Arthrogryposis Multiplex Congenita
ثبت نشده
چکیده
Arthrogryposis multiplex congenita is a collective term applied to a very large number of different syndromes characterised by nonprogressive, multiple joint contractures present at birth. [1, 2]The joints usually develop normally in early embryonic life but, as gestation progresses, movements are required to facilitate normal development. Where there are abnormalities that prevent this from occurring, such as neurological or connective tissue disorders or physical restriction, the condition forms. The muscles involved are partially or completely replaced by fat and fibrous tissue. The most common form, accounting for 40% of cases, is amyoplasia.
منابع مشابه
Characterization of a group unrelated patients with arthrogryposis multiplex congenita.
OBJECTIVE Arthrogryposis multiplex congenita is a relatively rare neuromuscular syndrome, with a prevalence of 1:3000-5000 newborns. In this study, the authors describe the clinical features of a group of 50 unrelated Mexican patients with arthrogryposis multiplex congenita. METHODS Patients were diagnosed by physical and radiographic examination and the family history was evaluated. RESULT...
متن کاملArthrogryposis Multiplex Congenita, Epileptic Seizures and Cortical Dysplasia: a Case Report
We have presented a case of a 22-year-old patient having a rare variety of arthrogryposis multiplex congenita arthrogryposis with epileptic seizures and defect in neural migration. We have described the patient’s disease history, the clinical, and laboratory data by giving prominence to the lack of mental retardation and the late onset of the generalized tonic-clonic seizures, despite the prese...
متن کاملOrthognathic surgery for management of Arthrogryposis Multiplex Congenita: Case report and review of the literature.
Arthrogryposis Multiplex Congenita is a condition characterized by multiple contractures of the joints. Involvement of the temporomandibular joint is a common complication that limits mandibular opening. A case of Arthrogryposis Multiplex Congenita with anterior open bite and limited maximal incisal opening is presented. Orthognathic surgery, consisting of segmental LeFort I osteotomy and bilat...
متن کاملPrenatal diagnosis of arthrogryposis multiplex congenita.
Arthrogryposis multiplex congenita may be defined as a systemic articular dysplasia characterized by articular rigidity in a many locations of congenital origin. A case was presented in which this clinical sign was diagnosed at prenatal phase and it may have many underlying causes.
متن کاملArthrogryposis multiplex congenita distal type II associated with facial abnormality, renal abnormality, polydactyly and Hirschprung's disease.
A case of arthrogryposis multiplex congenita distal type II associated with facial abnormality, renal abnormality, postaxial poydactyly and Hirschprung's disease is described. It appears to be a new form of an autosomal recessive disorder.
متن کاملMouthstick prosthesis placement in a 19-month-old arthrogryposis multiplex congenita patient: case report.
Arthrogryposis multiplex congenita is a congenital disorder distinguished by generalized joint immobility and skeletal muscle hypoplasia. Although the afflicted individual has normal intelligence, physical constraints often prevent independent living. This case report describes the successful fabrication and placement of a mouthstick prosthesis in a 19-
متن کامل