ZTTK syndrome

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ORPHA:500150OMIM:617140Q87.8
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Overview

ZTTK syndrome (also called Zhu-Tokita-Takenouchi-Kim syndrome) is a rare genetic condition caused by changes in a gene called SON. This gene plays an important role in how cells process genetic instructions, and when it does not work properly, it can affect the development of many parts of the body — especially the brain. The condition was first described in 2015 and only a small number of people worldwide have been diagnosed so far. People with ZTTK syndrome typically have intellectual disability, which can range from mild to severe, along with delayed development in areas like talking, walking, and learning. Many also have weak muscle tone (called hypotonia), which can make movement and feeding difficult, especially in infancy. Seizures are common, and some individuals have structural differences in the brain that can be seen on MRI scans. Other features may include vision problems, feeding difficulties, and distinctive facial features. There is currently no cure for ZTTK syndrome. Treatment focuses on managing symptoms and supporting development. This usually involves a team of specialists and therapies such as speech therapy, physical therapy, and occupational therapy. Seizures are managed with anti-epileptic medications. Early intervention and educational support can make a meaningful difference in quality of life.

Also known as:

Key symptoms:

Intellectual disability (difficulty with learning and thinking)Delayed speech and language developmentDelayed motor milestones such as sitting and walkingLow muscle tone (hypotonia)Seizures or epilepsyFeeding difficulties, especially in infancyDistinctive facial featuresBrain structural differences visible on MRIVision problemsShort statureBehavioral challengesSkeletal abnormalities such as scoliosis or joint problems

Clinical phenotype terms (50)— hover any for plain English
Abnormal cerebral cortex morphologyHP:0002538Proportionate short statureHP:0003508
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

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 ZTTK syndrome.

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No actively recruiting trials found for ZTTK syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the ZTTK syndrome community →

No specialists are currently listed for ZTTK 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 ZTTK syndrome.

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Community

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Latest news about ZTTK syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 type of genetic change was found in the SON gene, and what does that mean for my child's outlook?,Should other family members be tested, and what is the chance of this happening again in a future pregnancy?,What therapies do you recommend starting right away, and how often should they happen?,What type of seizures does my child have, and what is the best medication to manage them?,Are there any ZTTK syndrome specialists or research centers we should connect with?,What developmental milestones should we realistically expect, and how can we best support progress?,Are there any clinical trials or research studies we could participate in?

Common questions about ZTTK syndrome

What is ZTTK syndrome?

ZTTK syndrome (also called Zhu-Tokita-Takenouchi-Kim syndrome) is a rare genetic condition caused by changes in a gene called SON. This gene plays an important role in how cells process genetic instructions, and when it does not work properly, it can affect the development of many parts of the body — especially the brain. The condition was first described in 2015 and only a small number of people worldwide have been diagnosed so far. People with ZTTK syndrome typically have intellectual disability, which can range from mild to severe, along with delayed development in areas like talking, walk

How is ZTTK syndrome inherited?

ZTTK syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does ZTTK syndrome typically begin?

Typical onset of ZTTK syndrome is neonatal. Age of onset can vary across affected individuals.