OBSOLETE: Congenital myopathy with vacuoles

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ORPHA:172985
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8Treatment centers

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Overview

Congenital myopathy with vacuoles is an extremely rare inherited muscle disorder that was previously recognized as a distinct condition in medical databases but has since been reclassified (marked as 'obsolete' in Orphanet). This means that cases once grouped under this name are now understood to fall under other, more precisely defined congenital myopathy subtypes. Congenital myopathies are a group of muscle diseases present from birth or early infancy that cause muscle weakness and poor muscle tone (hypotonia). In this particular form, muscle biopsy samples showed characteristic tiny fluid-filled spaces called vacuoles within the muscle fibers, which helped distinguish it from other congenital myopathies at the time. Patients with this condition typically experienced generalized muscle weakness, reduced muscle tone from birth, delayed motor milestones such as sitting and walking, and sometimes breathing difficulties. The severity could range from mild weakness to more significant disability. Because this diagnosis is now obsolete, patients who were previously given this label may benefit from updated genetic testing and re-evaluation to receive a more specific and current diagnosis. Treatment has generally been supportive, focusing on physical therapy, respiratory support when needed, and orthopedic management. There are no specific curative therapies, but advances in genetic understanding continue to improve diagnosis and may open doors to targeted treatments in the future.

Key symptoms:

Muscle weakness present from birthLow muscle tone (floppy baby)Delayed motor milestones like sitting and walkingDifficulty breathingFeeding difficulties in infancyFatigue and reduced enduranceThin or underdeveloped musclesSkeletal abnormalities such as scoliosisJoint contractures (stiff joints)Facial muscle weaknessDifficulty swallowing

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Congenital myopathy with vacuoles.

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No actively recruiting trials found for OBSOLETE: Congenital myopathy with vacuoles at this time.

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No specialists are currently listed for OBSOLETE: Congenital myopathy with vacuoles.

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 OBSOLETE: Congenital myopathy with vacuoles.

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Community

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Latest news about OBSOLETE: Congenital myopathy with vacuoles

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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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.Since this diagnosis is now considered obsolete, what is my (or my child's) updated diagnosis?,Should we pursue updated genetic testing such as whole exome sequencing?,What is the expected course of this condition based on the specific genetic findings?,What therapies and supports should we start right away?,Are there any clinical trials or emerging treatments that might be relevant?,How should we monitor and manage breathing problems?,What specialists should be part of our care team?

Common questions about OBSOLETE: Congenital myopathy with vacuoles

What is OBSOLETE: Congenital myopathy with vacuoles?

Congenital myopathy with vacuoles is an extremely rare inherited muscle disorder that was previously recognized as a distinct condition in medical databases but has since been reclassified (marked as 'obsolete' in Orphanet). This means that cases once grouped under this name are now understood to fall under other, more precisely defined congenital myopathy subtypes. Congenital myopathies are a group of muscle diseases present from birth or early infancy that cause muscle weakness and poor muscle tone (hypotonia). In this particular form, muscle biopsy samples showed characteristic tiny fluid-f

At what age does OBSOLETE: Congenital myopathy with vacuoles typically begin?

Typical onset of OBSOLETE: Congenital myopathy with vacuoles is neonatal. Age of onset can vary across affected individuals.