Bonnemann-Meinecke-Reich syndrome

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ORPHA:1261OMIM:225755Q04.8
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Overview

Bonnemann-Meinecke-Reich syndrome is an extremely rare genetic condition that primarily affects brain development. It is also sometimes referred to by its Orphanet designation (ORPHA:1261). The syndrome is characterized by abnormalities in the structure of the brain, particularly involving the cerebral cortex and other brain regions, which can lead to intellectual disability, seizures, and developmental delays. Because this condition affects the brain during its formation, signs are typically present from birth or early infancy. The condition was first described in the medical literature in a small number of patients, and very few cases have been reported worldwide. Due to its extreme rarity, much about the syndrome remains poorly understood, including the full range of symptoms and the precise genetic cause. Children with this condition may experience difficulties with motor skills, speech and language development, and learning. Seizures are a common feature and may require ongoing treatment with anti-seizure medications. There is currently no cure for Bonnemann-Meinecke-Reich syndrome. Treatment is supportive and focuses on managing individual symptoms. This may include anti-epileptic drugs for seizures, physical therapy, occupational therapy, and speech therapy to help maximize each child's developmental potential. A team of specialists typically works together to provide comprehensive care.

Also known as:

Key symptoms:

Intellectual disabilitySeizures or epilepsyDelayed motor developmentSpeech and language delaysAbnormal brain structure visible on imagingLow muscle tone in infancyDifficulty with coordination and balanceLearning difficultiesBehavioral challenges

Clinical phenotype terms (11)— hover any for plain English
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 Bonnemann-Meinecke-Reich syndrome.

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No actively recruiting trials found for Bonnemann-Meinecke-Reich syndrome at this time.

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No specialists are currently listed for Bonnemann-Meinecke-Reich syndrome.

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 Bonnemann-Meinecke-Reich syndrome.

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Community

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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.What specific brain abnormalities were found on my child's MRI?,Should we pursue genetic testing such as whole exome sequencing?,What seizure medications are best suited for my child, and what are the side effects?,What therapies should we start, and how often should they occur?,Are there any clinical trials or research studies we could participate in?,What developmental milestones should we be tracking?,What emergency plan should we have in place for seizures?

Common questions about Bonnemann-Meinecke-Reich syndrome

What is Bonnemann-Meinecke-Reich syndrome?

Bonnemann-Meinecke-Reich syndrome is an extremely rare genetic condition that primarily affects brain development. It is also sometimes referred to by its Orphanet designation (ORPHA:1261). The syndrome is characterized by abnormalities in the structure of the brain, particularly involving the cerebral cortex and other brain regions, which can lead to intellectual disability, seizures, and developmental delays. Because this condition affects the brain during its formation, signs are typically present from birth or early infancy. The condition was first described in the medical literature in a

At what age does Bonnemann-Meinecke-Reich syndrome typically begin?

Typical onset of Bonnemann-Meinecke-Reich syndrome is neonatal. Age of onset can vary across affected individuals.