ULLRICH-TURNER SYNDROME - NEURODEVELOPMENTAL CHANGES FROM CHILDHOOD THROUGH ADOLESCENCE

Our objective was to investigate whether the previously-described neur ocognitive pattern in girls with Ullrich-Turner syndrome is found in c hildhood and adolescence; we used a prospective, controlled study of n eurocognitive development in girls with Ullrich-Turner syndrome. The p atients included 56 girls with Ullrich-Turner syndrome, and 100 normal age- and verbal IQ-matched female control subjects, whose ages ranged hom 6-14 years. All girls with Ullrich-Turner syndrome and the normal control girls received a battery of neurocognitive tests designed to evaluate the following domains: general cognition, memory, academic ac hievement, language, visual-spatial/perceptual skills, visual-motor sk ills, attention, and affect recognition. Our results demonstrated cons istent findings in Ullrich-Turner syndrome girls across the age range studied. In general, the Ullrich-Turner girls resembled control subjec ts in terms of verbal and language abilities. We found relatively depr essed performance IQ and a significant verbal IQ-performance IQ differ ence. Significant differences were observed on examination of nonverba l abilities. The Ullrich-Turner girls performed more poorly than contr ol girls on 1) tests of visual-motor skills including the Beery Test o f Visual-Motor Integration, the Perceptual Organization Factor, and th e Rey-Osterrieth Figures; 2) tests of visual-spatial skills, including the Motor-Free Visual Perception Test; 3) tests of attention, includi ng the Freedom From Distractibility Factor; and 4) the Affective Proso dy Affect Recognition Test. Ullrich-Turner subjects showed evidence of multifocal or diffuse right cerebral dysfunction and deficits general ly involving nonverbal skills that may be due to X chromosome monosomy , gonadal dysgenesis, or both. Future studies will examine the role of estrogen replacement on cognitive function in Ullrich-Turner syndrome individuals. (C) 1995 Wiley-Liss, Inc.