Skeletal dysplasia-epilepsy-short stature syndrome

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ORPHA:1858OMIM:601187Q87.5
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Overview

Skeletal dysplasia-epilepsy-short stature syndrome is an extremely rare genetic condition that affects multiple body systems, primarily the bones, brain, and growth. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:1858). People with this condition typically have abnormal bone development (skeletal dysplasia), which can cause unusual bone shapes and joint problems. They also experience seizures (epilepsy) that may begin in childhood, along with significantly shorter height than expected for their age and family. Some individuals may also have intellectual disability or developmental delays. Because this syndrome is so rare, only a handful of cases have been described in the medical literature. The exact genetic cause has not been fully established, and much of what is known comes from individual case reports. Diagnosis is usually based on the combination of clinical features — skeletal abnormalities seen on X-rays, a history of seizures, and short stature — along with genetic evaluation to rule out other conditions. There is currently no cure for this syndrome. Treatment focuses on managing each symptom individually. Anti-seizure medications are used to control epilepsy, orthopedic care may be needed for bone and joint problems, and growth monitoring is important throughout childhood. A team of specialists working together provides the best care for affected individuals.

Also known as:

Key symptoms:

Short stature or growth failureSeizures or epilepsyAbnormal bone developmentUnusual bone shapes visible on X-raysJoint stiffness or limited joint movementDelayed development or intellectual disabilityAbnormal curvature of the spineShort limbs relative to the bodyDelayed motor milestones such as walkingFacial features that may appear different from family members

Clinical phenotype terms (15)— hover any for plain English
Hypoplastic iliac wingHP:0002866
Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Skeletal dysplasia-epilepsy-short stature syndrome.

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No actively recruiting trials found for Skeletal dysplasia-epilepsy-short stature syndrome at this time.

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No specialists are currently listed for Skeletal dysplasia-epilepsy-short stature 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 Skeletal dysplasia-epilepsy-short stature syndrome.

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Community

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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.What type of seizures does my child have, and what is the best medication to control them?,Are there any skeletal problems that need orthopedic treatment now or in the future?,Should we pursue genetic testing, and what might the results tell us?,Is growth hormone therapy an option for my child's short stature?,What developmental therapies or educational supports do you recommend?,How often should we schedule follow-up visits with each specialist?,Are there any clinical trials or research studies we could participate in?

Common questions about Skeletal dysplasia-epilepsy-short stature syndrome

What is Skeletal dysplasia-epilepsy-short stature syndrome?

Skeletal dysplasia-epilepsy-short stature syndrome is an extremely rare genetic condition that affects multiple body systems, primarily the bones, brain, and growth. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:1858). People with this condition typically have abnormal bone development (skeletal dysplasia), which can cause unusual bone shapes and joint problems. They also experience seizures (epilepsy) that may begin in childhood, along with significantly shorter height than expected for their age and family. Some individuals may also have intellectual disability or

At what age does Skeletal dysplasia-epilepsy-short stature syndrome typically begin?

Typical onset of Skeletal dysplasia-epilepsy-short stature syndrome is childhood. Age of onset can vary across affected individuals.