Oculogastrointestinal muscular dystrophy

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:1876OMIM:277320G71.0
Who is this for?
Show terms as
2Specialists8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Oculogastrointestinal muscular dystrophy (OGIMD), also known as oculogastrointestinal encephalopathy or sometimes referred to in the context of mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)-like conditions, is an extremely rare inherited disorder that affects the eyes, the digestive system, and the muscles. The disease causes progressive weakness of the muscles that control eye movement (leading to drooping eyelids and difficulty moving the eyes), along with serious problems in the gastrointestinal tract such as chronic diarrhea, poor absorption of nutrients, abdominal pain, and episodes resembling intestinal blockage. Skeletal muscles throughout the body also become weak over time, which can affect walking and daily activities. Patients often experience significant weight loss and malnutrition because the digestive system does not work properly. Nerve damage (peripheral neuropathy) may also occur, causing tingling or numbness in the hands and feet. Some patients develop hearing loss or brain-related changes visible on MRI scans. There is currently no cure for oculogastrointestinal muscular dystrophy. Treatment focuses on managing symptoms, including nutritional support (sometimes through feeding tubes or intravenous nutrition), physical therapy to maintain muscle strength, and surgical interventions when intestinal complications become severe. Because the disease affects multiple organ systems, a team of specialists is needed to provide comprehensive care. Early diagnosis and proactive management of nutritional status and gastrointestinal symptoms can help improve quality of life.

Also known as:

Key symptoms:

Drooping eyelids (ptosis)Difficulty moving the eyesChronic diarrheaAbdominal pain and bloatingEpisodes resembling intestinal blockageProgressive muscle weaknessSignificant weight loss and malnutritionPoor absorption of nutrients from foodTingling or numbness in hands and feetDifficulty swallowingFatigue and low energyHearing lossThin body buildNausea and vomiting

Clinical phenotype terms (13)— hover any for plain English
Abnormal mitral valve morphologyHP:0001633GastroparesisHP:0002578Intestinal pseudo-obstructionHP:0004389External ophthalmoplegiaHP:0000544Abnormal gastric mucosa morphologyHP:0004295Spontaneous esophageal perforationHP:0005203
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Oculogastrointestinal muscular dystrophy.

View clinical trials →

No actively recruiting trials found for Oculogastrointestinal muscular dystrophy at this time.

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

Search ClinicalTrials.gov ↗Join the Oculogastrointestinal muscular dystrophy community →

Specialists

2 foundView all specialists →
MM
Michio Hirano, MD
NEW YORK, NY
Specialist
PI on 10 active trials

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 Oculogastrointestinal muscular dystrophy.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Oculogastrointestinal muscular dystrophyForum →

No community posts yet. Be the first to share your experience with Oculogastrointestinal muscular dystrophy.

Start the conversation →

Latest news about Oculogastrointestinal muscular dystrophy

No recent news articles for Oculogastrointestinal muscular dystrophy.

Follow this condition to be notified when news becomes available.

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 specific genetic test confirmed my diagnosis, and what does the result mean for my family?,How should we monitor and manage my nutritional needs over time?,What are the warning signs of a gastrointestinal emergency I should watch for?,Are there any clinical trials or experimental treatments available for my condition?,How often should I see each specialist, and how will my care be coordinated?,What can I do to maintain my muscle strength and mobility for as long as possible?,Should my family members be tested for this condition?

Common questions about Oculogastrointestinal muscular dystrophy

What is Oculogastrointestinal muscular dystrophy?

Oculogastrointestinal muscular dystrophy (OGIMD), also known as oculogastrointestinal encephalopathy or sometimes referred to in the context of mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)-like conditions, is an extremely rare inherited disorder that affects the eyes, the digestive system, and the muscles. The disease causes progressive weakness of the muscles that control eye movement (leading to drooping eyelids and difficulty moving the eyes), along with serious problems in the gastrointestinal tract such as chronic diarrhea, poor absorption of nutrients, abdominal pain, an

How is Oculogastrointestinal muscular dystrophy inherited?

Oculogastrointestinal muscular dystrophy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Oculogastrointestinal muscular dystrophy?

2 specialists and care centers treating Oculogastrointestinal muscular dystrophy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.