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

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.

• Authors: Tanjala T Gipson, Heather Jennett, Lee Wachtel, Mary Gregory, Andrea Poretti, Michael V Johnston
• Journal: Epilepsy & behavior case reports (Epilepsy Behav Case Rep) Vol. 1 Pg. 122-5 ( 2013) ISSN: 2213-3232 [Print] United States
• PMID: 25667844 (Publication Type: Journal Article)