Growth curve analyses of neuropsychological profiles in children with neurofibromatosis type 1: specific cognitive tests remain "spared" and "impaired" over time.

Cognitive deficits in neurofibromatosis Type 1 (NF-1) have been documented in both the verbal and visuospatial domains. Previous investigations from our laboratory have determined a specific pattern of "spared" (Picture Arrangement, Picture Completion, and Rapid Automatized Naming) and "impaired" (Judgment of Line Orientation, Vocabulary, and Block Design) performance on cognitive measures in this population when compared to sibling-matched controls in pairwise designs. Growth curve analyses were conducted on these repeated measures in 19 patients with NF-1 and their siblings to investigate the longitudinal course and growth pattern of these spared and impaired measures. Results indicated that over time children with NF-1 do not catch up to their siblings on impaired measures, and they continue to perform similarly to their siblings on the spared measures. With respect to growth rates, on average across the 6 cognitive measures there was no significant difference between the groups. However, the variation among families for level of performance was estimated to be larger than variation among siblings within a family for 2 out of 6 cognitive measures (i.e., providing for these 2, Vocabulary and Rapid Automatized Naming, evidence of substantial familial correlation), suggesting that there is need to consider NF-1 associated deficits within a familial context.