Disease Directory Central Core Disease
Neuromuscular

Central Core Disease

Also known as: CCD, RYR1-related myopathy, central core myopathy

Prevalence

Less than 1 in 100,000

Onset

Congenital or infancy

Type

Autosomal dominant (occasionally recessive)

Gene

RYR1

About Central Core Disease

Central Core Disease is a congenital myopathy caused by mutations in the RYR1 gene encoding the ryanodine receptor 1, which regulates calcium release from the sarcoplasmic reticulum in skeletal muscle. It is characterised by central cores — areas of reduced oxidative enzyme activity — seen on muscle biopsy. A critical associated risk is malignant hyperthermia susceptibility, a potentially fatal hypermetabolic response to volatile anaesthetic agents and succinylcholine.

Common Clinical Features

Neonatal or infantile hypotonia Proximal limb muscle weakness, predominantly lower limb Hip dislocation or dysplasia Delayed motor milestones (walking often delayed to 18–24 months) Scoliosis Malignant hyperthermia susceptibility Relatively non-progressive course in most patients

Clinical Trial Eligibility Tips

What to know before applying to Central Core Disease trials.

Muscle biopsy with NADH-TR staining showing central cores is essential for diagnosis confirmation required by most trials; electron microscopy may be additionally requested

All RYR1-related trials require documentation of MH risk status and prior anaesthetic history — carry an MH alert card to all pre-trial assessments

RYR1 mutation spectrum is broad (dominant vs recessive, gain-of-function vs loss-of-function); genotype-stratified trials are increasingly common so precise variant characterisation matters

Patient Resources

Patient Organization

RYR1 Foundation

Visit website ↗

Natural History Registry

RYR1 Foundation Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:597)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Central Core Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Central Core Disease. Updated daily.

Related Rare Diseases