arthrogryposis multiplex congenita

(redirected from arthrogryposis syndrome)

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 classic literature ?
The queen was bound to hang him for killing her kinsman, but I would not allow it: it was no crime to kill an assassin.
Then he added mournfully, "But I wish I could have been saved the shame of meeting an assassin on the field on honor.
Earnshaw accomplished this ere his guest reached the front; he then came and brought his chair to the other side of my table, leaning over it, and searching in my eyes for a sympathy with the burning hate that gleamed from his: as he both looked and felt like an assassin, he couldn't exactly find that; but he discovered enough to encourage him to speak.
Silent, humane, indispensable in hospital and prison, using his art equally among assassins and victims, he was a man apart.
I had the conscience of an assassin, and was haunted by a vague sense of enormous wickedness.
Strike," he added to an executioner who was by, "and rid me of this assassin.
And you, secretary, answer my lord the duke and tell him that all his commands shall be obeyed to the letter, as he directs; and say from me to my lady the duchess that I kiss her hands, and that I beg of her not to forget to send my letter and bundle to my wife Teresa Panza by a messenger; and I will take it as a great favour and will not fail to serve her in all that may lie within my power; and as you are about it you may enclose a kiss of the hand to my master Don Quixote that he may see I am grateful bread; and as a good secretary and a good Biscayan you may add whatever you like and whatever will come in best; and now take away this cloth and give me something to eat, and I'll be ready to meet all the spies and assassins and enchanters that may come against me or my island.
Guardsmen, officers, courtiers, servants, and slaves ran helter-skelter through the corridors and apartments carrying messages and orders, and searching for signs of the assassin.
I am an assassin, then, and not an officer of justice
It is clear that the assassins were in the room where Mademoiselle L'Espanaye was found, or at least in the room adjoining, when the party ascended the stairs.
He could not be an assassin, and it was repugnant to Monk to believe him to be a spy, but there was sufficient finesse and at the same time firmness in Athos to lead Monk to fancy he was a conspirator.
But it might have been an assassin that pulled the bell ready to kill a poor harmless woman.

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