Arthrogryposis multiplex congenita (amc)

Arthrogryposis Multiplex Congenita (AMC)

Arthrogryposis is the name given to the contracture that occurs in various parts of the body before birth. Contracture is a condition where the joints of the body get permanently fixed in a straight or a bent position which restricts the movement of the joints partially or permanently. Congenital contractures can occur in one or multiple parts of the body, the latter is known as Arthrogryposis Multiplex Congenita.

Signs and Symptoms: most of the Arthrogryposis Multiplex Congenita is usually caused by defects in the central nervous system which are usually structural or functional in nature. The signs of Arthrogryposis Multiplex Congenita are usually the following: 1.

Absence of movements around the smaller or larger joints.

2.

Long tube-like limbs which have a soft and doughy feeling.

3.

Clubbed foot or soft tissue webbing around the affected joints.

4.

A groove across the top of the mouth due to the absence of joining of the roof of the mouth.

5.

Long and abnormally slender bones of the limbs.

Although these are the common syndromes of Arthrogryposis Multiplex Congenita, there might be individual specific syndromes which depend on the severity of the disease in that particular individual. There are majorly two types of Arthrogryposis Multiplex Congenita, Amyoplasia and Distal Arthrogryposis which is usually caused by a group of genetic disorders.

Amyoplasia: Amyoplasia is usually one of the most popular forms of Arthrogryposis Multiplex Congenita where the multiple joints are affected along with inward curving of the shoulders extended elbows and flexed wrists. The most affected joints are usually the hips and shoulders. It is also featured by the presence of severely clubbed foot along with birth marks. This disease is usually sporadic and may not appear in any other family members.

Causes: The main causes of Arthrogryposis Multiplex Congenita has not yet been confirmed and it is said that in most cases it is usually caused by neural or muscular lack of movement in the fetus. Connective tissue problem, maternal illness or limited utero space are also thought to be the problems which leads to Arthrogryposis Multiplex Congenita.

Population: Arthrogryposis Multiplex Congenita is usually caused in both male and female population equally where 1 out of 3000 babies are affected by the disease. It majorly occurs in European, Asian and African populations.

Diagnosis: The primary diagnosis of Arthrogryposis Multiplex Congenita is done by observing the physical abnormalities in the child which is then tested to find the underlying cause of the disease.

Electromyography and imaging techniques are the most common methods of testing the underlying cause.

Standard Treatments: Arthrogryposis Multiplex Congenita is usually treated with physiotherapy which helps in increasing the movement of the joints that have been affected. In certain cases, surgery might be required in order to initiate the movement of the joints. Genetic therapy may be needed to benefit the individuals. Symptom specific treatments are also available.

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