Details
Condition Overview
Arthrogryposis multiplex congenita (AMC) refers to the development of multiple joint contractures affecting two or more areas of the body prior to birth. A contracture occurs when a joint becomes permanently fixed in a bent or straightened position, which can impact the function and range of motion of the joint and may lead to muscle atrophy. AMC is not a specific diagnosis, but rather a physical symptom that can be associated with many different medical conditions. It is suspected that AMC is related to decreased fetal movement during development which can have a variety of different causes, including environmental factors (i.e. maternal illness, limited space), single gene changes (autosomal dominant, autosomal recessive, X-linked), chromosomal abnormalities and various syndromes.
Definition
- Definition: It is not a specific disease, but rather a descriptive term for a group of conditions involving joint contractures (stiffness) in two or more body areas.
Causes
- Most cases result from decreased fetal movement in the womb. This can be caused by a lack of amniotic fluid, genetic defects, or neurological/muscular issues.
Types
- Amyoplasia: The most common type, characterized by severe joint contractures and muscle weakness symmetrically across the arms and legs.
- Distal Arthrogryposis: Primarily affects the hands and feet and is often hereditary.
Symptoms
- Multiple stiff or frozen joints (shoulders, elbows, wrists, hands, hips, knees, and feet).
- Permanently straight or bent joints that resist straightening.
- Underdeveloped or thin muscles, which may give limbs a bony appearance.
- Curvature of the spine or clubfoot in some cases.
Treatment and Outlook
- Non-Progressive: AMC is not a progressive disorder, meaning the joint contractures do not worsen over time.
- Therapy: Early physical and occupational therapy is crucial to stretch joints, build muscle strength, and increase functional independence.
- Other Interventions: Bracing, casting, or surgical procedures may be used to improve limb alignment and promote mobility.