Significance of Planum Temporale and Planum Parietale Morphologic Features in Neurofibromatosis Type 1

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Abstract
NEUROFIBROMATOSIS TYPE 1 (NF-1) is an autosomal dominant genetic disorder that affects approximately 1 in 4000 people.1 It is associated with a mutation on chromosome 17 and has highly variable phenotypic expression. Children with NF-1 have a high incidence of central nervous system anomalies. Areas of abnormal signal intensity in the brain, typically visualized using T2-weighted or fluid attenuation inversion recovery magnetic resonance imaging (MRI) protocols, are observed in approximately 70% of children with NF-1. These hyperintensities are most frequently observed in the brainstem, cerebellum, basal ganglia, and thalamus.2 Cognitive impairment is also frequently described, and whereas some studies have shown no relationship between the presence of hyperintensities and neuropsychological deficits,3,4 others have demonstrated associations between the presence or absence,5 number,6,7 or location8 of hyperintensities and cognitive impairment.