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Gipson TT, Jennett H, Wachtel L, et al. Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior. Epilepsy Behav Case Rep. 2013;1:122-5doi: 10.1016/j.ebcr.2013.06.004.
Gipson, T. T., Jennett, H., Wachtel, L., Gregory, M., Poretti, A., & Johnston, M. V. (2013). Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior. Epilepsy & behavior case reports, 1122-5. https://doi.org/10.1016/j.ebcr.2013.06.004
Gipson, Tanjala T, et al. "Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior." Epilepsy & behavior case reports vol. 1 (2013): 122-5. doi: https://doi.org/10.1016/j.ebcr.2013.06.004
Gipson TT, Jennett H, Wachtel L, Gregory M, Poretti A, Johnston MV. Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior. Epilepsy Behav Case Rep. 2013 Aug 24;1:122-5. doi: 10.1016/j.ebcr.2013.06.004. eCollection 2013. PMID: 25667844; PMCID: PMC4150649.
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Epilepsy Behav Case Rep. 2013 Aug 24;1:122-5. doi: 10.1016/j.ebcr.2013.06.004. eCollection 2013.

Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior.

Epilepsy & behavior case reports

Tanjala T Gipson, Heather Jennett, Lee Wachtel, Mary Gregory, Andrea Poretti, Michael V Johnston

Affiliations

  1. Tuberous Sclerosis Clinic, Kennedy Krieger Institute, Baltimore, MD, USA.
  2. Neurobehavioral Unit, Kennedy Krieger Institute, Baltimore, MD, USA.
  3. Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD, USA.
  4. Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

PMID: 25667844 PMCID: PMC4150649 DOI: 10.1016/j.ebcr.2013.06.004

Abstract

BACKGROUND: Self-injury and aggression have been reported in individuals with TSC (tuberous sclerosis complex), yet few data exist about treatment. Everolimus, an mTOR inhibitor, has been FDA-approved for subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas in TSC. However, clinical use of everolimus with direct, real-time observations of self-injury and aggression in an individual with TSC has not been reported.

METHODS: During an inpatient admission to a neurobehavioral unit, real-time measurements of behaviors and seizures were recorded. An interdisciplinary team used these data to make treatment decisions and applied behavioral and pharmacological treatments, one at a time, in order to evaluate their effects.

RESULTS: Aggression and self-injury improved with applied behavioral analysis (ABA), lithium, and asenapine. Improvements in SEGA size, facial angiofibromas, seizures, and the most stable low rates of self-injury were observed during the interval of treatment with everolimus.

CONCLUSION: Mechanism-based treatments in the setting of an evidence-based behavioral and psychopharmacological intervention program may be a model with utility for characterization and treatment of individuals with severe behavior and TSC.

Keywords: ABA, applied behavioral analysis; Aggression; Behavioral intervention and epilepsy; CYP3A4, cytochrome p450 3A4; Everolimus; FDA, Food and Drug Administration; SEGA, subependymal giant cell astrocytoma; Self-injury; TSC, tuberous sclerosis complex; Tuberous sclerosis complex; mTOR, mammalian target of rapamycin; mTORC, mammalian target of rapamycin complex

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