Infantile spasms-broad thumbs syndrome

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ORPHA:3173G40.4
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Overview

Infantile spasms-broad thumbs syndrome, also known as the syndrome of infantile spasms with broad thumbs, is an extremely rare genetic condition that combines a specific type of seizure disorder with distinctive physical features. The hallmark features include infantile spasms (a type of epileptic seizure that typically begins in the first year of life), unusually broad or wide thumbs, and intellectual disability. Infantile spasms are a serious form of epilepsy in babies, where the child experiences sudden, brief muscle contractions that often occur in clusters, particularly around waking. These spasms can significantly affect brain development if not treated promptly. Children with this syndrome may also have other physical differences, including distinctive facial features and developmental delays. The intellectual disability can range from mild to severe. Because this condition is so rare, with only a handful of cases described in the medical literature, the full range of symptoms and the best treatment approaches are still being understood. Treatment generally focuses on controlling the seizures as early as possible using anti-epileptic medications, along with supportive therapies such as physical therapy, occupational therapy, and special education services to help the child reach their full potential. Early intervention is key to improving outcomes.

Also known as:

Key symptoms:

Infantile spasms (sudden jerking movements in babies)Broad or wide thumbsIntellectual disabilityDevelopmental delaysSeizuresDistinctive facial featuresDelayed speech and languageDelayed motor milestones such as sitting and walkingAbnormal brain wave patterns on EEG (hypsarrhythmia)Low muscle toneLearning difficulties

Clinical phenotype terms (15)— hover any for plain English
Abnormal cranial suture/fontanelle morphologyHP:0000235Vaginal herniaHP:0100672
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

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 Infantile spasms-broad thumbs syndrome.

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No actively recruiting trials found for Infantile spasms-broad thumbs syndrome at this time.

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No specialists are currently listed for Infantile spasms-broad thumbs 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 Infantile spasms-broad thumbs syndrome.

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Community

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Caregiver Resources

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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 seizures does my child have, and what does the EEG show?,What treatment options are available, and what are the potential side effects?,Should my child have genetic testing, and what might the results tell us?,What developmental therapies should we start, and how soon?,What should I do if my child has a prolonged seizure at home?,How often will my child need follow-up EEGs and neurology visits?,Are there any clinical trials or research studies my child might be eligible for?

Common questions about Infantile spasms-broad thumbs syndrome

What is Infantile spasms-broad thumbs syndrome?

Infantile spasms-broad thumbs syndrome, also known as the syndrome of infantile spasms with broad thumbs, is an extremely rare genetic condition that combines a specific type of seizure disorder with distinctive physical features. The hallmark features include infantile spasms (a type of epileptic seizure that typically begins in the first year of life), unusually broad or wide thumbs, and intellectual disability. Infantile spasms are a serious form of epilepsy in babies, where the child experiences sudden, brief muscle contractions that often occur in clusters, particularly around waking. The

At what age does Infantile spasms-broad thumbs syndrome typically begin?

Typical onset of Infantile spasms-broad thumbs syndrome is infantile. Age of onset can vary across affected individuals.