Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome

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ORPHA:2579OMIM:158500G11.1
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1Specialists8Treatment centers

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Overview

Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome (sometimes called NARP-like syndrome or Barnard-Scholz syndrome) is an extremely rare multi-system disorder that affects the muscles, nervous system, eyes, and metabolism. The condition is characterized by a combination of four main features: progressive muscle wasting (muscular atrophy), problems with coordination and balance (ataxia), a degenerative eye condition that leads to vision loss (retinitis pigmentosa), and diabetes mellitus. Because this syndrome affects multiple organ systems at once, patients often experience a wide range of symptoms that worsen over time. The muscle wasting typically affects the limbs, making it harder to walk, grip objects, or perform daily tasks. Ataxia causes unsteadiness and difficulty with coordinated movements. Retinitis pigmentosa gradually destroys the light-sensing cells in the back of the eye, leading to night blindness and tunnel vision that can progress to significant vision loss. Diabetes mellitus means the body has trouble regulating blood sugar levels, which requires ongoing monitoring and treatment. There is currently no cure for this syndrome. Treatment focuses on managing each symptom individually. This may include physical therapy for muscle weakness, mobility aids, blood sugar management with insulin or oral medications, and low-vision aids or other ophthalmologic interventions. A coordinated team of specialists is essential for providing the best possible care.

Also known as:

Key symptoms:

Progressive muscle wasting and weaknessPoor balance and coordination (ataxia)Night blindnessTunnel vision or progressive vision lossHigh blood sugar or diabetesDifficulty walkingFatigue and low energyNumbness or tingling in hands and feetUnsteady gaitMuscle cramps or stiffnessDifficulty with fine motor tasks like writingWeight loss due to muscle lossFrequent urination and thirst from diabetes

Clinical phenotype terms (4)— hover any for plain English
Inheritance

Mitochondrial

Passed from mother to child through the energy-producing parts of the cell

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 Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome.

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No actively recruiting trials found for Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome at this time.

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Specialists

1 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 Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What genetic testing should be done to confirm the diagnosis, and should family members be tested?,How quickly might the muscle weakness and vision loss progress in my case?,What is the best plan for managing my diabetes alongside the other symptoms?,Are there any supplements like coenzyme Q10 that might help with mitochondrial function?,What physical therapy or rehabilitation programs do you recommend?,Are there any clinical trials or emerging treatments I should know about?,How often should I have eye exams and neurological check-ups?

Common questions about Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome

What is Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome?

Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome (sometimes called NARP-like syndrome or Barnard-Scholz syndrome) is an extremely rare multi-system disorder that affects the muscles, nervous system, eyes, and metabolism. The condition is characterized by a combination of four main features: progressive muscle wasting (muscular atrophy), problems with coordination and balance (ataxia), a degenerative eye condition that leads to vision loss (retinitis pigmentosa), and diabetes mellitus. Because this syndrome affects multiple organ systems at once, patients often experience

How is Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome inherited?

Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome follows a mitochondrial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome?

1 specialists and care centers treating Muscular atrophy-ataxia-retinitis pigmentosa-diabetes mellitus syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.