Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome

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ORPHA:480864OMIM:616878G31.8
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1Active trials8Treatment centers

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Overview

Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome is a very rare inherited condition that affects multiple body systems, including the brain, muscles, and heart. It is sometimes referred to by the abbreviation MERCA syndrome or by its Orphanet code 480864. The condition is caused by changes (mutations) in a specific gene that disrupts how cells produce energy, leading to a group of serious and recurring health problems. People with this syndrome experience repeated episodes called 'metabolic crises,' during which the body's energy systems break down. These crises can cause severe muscle breakdown (called rhabdomyolysis), dangerous heart rhythm problems (arrhythmias), and episodes of brain and muscle dysfunction (encephalomyopathy). Between crises, individuals may have intellectual disability and ongoing muscle weakness. The crises are often triggered by illness, fever, fasting, or physical stress. There is currently no cure for this condition. Treatment focuses on preventing and managing crises, protecting the heart, and supporting brain and muscle function. Early diagnosis is very important because prompt treatment during a crisis can be life-saving. A team of specialists is needed to manage the many aspects of this complex disease.

Also known as:

Key symptoms:

Repeated episodes of severe muscle breakdown (rhabdomyolysis)Irregular or dangerous heart rhythms (cardiac arrhythmia)Episodes of brain and muscle dysfunction (encephalomyopathic crises)Intellectual disability or learning difficultiesMuscle weaknessFatigue and low energyDark or brown-colored urine during crises (from muscle breakdown products)Muscle pain or crampingDevelopmental delaysSeizures during crisesConfusion or altered consciousness during episodes

Clinical phenotype terms (50)— hover any for plain English
Hypoglycemic seizuresHP:0002173Generalized tonic seizureHP:0010818Multifocal seizuresHP:0031165Elevated circulating acylcarnitine concentrationHP:0045045KetonuriaHP:0002919
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
May 2018Natural History Study and Establishment of a Biorepository-TANGO2-related Disorder

Baylor College of Medicine

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Other1 trial
Natural History Study and Establishment of a Biorepository-TANGO2-related Disorder
Actively Recruiting
· Sites: Houston, Texas

No specialists are currently listed for Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific mutations were found in the TANGO2 gene, and what do they mean for my child's prognosis?,What should our emergency action plan look like, and what should we tell emergency room staff?,Should my child take riboflavin or selenium supplements, and how will we know if they are helping?,How often does my child need heart monitoring, and what cardiac symptoms should prompt an emergency visit?,Are there any dietary restrictions or activity limits we need to follow to reduce the risk of a crisis?,Are there any clinical trials or research studies we could participate in?,What support services are available for intellectual disability and developmental delays?

Common questions about Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome

What is Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome?

Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome is a very rare inherited condition that affects multiple body systems, including the brain, muscles, and heart. It is sometimes referred to by the abbreviation MERCA syndrome or by its Orphanet code 480864. The condition is caused by changes (mutations) in a specific gene that disrupts how cells produce energy, leading to a group of serious and recurring health problems. People with this syndrome experience repeated episodes called 'metabolic crises,' during which the body's energy

How is Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome inherited?

Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome typically begin?

Typical onset of Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome is infantile. Age of onset can vary across affected individuals.

Are there clinical trials for Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome?

Yes — 1 recruiting clinical trial is currently listed for Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.