Intellectual disability-polydactyly-uncombable hair syndrome

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Overview

Intellectual disability-polydactyly-uncombable hair syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. As the name suggests, the three main features are intellectual disability (difficulty with learning and thinking skills), polydactyly (being born with extra fingers or toes), and uncombable hair (hair that is dry, frizzy, and stands away from the scalp, making it very difficult to comb flat). The hair often has a silvery or straw-like appearance. Children with this syndrome may also have distinctive facial features, such as a broad nose, and may experience delays in reaching developmental milestones like sitting, walking, and talking. Because this condition is so rare, with only a handful of cases described in the medical literature, the understanding of its full range of symptoms and long-term outlook is still limited. There is currently no cure for this syndrome. Treatment focuses on managing individual symptoms, such as special education support for intellectual disability, surgical correction of extra digits if needed, and gentle hair care routines. A team of specialists typically works together to provide the best care for affected individuals. Early intervention services, including speech therapy and occupational therapy, can help children reach their full potential.

Also known as:

Key symptoms:

Intellectual disability or learning difficultiesExtra fingers or toes (polydactyly)Dry, frizzy hair that cannot be combed flatSilvery or straw-colored hairDelayed speech and language developmentDelayed motor milestones like walkingDistinctive facial featuresBroad or flat noseShort statureLow muscle tone

Clinical phenotype terms (34)— hover any for plain English
Aplasia/Hypoplasia of toeHP:0001991Slow-growing hairHP:0002217
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Intellectual disability-polydactyly-uncombable hair syndrome.

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No actively recruiting trials found for Intellectual disability-polydactyly-uncombable hair syndrome at this time.

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No specialists are currently listed for Intellectual disability-polydactyly-uncombable hair 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 Intellectual disability-polydactyly-uncombable hair syndrome.

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Community

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Latest news about Intellectual disability-polydactyly-uncombable hair syndrome

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Caregiver Resources

NORD 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 intellectual disability, and what support services are available?,Should my child have genetic testing, and what can it tell us about this condition?,Does my child need surgery for the extra fingers or toes, and when is the best time?,What therapies (speech, occupational, physical) do you recommend, and how often?,Are there any other health problems we should watch for with this syndrome?,What is the chance that future children could also have this condition?,Can you refer us to a specialist team experienced with this rare syndrome?

Common questions about Intellectual disability-polydactyly-uncombable hair syndrome

What is Intellectual disability-polydactyly-uncombable hair syndrome?

Intellectual disability-polydactyly-uncombable hair syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. As the name suggests, the three main features are intellectual disability (difficulty with learning and thinking skills), polydactyly (being born with extra fingers or toes), and uncombable hair (hair that is dry, frizzy, and stands away from the scalp, making it very difficult to comb flat). The hair often has a silvery or straw-like appearance. Children with this syndrome may also have distinctive facial features, such as a broad nose, a

How is Intellectual disability-polydactyly-uncombable hair syndrome inherited?

Intellectual disability-polydactyly-uncombable hair syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Intellectual disability-polydactyly-uncombable hair syndrome typically begin?

Typical onset of Intellectual disability-polydactyly-uncombable hair syndrome is neonatal. Age of onset can vary across affected individuals.