Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Tuberous Sclerosis

  • Carol L. ArmstrongEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_162

Definition

Tuberous sclerosis occurring in the brain is a rare genetic disorder that can affect multiple critical organs, but when affecting the brain causes benign tumors to grow, and is associated with seizures, autism, mental retardation, behavioral problems, autism, and skin abnormalities, according to the National Institute of Neurological Disorders and Stroke. Cardiac lesions, renal lesions, pulmonary manifestations, and neurologic manifestations (epilepsy, infantile spasms, profound intellectual disability, subependymal giant-cell tumors causing obstructive hydrocephalus) are common. This disease cannot be cured and is managed through follow-up to monitor it and through treatment of symptoms. The common behavioral manifestations include increased rates of depression, anxiety, and aggressive outbursts (Pulsifer et al. 2007; deVries et al. 2007).

The tuberous sclerosis complex (TSC) is a multisystem, autosomal dominant disorder affecting children and adults resulting from...

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References and Reading

  1. Crino, P. B., Nathanson, K. L., & Henske, E. P. (2006). The tuberous sclerosis complex. The New England Journal of Medicine, 355, 1345–1356.CrossRefPubMedPubMedCentralGoogle Scholar
  2. deVries, P. J. (2010). Targeted treatments for cognitive and neurodevelopmental disorders in tuberous sclerosis complex. Neurotherapeutics, 7(3), 275–282.CrossRefGoogle Scholar
  3. deVries, P. J., Hunt, A., & Bolton, P. F. (2007). The psychopathologies of children and adolescents with tuberous sclerosis complex (TSC): A postal survey of UK families. European Child and Adolescent Psychiatry, 16(1), 16–24.CrossRefGoogle Scholar
  4. Pulsifer, M. B., Winterkorn, E. B., & Thiele, E. A. (2007). Psychological profile of adults with tuberous sclerosis complex. Epilepsy and Behavior, 10(3), 402–406.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Child and Adolescent Psychiatry and Behavioral SciencesThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA