Constitutional megaloblastic anemia with severe neurologic disease

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ORPHA:319651OMIM:613839D52.8
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Overview

Constitutional megaloblastic anemia with severe neurologic disease is an extremely rare inherited condition that affects both the blood and the nervous system. In this disease, the body has trouble properly using folate (a B vitamin), which is essential for making healthy red blood cells and for normal brain and nerve development. The term "megaloblastic anemia" means that the bone marrow produces abnormally large, immature red blood cells that do not work properly, leading to low red blood cell counts (anemia). This causes fatigue, weakness, and pale skin. What makes this condition particularly serious is the severe neurologic involvement. Affected individuals may experience seizures, intellectual disability, difficulty with movement and coordination, and other brain-related problems that can begin early in life. The neurologic symptoms can be progressive, meaning they may worsen over time if not addressed. This condition is related to defects in how the body transports or metabolizes folate within cells. Treatment typically involves folate supplementation, though the neurologic damage may not be fully reversible. Early diagnosis and treatment are critical to achieving the best possible outcomes. Because this disease is so rare, management often requires a team of specialists including hematologists, neurologists, and geneticists working together to provide comprehensive care.

Also known as:

Key symptoms:

Severe anemia (low red blood cell count)Pale skinFatigue and weaknessSeizuresIntellectual disabilityDelayed development or developmental regressionDifficulty with movement and coordinationPoor muscle tone or stiffnessDifficulty feeding in infancyFailure to thrive or poor growthAbnormally large red blood cells on blood testsSpeech and language delaysIrritability

Inheritance

Autosomal recessive

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

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Constitutional megaloblastic anemia with severe neurologic disease.

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No actively recruiting trials found for Constitutional megaloblastic anemia with severe neurologic disease at this time.

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No specialists are currently listed for Constitutional megaloblastic anemia with severe neurologic disease.

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 Constitutional megaloblastic anemia with severe neurologic disease.

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Community

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Caregiver Resources

NORD Caregiver Resources

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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.What specific genetic mutation is causing my child's condition, and does it affect treatment choices?,What is the best form and dose of folate supplementation for my child?,How will you monitor whether the treatment is working for both the anemia and the brain symptoms?,What seizure medications are safest and most effective for this condition?,What developmental therapies should we start, and how often?,Are there any clinical trials or experimental treatments available for this disease?,What is the long-term outlook for my child's development and independence?

Common questions about Constitutional megaloblastic anemia with severe neurologic disease

What is Constitutional megaloblastic anemia with severe neurologic disease?

Constitutional megaloblastic anemia with severe neurologic disease is an extremely rare inherited condition that affects both the blood and the nervous system. In this disease, the body has trouble properly using folate (a B vitamin), which is essential for making healthy red blood cells and for normal brain and nerve development. The term "megaloblastic anemia" means that the bone marrow produces abnormally large, immature red blood cells that do not work properly, leading to low red blood cell counts (anemia). This causes fatigue, weakness, and pale skin. What makes this condition particula

How is Constitutional megaloblastic anemia with severe neurologic disease inherited?

Constitutional megaloblastic anemia with severe neurologic disease follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Constitutional megaloblastic anemia with severe neurologic disease typically begin?

Typical onset of Constitutional megaloblastic anemia with severe neurologic disease is infantile. Age of onset can vary across affected individuals.