abnormalities were more frequent in the NFLD group, involving gross and fine motor coordination, praxis, and megencephaly. As predicted, clinical neuropsychological diagnostic ratings and composite neurobehavioral observation scores were consistent with compromise of frontal systems in the NFLD group. An unanticipated finding was that outcomes in the NFLD group were sex dependent:
Megencephaly was observed in females only; and the frontaVsubcortica1 neurobehavioral profile was more consistently observed in females. Females with NF-1 with megencephaly may be at increased risk for a neurobehavioral syndrome contributing to LD that is consistent with compromise of frontal/subcortical brain systems.