Alpha-B crystallin-related late-onset myopathy

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ORPHA:399058OMIM:608810G71.0
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Overview

Alpha-B crystallin-related late-onset myopathy is a rare inherited muscle disease caused by changes (mutations) in the CRYAB gene. This gene provides instructions for making a protein called alpha-B crystallin, which plays an important role in protecting muscle cells from stress and damage. When this protein does not work properly, muscle fibers gradually break down over time, leading to progressive muscle weakness. This condition typically begins in adulthood, often after age 40, and primarily affects the muscles of the limbs. People with this disease may first notice difficulty walking, climbing stairs, or lifting objects. Over time, the weakness can spread to other muscle groups. Some patients may also develop problems with their heart muscle (cardiomyopathy) or develop cataracts, since alpha-B crystallin is also found in the eye lens and heart tissue. There is currently no cure for this condition. Treatment focuses on managing symptoms, maintaining mobility through physical therapy, and monitoring for heart involvement. The disease tends to progress slowly, but the rate of progression can vary from person to person. Because it is so rare, awareness among healthcare providers is limited, which can lead to delays in diagnosis.

Also known as:

Key symptoms:

Progressive muscle weakness in the arms and legsDifficulty walking or climbing stairsTrouble lifting objects overheadMuscle wasting (loss of muscle bulk)Stiffness or rigidity in musclesDifficulty swallowing in some casesHeart muscle problems (cardiomyopathy)Cataracts or vision changesFatigue and reduced enduranceBreathing difficulties in advanced stagesMuscle cramps or painDifficulty rising from a seated position

Clinical phenotype terms (21)— hover any for plain English
Progressive distal muscle weaknessHP:0009063Limb-girdle muscle weaknessHP:0003325Autophagic vacuolesHP:0003736Accumulation of muscle fiber desminHP:0030225Abnormal circulating creatine kinase concentrationHP:0040081Posterior capsular cataractHP:0100020Muscle fiber inclusion bodiesHP:0100299Facial diplegiaHP:0001349
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Alpha-B crystallin-related late-onset myopathy.

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No actively recruiting trials found for Alpha-B crystallin-related late-onset myopathy at this time.

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No specialists are currently listed for Alpha-B crystallin-related late-onset 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 Alpha-B crystallin-related late-onset myopathy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Alpha-B crystallin-related late-onset myopathy

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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 specific CRYAB mutation do I have, and does it tell us anything about how my disease might progress?,How often should I have my heart checked for cardiomyopathy?,What type and intensity of exercise is safe and beneficial for me?,Should my family members be tested for this genetic change?,Are there any clinical trials or research studies I could participate in?,What signs should I watch for that would mean I need to come in sooner than my next appointment?,Can you refer me to a physical therapist who has experience with neuromuscular diseases?

Common questions about Alpha-B crystallin-related late-onset myopathy

What is Alpha-B crystallin-related late-onset myopathy?

Alpha-B crystallin-related late-onset myopathy is a rare inherited muscle disease caused by changes (mutations) in the CRYAB gene. This gene provides instructions for making a protein called alpha-B crystallin, which plays an important role in protecting muscle cells from stress and damage. When this protein does not work properly, muscle fibers gradually break down over time, leading to progressive muscle weakness. This condition typically begins in adulthood, often after age 40, and primarily affects the muscles of the limbs. People with this disease may first notice difficulty walking, cli

How is Alpha-B crystallin-related late-onset myopathy inherited?

Alpha-B crystallin-related late-onset myopathy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Alpha-B crystallin-related late-onset myopathy typically begin?

Typical onset of Alpha-B crystallin-related late-onset myopathy is late onset. Age of onset can vary across affected individuals.