Muscle-eye-brain disease with bilateral multicystic leucodystrophy

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ORPHA:370997OMIM:616538G71.0
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Overview

Muscle-eye-brain disease with bilateral multicystic leucodystrophy is an extremely rare and severe genetic condition that affects the muscles, eyes, and brain. It belongs to a group of disorders known as dystroglycanopathies, which are caused by problems in how certain proteins are processed in the body. These proteins are important for the normal development and function of muscles and the brain. In this condition, babies are typically born with significant muscle weakness (a type of congenital muscular dystrophy), serious eye abnormalities that can cause vision problems or blindness, and severe brain malformations. The brain shows a specific pattern of damage called bilateral multicystic leucodystrophy, meaning there are multiple cyst-like areas in the white matter on both sides of the brain. This leads to profound intellectual disability and developmental delays. Children with this condition usually have very limited motor development and may never achieve independent sitting or walking. Seizures are common. Unfortunately, there is currently no cure for this disease. Treatment focuses on managing symptoms, providing supportive care, and improving quality of life through therapies such as physical therapy, seizure management, nutritional support, and vision care. The condition is life-limiting, and affected children require intensive medical and caregiving support throughout their lives.

Also known as:

Key symptoms:

Severe muscle weakness from birth (floppy baby)Poor or absent muscle toneSevere intellectual disabilityEye abnormalities such as severe nearsightedness or glaucomaVision loss or blindnessSeizures or epilepsyInability to sit, stand, or walk independentlyFeeding difficulties and poor weight gainAbnormal brain development visible on MRIMultiple cysts in the white matter of the brainDelayed or absent speechBreathing difficultiesJoint contractures (stiff joints)Hydrocephalus (fluid buildup in the brain)

Clinical phenotype terms (23)— hover any for plain English
Cerebellar cystHP:0002350Abnormal brainstem morphologyHP:0002363LeukodystrophyHP:0002415Abnormal pons morphologyHP:0007361EEG with focal spike wavesHP:0011197Delayed ability to sitHP:0025336Hypoglycosylation of alpha-dystroglycanHP:0030046AgyriaHP:0031882
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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Muscle-eye-brain disease with bilateral multicystic leucodystrophy.

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No actively recruiting trials found for Muscle-eye-brain disease with bilateral multicystic leucodystrophy at this time.

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No specialists are currently listed for Muscle-eye-brain disease with bilateral multicystic leucodystrophy.

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 Muscle-eye-brain disease with bilateral multicystic leucodystrophy.

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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

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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 genetic mutation does my child have, and does it help predict the disease course?,What is the best plan for managing my child's seizures?,Should my child have a feeding tube, and when is the right time to consider one?,What therapies (physical, occupational) are most helpful at this stage?,Are there any clinical trials or research studies my child might be eligible for?,What palliative care and support services are available for our family?,What is the chance of having another child with this condition, and should we consider genetic counseling?

Common questions about Muscle-eye-brain disease with bilateral multicystic leucodystrophy

What is Muscle-eye-brain disease with bilateral multicystic leucodystrophy?

Muscle-eye-brain disease with bilateral multicystic leucodystrophy is an extremely rare and severe genetic condition that affects the muscles, eyes, and brain. It belongs to a group of disorders known as dystroglycanopathies, which are caused by problems in how certain proteins are processed in the body. These proteins are important for the normal development and function of muscles and the brain. In this condition, babies are typically born with significant muscle weakness (a type of congenital muscular dystrophy), serious eye abnormalities that can cause vision problems or blindness, and se

How is Muscle-eye-brain disease with bilateral multicystic leucodystrophy inherited?

Muscle-eye-brain disease with bilateral multicystic leucodystrophy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Muscle-eye-brain disease with bilateral multicystic leucodystrophy typically begin?

Typical onset of Muscle-eye-brain disease with bilateral multicystic leucodystrophy is neonatal. Age of onset can vary across affected individuals.