Carney complex-trismus-pseudocamptodactyly syndrome

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ORPHA:319340OMIM:608837Q68.8
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Overview

Carney complex-trismus-pseudocamptodactyly syndrome is an extremely rare genetic condition that combines features of two distinct disorders: Carney complex and trismus-pseudocamptodactyly syndrome. Carney complex is a condition that causes multiple tumors and abnormal skin pigmentation (such as dark spots or freckling), along with overactivity of certain hormone-producing glands. It can lead to heart tumors called myxomas, skin myxomas, and tumors of the adrenal glands, pituitary gland, or other endocrine organs. Trismus-pseudocamptodactyly syndrome involves limited ability to open the mouth fully (trismus) and an unusual hand posture where the fingers appear bent when the wrist is flexed, though they can straighten when the wrist is relaxed (pseudocamptodactyly). This combined syndrome is caused by mutations in the MYH8 gene, which provides instructions for making a muscle protein. Patients may experience a wide range of symptoms affecting the heart, skin, endocrine system, and musculoskeletal system. Because this condition is so rare, treatment is tailored to each patient's specific symptoms and may include surgery for tumors, hormone management, and physical therapy for joint and muscle problems. Close monitoring by a team of specialists is essential for managing the various aspects of this syndrome.

Also known as:

Key symptoms:

Limited ability to open the mouth fully (trismus)Fingers that appear bent when the wrist is flexed (pseudocamptodactyly)Dark spots or freckling on the skinHeart tumors (cardiac myxomas)Skin bumps or lumps (skin myxomas)Overactive hormone glands (endocrine overactivity)Short statureMuscle stiffness or tightnessDifficulty chewing or eating due to limited jaw openingAbnormal hand postureAdrenal gland tumorsPituitary gland abnormalitiesJoint stiffness or limited range of motion

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Carney complex-trismus-pseudocamptodactyly syndrome.

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No actively recruiting trials found for Carney complex-trismus-pseudocamptodactyly syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Carney complex-trismus-pseudocamptodactyly syndrome community →

No specialists are currently listed for Carney complex-trismus-pseudocamptodactyly 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 Carney complex-trismus-pseudocamptodactyly syndrome.

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Community

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Latest news about Carney complex-trismus-pseudocamptodactyly syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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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.How often should my heart be checked with echocardiography?,What endocrine tests should be done regularly, and how often?,Are there specific physical therapy exercises that can help with jaw opening and hand function?,What emergency signs should I watch for that would require immediate medical attention?,Should other family members be tested for this genetic condition?,Are there any clinical trials or research studies I could participate in?,What specialists should be part of my ongoing care team?

Common questions about Carney complex-trismus-pseudocamptodactyly syndrome

What is Carney complex-trismus-pseudocamptodactyly syndrome?

Carney complex-trismus-pseudocamptodactyly syndrome is an extremely rare genetic condition that combines features of two distinct disorders: Carney complex and trismus-pseudocamptodactyly syndrome. Carney complex is a condition that causes multiple tumors and abnormal skin pigmentation (such as dark spots or freckling), along with overactivity of certain hormone-producing glands. It can lead to heart tumors called myxomas, skin myxomas, and tumors of the adrenal glands, pituitary gland, or other endocrine organs. Trismus-pseudocamptodactyly syndrome involves limited ability to open the mouth f

How is Carney complex-trismus-pseudocamptodactyly syndrome inherited?

Carney complex-trismus-pseudocamptodactyly syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.