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Arthrogryposis multiplex congenita (AMC)

Name :
Congenital, multiple joint contractures.

Cause:
Prenatal development disorder that takes place around the 8th to 11th week of pregnancy and is characterised by a neurological dysgenesis.

Effects:

Owing to the dysgenesis the development of muscles is impaired preventing the joints from forming so that they can fulfill their functions. This generally causes a significant reduction of the range of movements of the joints affected in the flexed and extended positions.

Depending on the degree of severity AMC is divided into 3 types.

Type 1:
In this clinical picture the extremities are affected exclusively. We divide this group into subgroups. Mainly it is the hands and feet that are affected without or with insignificant affliction of the sections of the extremities close to the trunk, also of the entire extremities including the shoulder and hip joints. These patients have no further disorders or malformations. The mental and sensory development is normal.

Type 2:
Type 1 changes are present: In addition to this there are also malformations of various organs (for example the abdominal wall, bladder, head, spinal column).
Various known subgroups.

Type 3:
In addition to the stiff joints these children also have more or less marked malformations of the spinal column and of the central nervous system and can also mean very severe malformations.


Consequences:
The impairment of the musculoskeletal system ranges from individual joints on arms and legs to almost all joints, including the spinal column. As a rule the joints show a conspicuous myasthenia above all of the wrists and ankles. Mobility and hence strength impairment varies from one person to another.

Supplying a car:
Joint stiffness has a direct effect on mobility and strength of the arms and legs. This has to be compensated by technical means. Owing to the fact that the joints stiffen in a bent position the reach of the arms and legs must be expected to be reduced and this has to be taken into account for the steering equipment and for the gas and brake pedal control. 

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