arthrogryposis


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arthrogryposis

 [ahr″thro-grĭ-po´sis]
1. persistent flexion of a joint.
2. tetanoid spasm.

ar·thro·gry·po·sis

(ar'thrō-gri-pō'sis),
Congenital defect of the limbs characterized by severe contractures of multiple joints.
[arthro- + G. gryphōsis, a crooking]

arthrogryposis

(är′thrə-grə-pō′sĭs)
n. pl. arthrogrypo·ses (-sēz)
The permanent fixation of a joint in a contracted position.

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

(ahr'thrō-gri-pō'sis)
Congenital defect of the limbs characterized by contractures of multiple joints.
[arthro- + G.gryphōsis, a crooking]

arthrogryposis

Congenital fixation of the limb joints, usually in an extended position. The condition is probably due to a destructive disorder of the motor nerves to the muscles around the affected joints during the pregnancy.
References in periodicals archive ?
External anomalies of the extremities: rocker bottom feet, scissor legs, clenched and hyperflexed hands, and multiple contractures (arthrogryposis).
Arthrogryposis involving the hips, knees, ankles, and elbows was observed, along with bilateral talipes equinovarus.
It is successfully used in the treatment of non-idiopathic clubfeet as well - such as in arthrogryposis, myelomeningocele, untreated and later treated children, complex and resistant feet; recurrences, etc.
Caption: FINDING HIS GROOVE: David Segal is a professional drummer who was born with arthrogryposis, which made his hands and feet clubbed.
Distal arthrogryposis type 1: clinical analysis of a large kindred.
Multiple malformations were present, including hypertelorism, flat midface, low nasal bridge, short nose, severe arthrogryposis with upper and lower malformations of the limbs and joint contractures, craniosynostosis, microcephaly, and cryptorchidism.
SMA with pontocerebellar hypoplasia (SMAPCH1/PCH1), SMA with progressive myoclonic epilepsy (SMAPME), congenital SMA with arthrogryposis and fractures, and SMA caused by mitochondrial disorders display predominant proximal weakness.
First reported in 1938 by Freeman and Sheldon who initially called it craniocarpotarsal dystrophy [1], Freeman-Sheldon syndrome (FSS; OMIM #193700), also known as craniocarpal-tarsal dysplasia, windmill vane hand syndrome, distal arthrogryposis type 2A, and whistling-face syndrome, is a rare form of multiple congenital contracture syndrome.
Three infants had hip dysplasia, including one infant with arthrogryposis who had bilateral dislocated hips.
The 28-year-old world champion was born with arthrogryposis, a rare congenital disorder that affected all four of his limbs.
The second case was a pediatric patient diagnosed with congenital arthrogryposis multiplex.
Arthrogryposis Multiplex Congenita (AMC) is an uncommon, but easily recognizable syndrome of musculoskeletal system.

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