Ullrich‐Turner syndrome: Neurodevelopmental changes from childhood through adolescence

Publisher Website
Google Scholar
Abstract
Our objective was to investigate whether the previously‐described neurocognitive pattern in girls with Ullirich‐Turner syndrome is found in childhood and adolescence; we used a prospective, controlled study of neurocognitive development in girls with Ullrich‐Turner syndrome. The patients included 56 girls with Ullrich‐Turner syndrome, and 100 normal age‐ and verbal IQ‐matched female control subjects, whose ages ranged from 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 achievement, language, visualspatial/perceptual skills, visual‐motor skills, attention, and affect recognition. Our results demonstrated consistent findings in Ullrich‐Turner syndrome girls across the age range studied. In general, the Ullrich‐Turner girls resembled control subjects in terms of verbal and language abilities. We found relatively depressed performance IQ and a significant verbal IQ‐performance IQ difference. Significant differences were observed on examination of nonverbal abilities. The Ullrich‐Turner girls performed more poorly than control girls on 1) tests of visual‐motor skills including the Beery Test of Visual‐Motor Integration, the Perceptual Organization Factor, and the Rey‐Osterrieth Figures; 2) tests of visual‐spatial skills, including the Motor‐Free Visual Perception Test; 3) tests of attention, including the Freedom From Distractibility Factor; and 4) the Affective Prosody Affect Recognition Test. Ullrich‐Turner subjects showed evidence of multifocal or diffuse right cerebral dysfunction and deficits generally 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.