Visual-spatial performance deficits in children with neurofibromatosis type-1.

Neurofibromatosis type-1 (NF1) is a common genetic disorder associated with a variety of medical complications, cognitive impairments, and behavioral problems including a high incidence of Attention Deficit Hyperactivity Disorder (ADHD). The current study examined the hypotheses that deficits in visual-spatial/motor abilities enable one to discriminate and classify children with NF1 (n = 101) compared to control children (n = 37), beyond effects secondary to parent reported ADHD symptomology. Discriminant analysis showed a multivariate combination of visual-spatial/motor ability tests (Judgment of Line Orientation, Block Design subtest of the WISC-III, Recognition-Discrimination Test, Beery Visual-Motor Integration Test) to be a significant predictor of NF1 diagnostic status (P = 0.0000004; canonical R(2) = 0.2306). A significantly greater degree of ADHD behavior was found in the NF1 group, and a discriminant analysis using ADHD residualized visual-spatial motor scores indicated that the combination of tests continued to be a significant predictor of group membership after the level of ADHD behavior was controlled (P = 0.00002 and a canonical R(2) = 0.1818). This combination of tests proved to be a strong discriminator of NF1. It correctly identified 90% of individuals with the diagnosis, and may be useful to educators to provide assistance and alternatives to minimize the impact of learning problems in those with either known or suspected NF1.
(Copyright 2003 Wiley-Liss, Inc.)