Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome

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ORPHA:457185OMIM:616276E88.8
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Overview

Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome is an extremely rare and severe genetic condition that affects newborn babies. This disease impacts multiple body systems at the same time, including the brain (encephalopathy), the muscles (myopathy), the heart (cardiomyopathy), and the lungs (respiratory distress). Babies born with this condition typically show signs of serious illness very soon after birth. The condition is caused by problems in the way cells produce energy, specifically involving defects in mitochondrial function — the tiny power plants inside every cell. When these energy-producing systems do not work properly, organs that need the most energy, such as the brain, heart, and muscles, are affected the most. Affected newborns may have difficulty breathing, poor muscle tone (floppiness), seizures, feeding difficulties, and signs of heart failure. Because this disease is so rare, treatment options are very limited and are mainly supportive. This means doctors focus on managing symptoms such as breathing support, heart medications, and seizure control rather than treating the underlying cause. Research is ongoing, but currently there is no cure. The prognosis is generally very poor, and many affected infants face life-threatening complications early in life. Families dealing with this diagnosis should work closely with a team of specialists to ensure the best possible care and support.

Also known as:

Key symptoms:

Severe breathing difficulties at birthWeak or floppy muscles (poor muscle tone)Heart muscle weakness (cardiomyopathy)SeizuresFeeding difficultiesBrain dysfunction (encephalopathy)Lactic acidosis (buildup of acid in the blood)Failure to thrive or poor weight gainLow energy and lethargyOrgan failureAbnormal movementsPoor reflexesSwelling due to heart failure

Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome.

View clinical trials →

No actively recruiting trials found for Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome community →

No specialists are currently listed for Neonatal encephalomyopathy-cardiomyopathy-respiratory distress 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 Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is the specific genetic cause of my child's condition, and has genetic testing been completed?,What is the expected course of this disease for my child?,What supportive treatments are available, and what are their risks and benefits?,Should we consider palliative care, and what does that involve?,Are there any clinical trials or experimental treatments that might be appropriate?,What should we watch for at home that would require emergency medical attention?,Is genetic counseling available for our family to understand the risk for future pregnancies?

Common questions about Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome

What is Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome?

Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome is an extremely rare and severe genetic condition that affects newborn babies. This disease impacts multiple body systems at the same time, including the brain (encephalopathy), the muscles (myopathy), the heart (cardiomyopathy), and the lungs (respiratory distress). Babies born with this condition typically show signs of serious illness very soon after birth. The condition is caused by problems in the way cells produce energy, specifically involving defects in mitochondrial function — the tiny power plants inside every c

How is Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome inherited?

Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome typically begin?

Typical onset of Neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome is neonatal. Age of onset can vary across affected individuals.