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 ?
Arthrogryposis Multiplex Congenita (AMC) is a disorder with multiple aetiological factors.
The medical terminology for the disorder is Arthrogryposis Multiplex Congenita.
Ian was born with Arthrogryposis Multiplex Congenita, which mean his joints are permanently fixed.
Oral pathologies and alterations in a 10 year old boy with arthrogryposis multiplex congenita (AMC).
Beals Hecht syndrome, also known as congenital contractural arachnodactyly, is a member of arthrogryposis multiplex congenita family.
Contributors working in human performance and rehabilitation, sport and exercise, physiology, and other fields around the world discuss human motor development in children with and without disabilities; developmental coordination disorder; motor skill development in able-bodied and impaired swimmers; assessment of skills; impairments due to stroke; motor learning; the performance of professional golfers and tennis players; oromotor impairment in cerebral palsy; arthrogryposis multiplex congenita; comparison of the skills of chimpanzees and humans; traditional mirror therapy for movement and postural control problems; muscle strength testing in children; children with autism; and other topics.
Arthrogryposis Multiplex Congenita (AMC) is characterized by multiple, congenital, non progressive joint contractures.
Contributors working in obstetrics and gynecology, genetics, pediatrics, and other medical and research fields discuss topics such as the diagnosis of Down Syndrome; the connection between hypertensive dysfunction in pregnancy and the development of cardiovascular diseases; the techniques and applications of MRI in fetal imaging; prenatal genetic diagnosis; the relation between antithrombin congenital deficiency and high risk miscarriages and recurrent abortions; arthrogryposis multiplex congenita; and primary prevention efforts and risk factors such as age and sociodemographic factors, obstetric history, maternal medical conditions, genetic disorders, psychosocial issues, and infections and vaccination.
Lastly, although more common conditions such as cerebral palsy, rheumatic disorders, Down syndrome and sports injuries are covered in this book (along with traumatic brain injury, shaken baby syndrome, arthrogryposis multiplex congenita, asthma, autism, burns and limb deficiencies), unfortunately it does not include conditions that are prevalent in a physiotherapist's case load, such as the consequences of premature birth, or cystic fibrosis and bronchiectasis.
Mills-Testi was born with a disability called arthrogryposis multiplex congenita, which means she cannot use her arms or hands and has limited use of her legs and feet.
A case report of arthrogryposis multiplex congenita in monochorionic biamniotic twins

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