The adverse influence of attention-deficit disorder with or without hyperactivity on cognition in neurofibromatosis type 1

Abstract

Aim: A substantial proportion of patients with neurofibromatosis type 1 (NF1) have attention-deficit disorder with or without hyperactivity (AD[H]D). This study explored the influence of AD(H)D symptoms on the intellectual profile of patients with NF1.

Method: We retrospectively analysed neuropsychological data from 114 children (66 males, 48 females; age range 6-16y; mean age 9y 3mo [SE 3mo]) with NF1 from an NF1 outpatients department. Assessment included psychiatric diagnosis of AD(H)D (DSM-IV-TR criteria) and intelligence testing (Wechsler Intelligence Scale for Children, German version). Magnetic resonance images were available for all patients, intracranial findings being an exclusion criterion. The effects of AD(H)D symptoms on intelligence and on the cognitive profile were tested by analyses of variance.

Results: Patients with AD(H)D symptoms performed significantly worse than those without AD(H)D symptoms on all intelligence measures (main effects for Full-scale, Verbal, and Performance IQ; p<0.005). Subtests typically impaired in patients with NF1 (visuospatial skills and arithmetic) were not specifically influenced by AD(H)D symptoms. There were no differences between AD(H)D subtypes.

Interpretation: AD(H)D symptoms have a negative impact on the intellectual development of children with NF1. This impact seems to be of an unspecific nature, with a general attenuation of the cognitive profile.