Timothy syndrome

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ORPHA:65283OMIM:618447I49.8
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15Specialists8Treatment centers

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

Timothy syndrome is a very rare genetic condition that affects the heart and several other parts of the body. It is sometimes called LQT8 (Long QT syndrome type 8) because one of its main features is a problem with the heart's electrical system called long QT syndrome. This means the heart takes longer than normal to recharge between beats, which can lead to dangerous irregular heartbeats (arrhythmias) and even sudden cardiac arrest. The condition is caused by a change (mutation) in a gene called CACNA1C, which controls a channel in heart and other cells that lets calcium flow in and out. When this channel does not work properly, it affects not just the heart but also the brain, immune system, fingers and toes, and facial features. Many children with Timothy syndrome also have autism spectrum disorder or other developmental delays, and some are born with webbed fingers or toes (a feature called syndactyly). There are two forms: Classic Timothy syndrome (Type 1) and a more severe form (Type 2). Symptoms often appear at birth or in early infancy. Treatment focuses on managing the heart rhythm problems using medications, implanted devices like pacemakers or defibrillators, and close monitoring. There is currently no cure, but careful heart management can help improve quality of life and reduce the risk of life-threatening events.

Also known as:

Key symptoms:

Dangerously irregular heartbeat (arrhythmia)Long QT interval on heart tracing (ECG)Webbed fingers or toes (syndactyly)Autism spectrum disorder or social communication difficultiesIntellectual disability or developmental delaysLow blood sugar (hypoglycemia)Weak immune system leading to frequent infectionsDistinctive facial features such as a flat nasal bridge and low-set earsSeizuresSudden fainting or loss of consciousnessHeart defects present at birth (congenital heart defects)Slow heart rate (bradycardia)

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Oct 2024A Causal Role for Voltage-gated Cav1.2 Calcium Channels in Mediating 5G FR1 Effects on Sleep-associated Brain Health in Humans

Hans-Peter Landolt — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Timothy syndrome.

View clinical trials →

No actively recruiting trials found for Timothy syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Timothy syndrome community →

Specialists

15 foundView all specialists →
KT
Katherine W Timothy
Specialist
5 Timothy syndrome publications
AG
Andy Golden
Specialist
5 Timothy syndrome publications
RB
Rosemary Bauer
PLYMOUTH MEETING, PA
Specialist
4 Timothy syndrome publications
GP
Geoffrey S Pitt
NEW YORK, NY
Specialist
4 Timothy syndrome publications
ID
Ivy E Dick
Specialist
3 Timothy syndrome publications
FB
Fikri Birey
Specialist
2 Timothy syndrome publications
ML
Min-Yin Li
DENTON, TX
Specialist
2 Timothy syndrome publications
OR
Omer Revah
Specialist
2 Timothy syndrome publications
KL
Kerry A Larkin
BROAD CHANNEL, NY
Specialist
2 Timothy syndrome publications
EW
Edward P Walsh
Specialist
2 Timothy syndrome publications
DA
Dominic J Abrams
BOSTON, MA
Specialist
2 Timothy syndrome publications
CC
Cecilia Gonzalez Corcia
Specialist
2 Timothy syndrome publications
AH
Alexis Hermida
LOS ANGELES, CA
Specialist
2 Timothy syndrome publications
JH
Jean-Sylvain Hermida
Specialist
2 Timothy syndrome publications
AV
Alexandra Valsamakis
BALTIMORE, MD
Specialist
2 Timothy syndrome publications

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 Timothy syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Timothy syndrome

Disease timeline:

New recruiting trial: A Causal Role for Voltage-gated Cav1.2 Calcium Channels in Mediating 5G FR1 Effects on Sleep-associated Brain Health in Humans

A new clinical trial is recruiting patients for Timothy syndrome

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.Does my child need an implantable defibrillator (ICD), and what are the risks and benefits?,Which medications are best for managing the heart rhythm, and what side effects should I watch for?,What physical activities are safe, and which ones should my child avoid?,Should other family members be tested for CACNA1C mutations?,What developmental or behavioral therapies do you recommend, and how do we access them?,What should I do if my child faints or has a seizure at home or at school?,Are there any clinical trials or research studies we should consider joining?

Common questions about Timothy syndrome

What is Timothy syndrome?

Timothy syndrome is a very rare genetic condition that affects the heart and several other parts of the body. It is sometimes called LQT8 (Long QT syndrome type 8) because one of its main features is a problem with the heart's electrical system called long QT syndrome. This means the heart takes longer than normal to recharge between beats, which can lead to dangerous irregular heartbeats (arrhythmias) and even sudden cardiac arrest. The condition is caused by a change (mutation) in a gene called CACNA1C, which controls a channel in heart and other cells that lets calcium flow in and out. Whe

How is Timothy syndrome inherited?

Timothy syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Timothy syndrome typically begin?

Typical onset of Timothy syndrome is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Timothy syndrome?

15 specialists and care centers treating Timothy syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.