Arthrogryposis Multiplex Congenita (AMC)
Reports from the MHC data base
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 Documentation from the Ågrenska national competence centre for rare diseases.