OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome

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Overview

Intellectual disability-hypotonia-skin hyperpigmentation syndrome is an extremely rare genetic condition that has been described in only a very small number of individuals in the medical literature. The term 'OBSOLETE' in its name indicates that this diagnosis may have been reclassified or merged with another condition as medical understanding has advanced. The syndrome was originally characterized by three main features: intellectual disability (difficulty with learning, reasoning, and adaptive skills), hypotonia (low muscle tone, meaning muscles feel floppy or less firm than expected), and skin hyperpigmentation (patches of skin that are darker than surrounding areas). Children with this condition typically showed developmental delays from early in life, including delays in reaching motor milestones like sitting and walking, as well as delays in speech and language development. Because this condition is so rare and has been marked as obsolete, the exact genetic cause may not be fully established, and it is possible that affected individuals today would receive a more specific or updated diagnosis based on modern genetic testing. Treatment has generally been supportive, focusing on therapies to help with development and managing individual symptoms. There are no specific curative treatments known for this condition.

Also known as:

Key symptoms:

Intellectual disability or learning difficultiesLow muscle tone (floppy muscles)Dark patches on the skinDelayed motor milestones such as sitting and walkingSpeech and language delaysPoor coordinationFeeding difficulties in infancyGeneralized weakness

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 OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome.

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No actively recruiting trials found for OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome at this time.

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No specialists are currently listed for OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation 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 OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome.

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Community

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Latest news about OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome

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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.Is this diagnosis still considered current, or should we pursue updated genetic testing for a more specific diagnosis?,What type of genetic testing would you recommend for our family?,What therapies are most important to start right away?,How will my child's muscle tone and development likely change over time?,Are there any clinical trials or research studies we should know about?,What educational supports should we request for our child?,Should other family members be tested for this condition?

Common questions about OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome

What is OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome?

Intellectual disability-hypotonia-skin hyperpigmentation syndrome is an extremely rare genetic condition that has been described in only a very small number of individuals in the medical literature. The term 'OBSOLETE' in its name indicates that this diagnosis may have been reclassified or merged with another condition as medical understanding has advanced. The syndrome was originally characterized by three main features: intellectual disability (difficulty with learning, reasoning, and adaptive skills), hypotonia (low muscle tone, meaning muscles feel floppy or less firm than expected), and s

At what age does OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome typically begin?

Typical onset of OBSOLETE: Intellectual disability-hypotonia-skin hyperpigmentation syndrome is infantile. Age of onset can vary across affected individuals.