Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:363686OMIM:615074Q87.8
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome is an extremely rare genetic condition that affects brain development and physical features. The name describes its main characteristics: children with this condition have significant intellectual disability, meaning they have serious difficulties with learning, thinking, and problem-solving. Their ability to develop speech and language is very limited. They also have strabismus, which means their eyes do not line up properly (sometimes called crossed eyes or squint). Their facial expressions may appear unusual, often described as a grimacing appearance. Their fingers tend to be noticeably long. Because this syndrome affects multiple body systems — the brain, eyes, face, and hands — it is classified as a multi-system developmental disorder. This condition is so rare that very few cases have been described in the medical literature, which means knowledge about it is still growing. There is currently no cure for this syndrome. Treatment focuses on managing individual symptoms and providing supportive therapies such as speech therapy, physical therapy, occupational therapy, and special education services. Eye care, including possible surgery for strabismus, may also be needed. Early intervention programs can help children reach their best potential, even though challenges will remain throughout life.

Key symptoms:

Severe intellectual disabilityVery limited or absent speech and languageCrossed eyes or misaligned eyes (strabismus)Unusual grimacing facial expressionsAbnormally long fingersDelayed developmental milestonesDifficulty with fine motor skillsBehavioral challengesDifficulty with self-care and daily activitiesPossible feeding difficulties in early life

Clinical phenotype terms (48)— hover any for plain English
Facial grimacingHP:0000273Hyperopic astigmatismHP:0000484Generalized non-motor (absence) seizureHP:0002121Long fingersHP:0100807
Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome.

View clinical trials →

No actively recruiting trials found for Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome community →

No specialists are currently listed for Severe intellectual disability-poor language-strabismus-grimacing face-long fingers 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 Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndromeForum →

No community posts yet. Be the first to share your experience with Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome.

Start the conversation →

Latest news about Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome

No recent news articles for Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome.

Follow this condition to be notified when news becomes available.

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 genetic tests should be done to confirm the diagnosis and look for the underlying cause?,What therapies are most important to start right away for my child's development?,How should we manage the eye misalignment, and will surgery be needed?,Are there any signs of seizures or other neurological concerns we should watch for?,What educational and support services are available for my child?,Is genetic counseling recommended for our family regarding future pregnancies?,Are there any research studies or clinical trials we could participate in?

Common questions about Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome

What is Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome?

Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome is an extremely rare genetic condition that affects brain development and physical features. The name describes its main characteristics: children with this condition have significant intellectual disability, meaning they have serious difficulties with learning, thinking, and problem-solving. Their ability to develop speech and language is very limited. They also have strabismus, which means their eyes do not line up properly (sometimes called crossed eyes or squint). Their facial expressions may appea

At what age does Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome typically begin?

Typical onset of Severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome is infantile. Age of onset can vary across affected individuals.