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]
Farlex Partner Medical Dictionary © Farlex 2012

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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

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.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
References in periodicals archive ?
Diagnosing arthrogryposis multiplex congenita: a review.
Anaesthesia recommendations in patients suffering from arthrogryposis multiplex congenita. Orpha.net.
Figure 1 and 2: Arthrogryposis multiplex congenita with omphalocele minor, Bladder exstrophy and inguinal hernia
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.
Nadine said: "Cal has arthrogryposis multiplex congenita. His legs aren't in the correct position, he has a club left foot and his muscles are shrinking.

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