Neuromuscular

McArdle Disease

Also known as GSD type V, glycogen storage disease type 5, myophosphorylase deficiency

McArdle Disease is caused by bi-allelic mutations in the PYGM gene, resulting in complete absence of myophosphorylase — the muscle isoform of glycogen phosphorylase — which blocks glycogen breakdown in skeletal muscle during exercise. This

ORPHA:368 ↗Gene PYGMPrevalence 1 in 100,000 to 1 in 167,000Onset Childhood (symptoms typically apparent by age 10, though diagnosis often delayed)Autosomal recessive

0

studies recruiting now

as of 7 Sept 2026

17

studies registered in total

as of 7 Sept 2026

0

countries with a recruiting site

as of 7 Sept 2026

None

recruiting study posted to date

among recruiting studies

Recruiting trials

No recruiting trial found right now.

17 studies are registered for McArdle Disease, but none was recruiting as of 7 Sept 2026. Here is what is still worth doing.

Keep watching

Get an email when a new McArdle Disease study opens.

One email a day at most. Unsubscribe with one click.

Used only for these alerts. Privacy.

Support

Patient organisations

Association for Glycogen Storage DiseasePatient association
Visit website ↗

Registry: IamGSD Registry · Join ↗. Registries connect patients to researchers and often hear about trials first.

About McArdle Disease

McArdle Disease is caused by bi-allelic mutations in the PYGM gene, resulting in complete absence of myophosphorylase — the muscle isoform of glycogen phosphorylase — which blocks glycogen breakdown in skeletal muscle during exercise. This leads to exercise intolerance, myalgia, and rhabdomyolysis, but the pathognomonic 'second wind' phenomenon — a spontaneous improvement in exercise capacity after 8–10 minutes — distinguishes it from other exercise-induced myopathies. Severe rhabdomyolysis can cause acute kidney injury.

Common clinical features

Exercise intolerance, myalgia, and early fatigue during exertionSecond wind phenomenon (recovery of exercise tolerance after 8–10 minutes)Rhabdomyolysis with dark (cola-coloured) urine after intense exerciseMuscle cramps and contractures during exercisePersistent proximal weakness in older patientsMarkedly elevated serum creatine kinase (even at rest)Myoglobinuria and risk of acute renal failure

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Treatments being studied

3 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.

Phase 2Triheptanoin (Dojolvi)
Phase 2Valproate Sodium (Delepsine)
Phase 1Mavodelpar

Before you apply

Things trial teams commonly ask about for McArdle Disease. Not eligibility rules; those are set by each study.

  • Forearm ischaemic (or non-ischaemic) exercise test showing absent venous lactate rise with normal ammonia rise is diagnostic and frequently documented as part of eligibility screening
  • Genetic confirmation of bi-allelic PYGM mutations is required; the common p.R50X variant is found in approximately 60% of European alleles and can be rapidly screened
  • Cardiopulmonary exercise testing (CPET) and peak VO2 are increasingly used as primary endpoints; a structured exercise test with a physiologist prior to trial application is highly recommended

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).