arthrogryposis multiplex congenita


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ar·thro·gry·po·sis mul·'ti·plex con·gen·'i·ta

[MIM*108110]
limitation of range of joint motion and contractures present at birth, usually involving multiple joints; a syndrome probably of diverse etiology that may result from changes in spinal cord, muscle, or connective tissue. Several forms exist, autosomal dominant [MIM*108110, 108120, 108130, 108140, 108145, 108200], recessive [MIM*208080, 208081, 208085, 208100, 208150, 208155, 208200], and X-linked [MIM*301830]

arthrogryposis multiplex congenita

Etymology: Gk, arthron + gryposis, joint curve; L, multus, many, plica, fold, congenitus, born with
fibrous stiffness of one or more joints, present at birth. It is often associated with incomplete development of the muscles that move the involved joints and degenerative changes of the motor neurons that innervate those muscles. The cause of the condition, which is uncommon, is unknown, although possible causes are fetal crowding and maternal neuromuscular disease. Physiotherapy to loosen the joints is the only treatment. Also called amyoplasia congenita [əmī′ōplā′zhə] .

arthrogryposis multiplex congenita

A rare sporadic condition characterised by joint contractures, dislocations, rigid skeletal deformities (e.g., clubfoot or talipes equinovarus), skin atrophy and replacement of limb muscles with fibrous tissue. AMC is not a sui generis disease, but rather a descriptive term that signifies multiple congenital contractures. The aetiologies encompass both neurogenic and primary myopathic diseases, but most cases are not due to neuromuscular disease.
 
Pathogenesis
Uncertain; a common link may be intrauterine movement during a critical period of limb development.
 
Management
Arthrodesis.

ar·thro·gry·po·sis mul·ti·plex con·gen·i·ta

(ahr'thrō-gri-pō'sis mŭl'ti-pleks kon-jen'i-tă)
Limitation of range of joint motion and contractures present at birth, usually involving multiple joints; a syndrome probably of diverse etiology that may result from changes in spinal cord, muscle, or connective tissue.

arthrogryposis multiplex congenita

congenital limb defect characterized by joint and skin contractures and marked joint movement limitation; a cause of talipes equinovarus

arthrogryposis

1. persistent flexion of a joint.
2. tetanoid spasm.

congenital arthrogryposis with dysraphism
arthrogryposis with delayed or arrested closure of the neural tube. Called also arthrogryposis multiplex congenita. See also complex vertebral malformation.
arthrogryposis and hydranencephaly
see akabane virus disease.
inherited arthrogryposis
occurs in catle, pigs and sheep. In cattle it is commonly associated with cleft palate, sometimes with other skeletal defects and also prolonged gestation.
lupine-induced arthrogryposis
occurs in calves whose dams have ingested Lupinus spp. that contain the teratogenic alkaloids anagyrine and/or ammodendrine between 35 and 100 days gestation. These alkaloids impair the natural active movement of the developing fetus so that it grows in a static state resulting in deformities of the limbs. Many western lupine species, bitter lupines, contain these teratogenic alkaloids but they are usually not palatable and not eaten. The alkaloid conine in Conium maculatum can also produce this syndrome.
arthrogryposis multiplex congenita
see congenital arthrogryposis with dysraphism (above).
References in periodicals archive ?
Describe the diseases and disorders of oral development in the case of a ten year old boy with arthrogryposis multiplex congenita (AMC).
Deborah who is 20 and has arthrogryposis multiplex congenita (AMC).
1) It is well known that, infants of diabetic mother (IDM) are at an increased risk of morbidity and mortality related to the respiratory distress, growth abnormalities, hyperviscosity, hypoglycemia and congenital malformations like arthrogryposis multiplex congenita along with central nervous system, renal, cardiovascular, and gastrointestinal system anomalies.
Born with arthrogryposis multiplex congenita, Lee has reduced joint mobility and despite 15 operations he still cannot move his ankles or knees, forcing him to control his horse from his hips.
Michael, 50, was born with Arthrogryposis Multiplex Congenita, affecting his major joints.
2] The term is synonymously used with Distal Arthrogryposis Type IIA, Camptodactyly-Club foot-Cleft Palate, Arthrogryposis Multiplex Congenita Distal type IIA.
The radiological differential diagnosis include osteopoikilosis, osteopetrosis, arthrogryposis multiplex congenita, and osteopathia striata (11).
Lee has arthrogryposis multiplex congenita, a joint condition, and collected a CBE in 2009.

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