Forty-six of 93 children with neurofibromatosis type 1 (NF1) were found to satisfy the diagnostic criteria for attention-deficit-hyperactivity disorder (ADHD). Detailed comparisons were made among 20 children with NF1 and ADHD (12 males, 8 females; mean age 10.7 years, SD 2.2), 26 control children with NF1 (15 males, 11 females; mean age 11.3 years, SD 2.3), 14 control children with ADHD (7 males; mean age 9.9 years, SD 1.9), and 14 normally developing control children (7 males; mean age 11.2 years, SD 2.8). Children with NF1 and ADHD had the lowest IQ scores among the four groups. Test of Variables of Attention (TOVA) scores were poorer in the NF1-ADHD and ADHD control groups than in the two non-ADHD groups. Those with NF1 and ADHD were rated significantly poorer on the Child Behavior Checklist (CBCL) than were the NF1 control group. By administrating low doses (5 to 15 mg) of methylphenidate to the NF1-ADHD group, significantly improved TOVA scores were obtained. One-year follow-up yielded significantly improved CBCL scores. Our results show a high incidence of ADHD in NF1 and support an association between ADHD and learning and social problems in children with NF1. It was demonstrated that stimulant medication can lead to improvement in cognitive, academic, and social problems of children with NF1 and ADHD.

Forty-six of 93 children with neurofibromatosis type 1(NF1) were found to satisfy the diagnostic criteria for attentiondeficit-hyperactivity disorder (ADHD). Detailed comparisons were made among 20 children with NF1 and ADHD (12 males, 8 females; mean age 10.7 years, SD 2.2), 26 control children with NF1 (15 males, 11 females; mean age 11.3 years, SD 2.3), 14 control children with ADHD (7 males; mean age 9.9 years, SD 1.9), and 14 normally developing control children (7 males; mean age 11.2 years, SD 2.8).Children with NF1 and ADHD had the lowest IQ scores among the four groups. Test of Variables of Attention (TOVA) scores were poorer in the NF1-ADHD and ADHD control groups than in the two non-ADHD groups. Those with NF1 and ADHD were rated significantly poorer on the Child Behavior Checklist (CBCL) than were the NF1 control group. By administrating low doses (5 to 15 mg) of methylphenidate to the NF1-ADHD group, significantly improved TOVA scores were obtained. One-year follow-up yielded significantly improved CBCL scores. Our results show a high incidence of ADHD in NF1 and support an association between ADHD and learning and social problems in children with NF1. It was demonstrated that stimulant medication can lead to improvement in cognitive, academic, and social problems of children with NF1 and ADHD.

Test-retest reliability of the Test of Variables of Attention (T.O.V.A.) was investigated in two studies using two different time intervals: 90 min and 1 week (2 days). To investigate the 90-min reliability, 31 school-age children (M = 10 years, SD = 2.66) were administered the T.O.V.A. then read ministered the test 90 min afterward. Significant reliability coefficients were obtained across omission (.70), commission (.78), response time (.84), and response time variability (.87). For the second study, a different sample of 33 school-age children (M = 10.01 years, SD = 2.59) were administered the test then read ministered the test 1 week later. Significant reliability coefficients were obtained for omission (.86), commission (.74), response time (.79), and response time variability (.87). Standard error of measurement statistics were calculated using the obtained coefficients. Commission scores were significantly higher on second trials for each retest interval.

Objective: To analyze the psychological phenotype of ADHD, and the effect of ADHD on life satisfaction and personality in adults with neurofibromatosis type 1 (NF1). Method: Adults with NF1 without ( n = 26) and with ADHD ( n = 22), and adults with ADHD only ( n = 27) completed questionnaires on personality traits and life satisfaction. Differences between groups were analyzed. Results: Participants with NF1 and ADHD present an emotionally instable psychological phenotype similar to adults with ADHD only, which differed significantly from that in adults with NF1 only. Participants with NF1 and ADHD had significantly lower overall life satisfaction than NF1 participants without such symptoms, affecting general health, self-satisfaction, sexuality, and family. Conclusion: The authors’ findings show that ADHD symptoms can persist through adulthood. These NF1 patients display problems similar to those of adults with ADHD only. This finding is highly relevant to understand the behavioral and psychological phenotype in adults with NF1 and to offer psychological and/or medical treatment.

This paper discusses briefly the development and rationale behind the Luria-Nebraska Neuropsychological Battery-Children's Revision. Three investigations are discussed: (a) the development of normative data on a group of 125 normal children between the ages of 96 and 155 months, (b) development of the critical level formula for determining the upper bounds for normal performance given a child's age, and (c) a validation of the ability of the battery to discriminate between normal and brain-injured children selected as a separate sample from that used in the initial investigations. In the third study, a MANOVA as well as subsequent individual t tests indicated highly significant group differences. Counting scores above the patient's critical level (as determined from the formula designed in the second investigation) resulted in correct classification of 89% of the normal children and 79% of the brain-injured children. Methods of interpreting the battery and limitations of the current study are discussed.