Distal 17p13.1 microdeletion syndrome

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Overview

Distal 17p13.1 microdeletion syndrome is a rare genetic condition caused by a small missing piece (deletion) of genetic material on the short arm of chromosome 17, specifically in the 17p13.1 region. This deletion removes several genes that are important for brain development and overall growth. Because multiple genes are affected, the syndrome can impact several body systems at once. The most common features include intellectual disability, delayed speech and language development, and learning difficulties. Many children with this condition also experience delayed motor milestones, meaning they may be slower to sit, crawl, or walk. Some individuals have distinctive facial features, such as a broad forehead, widely spaced eyes, or a flat nasal bridge. Behavioral challenges, including attention difficulties and sometimes features of autism spectrum disorder, have also been reported. Some affected individuals may have seizures or structural brain differences seen on MRI. There is currently no cure for distal 17p13.1 microdeletion syndrome. Treatment focuses on managing individual symptoms and supporting development. This typically includes early intervention services such as speech therapy, occupational therapy, physical therapy, and special education programs. Seizures, if present, are managed with anti-seizure medications. Regular follow-up with a team of specialists is important to monitor development and address new concerns as they arise.

Also known as:

Key symptoms:

Intellectual disabilityDelayed speech and language developmentDelayed motor milestones such as sitting and walkingLearning difficultiesDistinctive facial featuresBehavioral problems including attention difficultiesFeatures of autism spectrum disorderSeizures or epilepsyLow muscle tone (floppiness in infancy)Short stature or growth delaysFeeding difficulties in infancyStructural brain differencesPoor coordination or clumsinessAnxiety or mood difficulties

Clinical phenotype terms (23)— hover any for plain English
Increased overbiteHP:0011094Moderate global developmental delayHP:0011343Generalized joint hypermobilityHP:0002761Limited elbow movementHP:0002996Flat occiputHP:0005469Abnormal hand morphologyHP:0005922Unilateral polymicrogyriaHP:0006927
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Distal 17p13.1 microdeletion syndrome.

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No actively recruiting trials found for Distal 17p13.1 microdeletion syndrome at this time.

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No specialists are currently listed for Distal 17p13.1 microdeletion 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 Distal 17p13.1 microdeletion syndrome.

Search all travel grants →NORD Financial Assistance ↗

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 is the exact size and location of my child's deletion, and which genes are affected?,Was this deletion inherited or did it happen for the first time in my child?,What therapies should we start right away, and how often should they occur?,Should my child be screened for seizures, heart problems, or vision issues?,What educational supports should we request from the school?,Are there any clinical studies or research programs we could participate in?,What is the chance of this happening again in a future pregnancy?

Common questions about Distal 17p13.1 microdeletion syndrome

What is Distal 17p13.1 microdeletion syndrome?

Distal 17p13.1 microdeletion syndrome is a rare genetic condition caused by a small missing piece (deletion) of genetic material on the short arm of chromosome 17, specifically in the 17p13.1 region. This deletion removes several genes that are important for brain development and overall growth. Because multiple genes are affected, the syndrome can impact several body systems at once. The most common features include intellectual disability, delayed speech and language development, and learning difficulties. Many children with this condition also experience delayed motor milestones, meaning t

How is Distal 17p13.1 microdeletion syndrome inherited?

Distal 17p13.1 microdeletion syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Distal 17p13.1 microdeletion syndrome typically begin?

Typical onset of Distal 17p13.1 microdeletion syndrome is infantile. Age of onset can vary across affected individuals.