Seminars in Pediatric Neurology
Volume 13, Issue 1 , Pages 27-36, March 2006

The Diverse Clinical Manifestations of Tuberous Sclerosis Complex: A Review

  • Tena Rosser, MD

      Affiliations

    • Department of Neurology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA
    • Corresponding Author InformationAddress reprint requests to Tena Rosser, MD, Children’s Hospital Los Angeles, Department of Neurology, MS# 82, 4650 Sunset Boulevard, Los Angeles, CA 90027.
    • ,
  • Ashok Panigrahy, MD

      Affiliations

    • Department of Radiology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA
    • ,
  • William McClintock, MD

      Affiliations

    • Department of Neurology, Center for Neuroscience and Behavioral Medicine, Children’s National Medical Center, The George Washington University, Washington, DC

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous disorder. TSC results in hamartomatous lesions primarily involving the skin, central nervous system, kidneys, eyes, heart, and lungs. The clinical findings and severity of TSC are highly variable. Recent advances in our understanding of the complexities of the TSC1 and TSC2 genes are making genotype-phenotype correlations possible. While managing seizures, cognitive dysfunction, and behavioral abnormalities are the primary responsibility of the neurologist, familiarity with all aspects of this disease helps provide better comprehensive care for affected individuals.

Keywords:  tuberous sclerosis , epilepsy , infantile spasms , autism , subependymal giant cell astrocytoma , angiomyolipoma , cardiac rhabdomyoma

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PII: S1071-9091(06)00012-X

doi:10.1016/j.spen.2006.01.008

Seminars in Pediatric Neurology
Volume 13, Issue 1 , Pages 27-36, March 2006

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