Arthrogryposis Multiplex Congenita (AMC)

The following report from the MHC-database is based on the latest version of the Mun-H-Center Observation Chart that has been in use since 2008. Please note, that this report exclude children younger than 3 years.

Reports based on the original forms (1996-2008) are presented at the bottom of this page - below the description of the diagnosis

ICD-10
Q74.3

1:4 500 live births.

There are more than 150 different diseases within this diagnosis and the genetic cause varies. The etiology may be classified as:

• Muscular causes (various congenital muscular diseases).
• Neurogenic causes (deformities of the central nervous system, loss of motor neurons in the spinal marrow, loss of peripheral nerves).
• Connective tissue diseases.
• Reduced mobility of the fetus owing to insufficient space in the uterus (lack of amniotic fluid, twin pregnancy, heart-shaped uterus).
• Maternal illness during pregnancy.

Stiffness in several joints is the common denominator. The disease is not progressive; on the contrary treatment may result in improvement. It is common for these children to be born with one dislocated hip and a club foot.

The jaw-joints may also be affected, which results in a restricted ability to open the jaws. An overjet and open bite in the region of the front teeth, a narrow, high palate and a small lower jaw all occur. Many individuals have restricted oral motor skills, which affects their ability to suck, chew, swallow, and speak. Drooling and teeth grinding occur frequently.

• It is important that individuals with this diagnosis receive dental preventive treatment, with extra prophylactic care and information on oral hygiene from an early age.
• When the jaws do not open properly, the function of the jaw joint should be investigated, and appropriate treatment thereafter prescribed (such as jaw-stretching exercises or surgery).
• Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
• Teeth grinding should be followed up, and be managed with a splint when necessary.
• Training in oral motor skills and extra stimulation in cases of eating disorders, speech difficulties and drooling.

The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
The Newsletter of the Ågrenska Center.

Report from Questionnaires
Report from Observation Chart