Blepharophimosis-intellectual disability syndrome, MKB type

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ORPHA:293707OMIM:300895Q87.8
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Overview

Blepharophimosis-intellectual disability syndrome, MKB type (also known as MKB syndrome, named after the initials of the researchers who first described it) is an extremely rare genetic condition that affects multiple parts of the body. The hallmark features include blepharophimosis, which means the eye openings are abnormally narrow, combined with intellectual disability of varying degrees. People with this condition may also have distinctive facial features, short stature, and other developmental differences. The syndrome typically becomes apparent at birth or during early infancy, when the narrow eye openings and unusual facial features are first noticed. Intellectual disability may range from mild to moderate, and affected children often experience delays in reaching developmental milestones such as sitting, walking, and speaking. Some individuals may also have additional physical findings such as small head size (microcephaly), ear abnormalities, and skeletal differences. Because this condition is so rare, there is no specific cure or targeted treatment available. Management focuses on addressing individual symptoms and supporting development. This may include special education services, speech therapy, occupational therapy, and surgical correction of eye abnormalities if needed. Regular follow-up with multiple specialists is important to monitor growth, development, and any associated health concerns.

Also known as:

Key symptoms:

Narrow eye openings (blepharophimosis)Intellectual disabilityDelayed speech and language developmentShort statureSmall head sizeDistinctive facial featuresDelayed motor milestonesEar abnormalitiesDroopy eyelids (ptosis)Learning difficultiesWidely spaced eyesFlat nasal bridge

Clinical phenotype terms (8)— hover any for plain English
Thick nasal alaeHP:0009928
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 Blepharophimosis-intellectual disability syndrome, MKB type.

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No actively recruiting trials found for Blepharophimosis-intellectual disability syndrome, MKB type at this time.

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No specialists are currently listed for Blepharophimosis-intellectual disability syndrome, MKB type.

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 Blepharophimosis-intellectual disability syndrome, MKB type.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the severity of my child's blepharophimosis, and will surgery be needed?,What developmental therapies should we start, and how soon?,Should we pursue genetic testing such as whole exome sequencing to confirm the diagnosis?,What is the expected range of intellectual ability for my child?,Are there any other health problems we should screen for?,How often should we schedule follow-up visits with each specialist?,Are there any clinical studies or research programs we could participate in?

Common questions about Blepharophimosis-intellectual disability syndrome, MKB type

What is Blepharophimosis-intellectual disability syndrome, MKB type?

Blepharophimosis-intellectual disability syndrome, MKB type (also known as MKB syndrome, named after the initials of the researchers who first described it) is an extremely rare genetic condition that affects multiple parts of the body. The hallmark features include blepharophimosis, which means the eye openings are abnormally narrow, combined with intellectual disability of varying degrees. People with this condition may also have distinctive facial features, short stature, and other developmental differences. The syndrome typically becomes apparent at birth or during early infancy, when the

How is Blepharophimosis-intellectual disability syndrome, MKB type inherited?

Blepharophimosis-intellectual disability syndrome, MKB type follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Blepharophimosis-intellectual disability syndrome, MKB type typically begin?

Typical onset of Blepharophimosis-intellectual disability syndrome, MKB type is neonatal. Age of onset can vary across affected individuals.