Quantitative EEG relative power comparisons between ADHD children and matched controls: A replication

Williams Syndrome (WS) is a rare (1:25,000) autosomal dominant disorder characterized by deficient visuo-motor and visual spatial analysis skills.In contrast, facial recognition abilities are normal as are certain language abilities.Ninety-five percent are also diagnosed with Mental Retardation (MR).Brain imaging studies have not revealed particular lesions that would explain the diminished cognitive capacities in adolescents with WS.Strong performance of WS subjects on semantic and fluency tasks is compatible with the suggestion that their frontal areas may be better preserved than in other MR disorders such as Downs Syndrome, but to what extent this is true is yet unknown.A case study is presented of a 16year-old female with WS who is not MR (Full Scale IQ = 84; Verbal IQ = 91; Performance IQ = 79), but has an Arithmetic Disorder.She fails to demonstrate significant visual spatial impairment, but does display mild problems on copying and the more complex Block Design tasks.On other visual spatial measures she performs within the normal range.Neuropsychological results also indicated a lateralizing asymmetry in motor functioning and severely impaired Trails B performance.This is consistent with an earlier abnormal SPECT finding of hypoperfusion in the right frontal area.Frontal lobe dysfunction has not been previously associated with WS.In light of the above atypical case, a more detailed examination of frontal lobe functioning on clinical examination is indicated in this population.