Cortical morphology associated with language function in neurofibromatosis, type I.

Neurofibromatosis, type I (NF-I) is associated with verbal and nonverbal neuropsychological deficits and neuroanatomical anomalies. Few relationships between CNS abnormalities and cognitive function in this population, however, have been found. Reading disabilities and developmental language impairments in the general population have been associated with particular morphologic features in inferior frontal gyrus (IFG) and Heschl's gyrus (HG). We compared the morphology of these regions in children with NF-I and controls. Verbal skills in NF-I were related to IFG morphology, such that individuals with NF-I who showed "typical" gyral patterns in the right hemisphere performed worse across language measures than those showing an extra "atypical" gyrus. A doubling of HG in the left and right hemispheres was also significantly associated with performance on several neuropsychological measures. This is the first study to link regional gyral morphology with language function in NF-I. A possible molecular basis for the observed relationships is discussed.