Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy

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ORPHA:329336OMIM:616479G71.3
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1Active trials8Treatment centers

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Overview

Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy (often called adult-onset CPEO with myopathy) is a rare condition that affects the muscles, particularly those that control eye movement and the eyelids. It is caused by problems in the mitochondria, which are the tiny energy-producing structures inside every cell of your body. When mitochondria don't work properly, the muscles that need the most energy are usually affected first. The hallmark symptoms are a gradual drooping of the eyelids (called ptosis) and a progressive inability to move the eyes fully in all directions (ophthalmoplegia). Because this form begins in adulthood, many people notice these eye problems developing slowly in their 20s, 30s, or later. Over time, weakness can spread to other muscles in the body, particularly those in the limbs and sometimes the throat, leading to difficulty swallowing or exercise intolerance. There is currently no cure for this condition. Treatment focuses on managing symptoms and maintaining quality of life. This may include surgery to correct eyelid drooping, physical therapy to preserve muscle strength, and supportive care from a team of specialists. Some patients are given supplements like coenzyme Q10 or L-carnitine to support mitochondrial function, though strong evidence for their effectiveness is limited. Research into mitochondrial disease therapies is ongoing and offers hope for future treatments.

Also known as:

Key symptoms:

Drooping eyelids (ptosis)Difficulty moving the eyes in all directionsMuscle weakness in the arms and legsExercise intolerance and easy fatigueDifficulty swallowing (dysphagia)Double visionGeneralized muscle wastingWeakness in facial musclesDifficulty with physical activities like climbing stairsHearing loss in some casesHeart rhythm problems in some casesReduced stamina and endurance

Clinical phenotype terms (32)— hover any for plain English
Progressive external ophthalmoplegiaHP:0000590Limb muscle weaknessHP:0003690Areflexia of lower limbsHP:0002522Fatigable weakness of respiratory musclesHP:0030196Weakness of facial musculatureHP:0030319
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
May 2023A Study to Evaluate Vitamin B3 Derivative to Treat Mitochondrial Myopathy

Ralitza Gavrilova — PHASE2

TrialACTIVE NOT RECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy.

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

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Phase 21 trial
A Study to Evaluate Vitamin B3 Derivative to Treat Mitochondrial Myopathy
Phase 2
Active
PI: Ralitza H. Gavrilova, MD (Mayo Clinic) · Sites: Rochester, Minnesota · Age: 1899 yrs

No specialists are currently listed for Adult-onset chronic progressive external ophthalmoplegia with mitochondrial 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 Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy.

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific genetic mutation is causing my condition, and does it affect my family members?,How quickly is this disease likely to progress in my case?,Are there any medications or substances I should avoid because of my mitochondrial disease?,Should I have my heart checked regularly, and how often?,Would supplements like coenzyme Q10 or L-carnitine be helpful for me?,What are my options for managing my drooping eyelids?,Are there any clinical trials or new treatments I should know about?

Common questions about Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy

What is Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy?

Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy (often called adult-onset CPEO with myopathy) is a rare condition that affects the muscles, particularly those that control eye movement and the eyelids. It is caused by problems in the mitochondria, which are the tiny energy-producing structures inside every cell of your body. When mitochondria don't work properly, the muscles that need the most energy are usually affected first. The hallmark symptoms are a gradual drooping of the eyelids (called ptosis) and a progressive inability to move the eyes fully in

At what age does Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy typically begin?

Typical onset of Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy?

Yes — 1 recruiting clinical trial is currently listed for Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.