What Is Trismus-Pseudocamptodactyly Syndrome?
Trismus-pseudocamptodactyly syndrome (TPS) is a rare muscle disorder that affects the mouth, hands, and feet. The syndrome is also known as Dutch-Kennedy syndrome and Hecht syndrome.
TPS occurs as a result of a mutation of the MYH8 gene. It causes shortened muscles and tendons. The most
common complication caused by TPS is an inability to open the mouth fully. The short tendons can also result in the curving of the fingers and sometimes the feet as well.
What Causes Trismus-Pseudocamptodactyly Syndrome?
TPS is an inherited disease, categorized as autosomal dominant. This means that a person can inherit the
abnormal gene from only one parent. The disorder is rare, so there are no distinguishing factors for a person’s likelihood to develop TPS, other than a family history of the disorder.
What Are the Symptoms of Trismus-Pseudocamptodactyly Syndrome?
The symptoms of TPS vary from person to person. The most common symptom is limited mobility of the mouth,
which can cause problems with chewing. Other complications can include:
- limited motion of the arms and/or legs
- clinched fists
- club foot
- abnormalities with the feet and hands
How Is Trismus-Pseudocamptodactyly Syndrome Diagnosed?
A doctor can usually diagnose a case of TPS at birth. It requires a full physical examination, taking into account the family’s medical history, as TPS is an inherited syndrome. The signs of TPS begin to show during infancy.
How Is Trismus-Pseudocamptodactyly Syndrome Treated?
There is no cure for TPS. However, patients can undergo surgery to alleviate some of the symptoms of TPS.
Doctors also often suggest physical and/or occupational therapy for people with TPS who have trouble walking or who have dexterity issues.