title: Cognitive and adaptive development of patients with tuberous sclerosis complex: a retrospective, longitudinal investigation.
abstract: OBJECTIVE
  The aim of the work described here was to systematically analyze the developmental trajectory of patients with tuberous sclerosis complex (TSC).
  
  
  METHODS
  A retrospective longitudinal chart review was performed, selecting patients who received multiple neuropsychological assessments. Intellectual/Developmental Quotients, Adaptive Behavior Composite scores, and clinical data were collected. On available EEGs, interictal epileptiform discharges were counted.
  
  
  RESULTS
  Sixty-six (18%) patients with TSC received multiple cognitive and adaptive development assessments. The mean intelligence of this study group remained relatively stable, albeit variable. Significant decline in adaptive functioning was observed, associated with lower age at seizure onset. Patients who underwent neurosurgery prior to baseline testing showed cognitive improvement. Developmental declines were significantly associated with increased numbers of antiepileptic drugs, with a trend toward association with mutation type and interictal epileptiform discharges.
  
  
  CONCLUSIONS
  This study suggests that the developmental course of patients with TSC may be altered by epilepsy comorbidity and neurosurgery, underlining the need for early and effective interventions in this population.
authors.affiliation: Department of Neurology, Massachusetts General Hospital, Boston, MA, USA.
authors.forename: Agnies M
authors.initials: AM
authors.lastname: van Eeghen
authors.forename: Catherine J
authors.initials: CJ
authors.lastname: Chu-Shore
authors.forename: Margaret B
authors.initials: MB
authors.lastname: Pulsifer
authors.forename: Susana E
authors.initials: SE
authors.lastname: Camposano
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Thiele
mesh_terms: D000223:Adaptation, Psychological / Q000502:physiology*; D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D007360:Intelligence / Q000502:physiology; D007361:Intelligence Tests; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D012189:Retrospective Studies; D014402:Tuberous Sclerosis / Q000150:complications* / Q000523:psychology* / Q000601:surgery; D055815:Young Adult