Benign Samaritan congenital myopathy

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Overview

Benign Samaritan congenital myopathy is a very rare inherited muscle disease that is present from birth or early childhood. 'Congenital' means it is present at birth, and 'myopathy' means it affects the muscles. This condition belongs to a group of diseases called congenital myopathies, which cause muscle weakness and low muscle tone (sometimes called 'floppy baby' syndrome) from a very early age. The word 'benign' in the name reflects that, compared to some other congenital myopathies, the disease course tends to be relatively stable and non-progressive or only slowly progressive in many patients, meaning the muscle weakness does not worsen dramatically over time for most people. The main features include muscle weakness, particularly in the muscles closest to the center of the body (like the hips, shoulders, and trunk), reduced muscle tone, and sometimes breathing difficulties. Some children may have delayed motor milestones, such as sitting up or walking later than expected. Facial muscles can also be affected in some cases, leading to a distinctive facial appearance. There is currently no cure for Benign Samaritan congenital myopathy. Treatment focuses on managing symptoms and improving quality of life. This includes physical therapy to maintain strength and mobility, respiratory support if breathing is affected, and orthopedic care for complications like scoliosis. A team of specialists works together to support patients throughout their lives. Research into this and related congenital myopathies is ongoing.

Key symptoms:

Muscle weakness, especially in the shoulders, hips, and trunkLow muscle tone (floppy muscles) from birth or early infancyDelayed motor milestones such as late sitting, standing, or walkingDifficulty climbing stairs or rising from the floorReduced exercise tolerance and easy fatigueBreathing difficulties, especially during sleep or illnessWeak facial muscles, which may cause a flat or expressionless appearanceScoliosis (curved spine) in some patientsDifficulty swallowing in some casesFoot deformities such as clubfoot in some newborns

Clinical phenotype terms (19)— hover any for plain English
Weak cryHP:0001612Myopathic faciesHP:0002058Abnormal respiratory system physiologyHP:0002795Centrally nucleated skeletal muscle fibersHP:0003687Frog-leg postureHP:0031139Internally nucleated skeletal muscle fibersHP:0031237
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Benign Samaritan congenital myopathy.

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No actively recruiting trials found for Benign Samaritan congenital myopathy at this time.

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

Search ClinicalTrials.gov ↗Join the Benign Samaritan congenital myopathy community →

No specialists are currently listed for Benign Samaritan congenital myopathy.

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 Benign Samaritan congenital myopathy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What gene is responsible for this condition in my family, and should other family members be tested?,How often should my breathing be monitored, and what signs should prompt me to seek urgent care?,What therapies — physical, occupational, or respiratory — do you recommend, and how often should we do them?,Are there any clinical trials or research studies I or my child could participate in?,What should I watch for that might indicate the condition is getting worse?,Are there any activity restrictions, or are there exercises that are particularly helpful or harmful?,What support services or patient organizations are available to help our family?

Common questions about Benign Samaritan congenital myopathy

What is Benign Samaritan congenital myopathy?

Benign Samaritan congenital myopathy is a very rare inherited muscle disease that is present from birth or early childhood. 'Congenital' means it is present at birth, and 'myopathy' means it affects the muscles. This condition belongs to a group of diseases called congenital myopathies, which cause muscle weakness and low muscle tone (sometimes called 'floppy baby' syndrome) from a very early age. The word 'benign' in the name reflects that, compared to some other congenital myopathies, the disease course tends to be relatively stable and non-progressive or only slowly progressive in many pati

How is Benign Samaritan congenital myopathy inherited?

Benign Samaritan congenital myopathy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Benign Samaritan congenital myopathy typically begin?

Typical onset of Benign Samaritan congenital myopathy is neonatal. Age of onset can vary across affected individuals.