Disease Directory Tuberous Sclerosis Complex
Neurological

Tuberous Sclerosis Complex

Also known as: TSC, tuberous sclerosis, Bourneville disease, TSC1/TSC2 haploinsufficiency

Prevalence

1-5 per 10,000 (Orphanet)

Onset

Neonatal, Infantile, Childhood

Type

Autosomal dominant genetic (de novo in ~2/3)

Gene

TSC1, TSC2

About Tuberous Sclerosis Complex

Tuberous sclerosis complex (TSC) is a multi-system genetic disorder caused by mutations in TSC1 or TSC2, encoding hamartin and tuberin respectively, which together regulate the mTOR signaling pathway. Loss of function leads to benign tumors (hamartomas) in multiple organs including the brain (cortical tubers, subependymal nodules, SEGA), kidneys (angiomyolipomata), lungs (LAM), and skin. Neurological manifestations include epilepsy, intellectual disability, autism spectrum disorder, and ADHD.

Common Clinical Features

Cortical tubers causing epilepsy Subependymal giant cell astrocytoma (SEGA) Renal angiomyolipomata Skin manifestations (facial angiofibromas, ash-leaf spots) Intellectual disability Autism spectrum disorder Pulmonary LAM

Clinical Trial Eligibility Tips

What to know before applying to Tuberous Sclerosis Complex trials.

TSC1 versus TSC2 mutation affects phenotype severity — TSC2 mutations are generally more severe; genotype must be documented

mTOR inhibitors (everolimus, sirolimus) are approved for SEGA, renal AML, and pulmonary LAM — prior mTOR inhibitor use and current blood levels must be documented

Seizure frequency, type, and current antiseizure medication regimen are key eligibility determinants for epilepsy trials

TSC-associated neuropsychiatric disorders (TAND) assessment scores are eligibility and outcome measures for behavioral trials

Patient Resources

Patient Organization

Tuberous Sclerosis Alliance

Visit website ↗

Natural History Registry

TSC Natural History Database

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:805)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Tuberous Sclerosis Complex trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Tuberous Sclerosis Complex. Updated daily.

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