Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Arthrogryposis, also known as Arthrogryposis Multiplex Congenita, is a rare congenital disorder that is characterized by multiple joint contractures and can include muscle weakness and fibrosis. It is a non-progressive disease. The disease derives its name from Greek, literally meaning ‘curved or hooked joints’. [1] [2] [3] [4] [5] [6] [7] [8] [7] [9] [10]
There are many known subgroups of AMC, with differing signs, symptoms, causes etc.[3]. In some cases, few joints may be affected and may have a nearly full range of motion. In the most common type of arthrogryposis, hands, wrists, elbows, shoulders, hips, feet and knees are affected. In the most severe types, nearly every joint is involved, including the jaw and back.
Frequently, the contractures are accompanied by muscle weakness, which further limits movement. AMC is typically symmetrical and involves all four extremities with some variation seen.[5][1]
Some of the different types of AMC include:
There are numerous symptoms for this group of conditions.[4] Some of the more common signs and symptoms are associated with the shoulder (internal rotation), elbow (extension and pronation), wrist (volar and ulnar), hand (fingers in fixed flexion and thumb-in-palm), hip (flexed, abducted and externally rotated, often dislocated), knee (flexion) and foot (clubfoot).[8] Complications may include scoliosis, lung hypoplasia, respiratory problems, growth retardation, midfacial hemangioma, facial and jaw variations, and abdominal hernias. Cognition and language are usually normal.[5]
The cause is unknown[7], although several mechanisms have been suggested. This includes hyperthermia of the fetus, prenatal virus, fetal vascular compromise, septum of the uterus, decreased amniotic fluid, muscle and connective tissue developmental abnormalities. [5][6] In general, the causes can be classified into extrinsic and intrinsic factors.
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