Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies

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Overview

Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies (also known by the abbreviation CMD-dystroglycanopathy, and sometimes called Walker-Warburg syndrome or related conditions depending on the specific gene involved) is a rare inherited muscle disease that is present from birth. It belongs to a group of conditions called dystroglycanopathies, where a key protein called alpha-dystroglycan does not work properly because it is not correctly modified with sugar molecules. This protein normally acts like a glue that holds muscle cells and brain cells together and in the right place. The disease affects multiple body systems at once. Muscles are very weak from birth, the brain does not develop its normal shape and structure, and the eyes often have serious problems. Babies with this condition typically have very low muscle tone (called 'floppy baby' syndrome), significant intellectual disability, seizures, and vision problems that can range from severe nearsightedness to complete blindness. There is currently no cure for this condition. Treatment focuses on managing symptoms, supporting development, and improving quality of life. This includes physical therapy, seizure medications, eye care, and nutritional support. The severity can vary depending on which gene is affected and the specific changes in that gene.

Also known as:

Key symptoms:

Very low muscle tone from birth (floppy baby)Severe muscle weaknessIntellectual disability ranging from moderate to profoundSeizures or epilepsyVision problems or blindnessAbnormal brain structure (such as smooth brain or cobblestone brain appearance)Difficulty feeding as a newbornLittle or no ability to sit, stand, or walkEye abnormalities such as small eyes (microphthalmia) or cloudy lenses (cataracts)Hydrocephalus (fluid buildup in the brain) in some casesBreathing difficultiesDelayed or absent speech and language developmentElevated muscle enzyme levels in the blood (creatine kinase)

Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies.

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No actively recruiting trials found for Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies at this time.

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No specialists are currently listed for Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies.

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 Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies.

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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.Which specific gene is causing my child's condition, and what does that mean for how severe it will be?,What brain and eye findings were seen on imaging, and how will these affect my child's development?,What therapies — physical, occupational, speech — should we start right away, and how often?,What is the plan for managing seizures, and when should I call for emergency help?,Will my child need breathing support now or in the future, and how do we prepare for that?,Are there any clinical trials or research studies we should know about?,What does this diagnosis mean for future pregnancies, and should other family members be tested?

Common questions about Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies

What is Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies?

Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies (also known by the abbreviation CMD-dystroglycanopathy, and sometimes called Walker-Warburg syndrome or related conditions depending on the specific gene involved) is a rare inherited muscle disease that is present from birth. It belongs to a group of conditions called dystroglycanopathies, where a key protein called alpha-dystroglycan does not work properly because it is not correctly modified with sugar molecules. This protein normally acts like a glue that holds muscle cells and brain cells together and in the right p

How is Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies inherited?

Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies typically begin?

Typical onset of Congenital muscular alpha-dystroglycanopathy with brain and eye anomalies is neonatal. Age of onset can vary across affected individuals.