TY - JOUR
T1 - Cognitive and adaptive development of patients with tuberous sclerosis complex
T2 - a retrospective, longitudinal investigation
AU - van Eeghen, Agnies M
AU - Chu-Shore, Catherine J
AU - Pulsifer, Margaret B
AU - Camposano, Susana E
AU - Thiele, Elizabeth A
N1 - Copyright © 2011 Elsevier Inc. All rights reserved.
PY - 2012/1
Y1 - 2012/1
N2 - 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.CONCLUSION: 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.
AB - 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.CONCLUSION: 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.
KW - Adaptation, Psychological/physiology
KW - Adolescent
KW - Age of Onset
KW - Child
KW - Child, Preschool
KW - Cognition Disorders/diagnosis
KW - Electroencephalography
KW - Female
KW - Humans
KW - Infant
KW - Intelligence Tests
KW - Intelligence/physiology
KW - Longitudinal Studies
KW - Male
KW - Neuropsychological Tests
KW - Retrospective Studies
KW - Tuberous Sclerosis/complications
KW - Young Adult
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=84855869473&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/22099526
U2 - https://doi.org/10.1016/j.yebeh.2011.10.005
DO - https://doi.org/10.1016/j.yebeh.2011.10.005
M3 - Article
C2 - 22099526
SN - 1525-5050
VL - 23
SP - 10
EP - 15
JO - Epilepsy & Behavior
JF - Epilepsy & Behavior
IS - 1
ER -