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Tuberous Sclerosis

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Encyclopedia of Clinical Neuropsychology
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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

  • Crino, P. B., Nathanson, K. L., & Henske, E. P. (2006). The tuberous sclerosis complex. The New England Journal of Medicine, 355, 1345–1356.

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  • deVries, P. J. (2010). Targeted treatments for cognitive and neurodevelopmental disorders in tuberous sclerosis complex. Neurotherapeutics, 7(3), 275–282.

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  • 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.

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  • 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.

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Correspondence to Carol L. Armstrong .

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Armstrong, C.L. (2018). Tuberous Sclerosis. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_162

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