Arthrogryposis, from which the Minister of Solidarity Damien Abad suffers, is a rare congenital neuromuscular disease which results in joint deformities and stiffness, limiting the range of motion.
The term “arthrogryposis” comes from the Greek and means twisted joints.
Different joints can be affected: the hand, the foot, the knee, the wrist… We speak of arthrogryposis multiplex congenita (AMC) when at least two different types of joints are involved.
The prevalence of this disease is between one birth in 3,000 and one birth in 12,000, according to the High Health Authority.
AMCs are always linked to reduced fetal movements and abnormal fetal positioning, leading to joint abnormalities that can reach the upper and lower extremities.
The precocity and the importance of the decline in fetal movements during pregnancy determine the severity of the manifestations at birth, explains the High Authority for Health.
They are particularly associated with severe hypotonia at birth (lack of tone), clubfoot on both sides, delayed motor development, lack of reflexes, etc.
There are two main types of congenital arthrogryposis multiplex. Amyoplasia (classic arthrogryposis) is manifested by multiple retractions reaching the limbs symmetrically. With distal arthrogryposis, the hands and feet are affected but the large joints are usually spared.
According to the Alliance Arthrogryposis association, the disease can result in a wide range of damage, with consequences ranging from mild to severe. Some people will be able to lead an independent life, others will be dependent on technical or human aids.
Motor disability is thus very different from one person to another: some can carry out the gestures of daily life, others are in a wheelchair, others only move their heads.
The vast majority of arthrogryposis is not accompanied by cognitive or behavioral disability.
At birth, once the diagnosis has been made, physiotherapy and possibly surgery can significantly recover some of the congenital malformations, explains the association.
After two years, neuromuscular development slows down and treatment gains are less visible. The approach then turns more towards compensating for the handicap to maximize comfort and autonomy, through functional rehabilitation, occupational therapy, equipment.
“The absence of drug or surgical treatment and the absence of regression mean that arthrogryposis becomes more of a stable handicap than a disease”, underlines the Alliance Arthrogryposis association.
24/05/2022 14:57:51 – Paris (AFP) – © 2022 AFP
