title: Cognitive and speech-language performance in children with ataxia telangiectasia.
abstract: OBJECTIVE
  To describe cognitive and speech-language functioning of patients with ataxia-telangiectasia (A-T) in relation to their deteriorating (oculo)motor function.
  
  
  METHODS
  Observational case series.
  
  
  METHODS
  Cognitive functioning, language, speech and oral-motor functioning were examined in eight individuals with A-T (six boys, two girls), taking into account the confounding effects of motor functioning on test performance.
  
  
  RESULTS
  All patients, except the youngest one, suffered from mild-to-moderate/severe intellectual impairment. Compared to developmental age, patients showed cognitive deficits in attention, (non)verbal memory and verbal fluency. Furthermore, dysarthria and weak oral-motor performance was found. Language was one of the patients' assets.
  
  
  CONCLUSIONS
  In contrast to the severe deterioration of motor functioning in A-T, cognitive and language functioning appeared to level off with a typical profile of neuropsychological strengths and weaknesses. Based on our experiences with A-T, suggestions are made to determine a valid assessment of the cognitive and speech-language manifestations.
authors.affiliation: Department of Medical Psychology, Radboud University Nijmegen Medical Centre, The Netherlands. a.vinck@mps.umcn.nl
authors.forename: Anja
authors.initials: A
authors.lastname: Vinck
authors.forename: Mijke M M
authors.initials: MM
authors.lastname: Verhagen
authors.forename: Marjo van
authors.initials: Mv
authors.lastname: Gerven
authors.forename: Imelda J M
authors.initials: IJ
authors.lastname: de Groot
authors.forename: Corry M R
authors.initials: CM
authors.lastname: Weemaes
authors.forename: Ben A M
authors.initials: BA
authors.lastname: Maassen
authors.forename: Michel A A P
authors.initials: MA
authors.lastname: Willemsen
mesh_terms: D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000523:psychology*; D001288:Attention / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003071:Cognition / Q000502:physiology*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000523:psychology*; D007802:Language; D008297:Male; D008568:Memory / Q000502:physiology; D009483:Neuropsychological Tests; D013060:Speech / Q000502:physiology*