Edit concept Question Editor Create issue ticket

Arthrogryposis due to Muscular Dystrophy


Presentation

  • Abstract The clinical and pathologic findings in two male siblings with congenital myopathy are presented. The pathologic changes in both cases were identical to those seen in progressive muscular dystrophy.[pediatrics.aappublications.org]
  • With this goal in mind, the editors have taken great care to ensure that the presentation of complex clinical information is at once scientifically accurate, patient oriented, and accessible to readers without a medical background.[books.google.com]
  • The most common type of nemaline rod myopathy presents in the infantile stage with 42% of patients presenting in the neonatal period. [6] While there has been an association with polyhydramnios, decreased fetal movements, and an abnormal fetal presentation[emedicine.medscape.com]
  • The subject presented with pronounced mental retardation, was slightly underweight, and had facial dymorphism, and the typical alterations at the level of the thumbs (short, wide and radially deviated) and toes.[moh-it.pure.elsevier.com]
  • Vertebral changes are often present (Figure 166.3). Figure 166.3. — Stuve-Widerman syndrome.[pediatricneuro.com]
Skin Atrophy
  • ., 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.[medical-dictionary.thefreedictionary.com]

Treatment

  • Knee Contractures Treatment operative soft tissue releases (especially hamstrings) indications flexion contracture 30 degrees best performed early (6-9 months of age) perform before hip reduction to assist in maintenance of reduction femoral angulation[orthobullets.com]
  • Based on this evaluation, we will recommend a treatment plan and review it with you and your child. Your child has therapy Monday to Saturday, typically in the morning and afternoon.[seattlechildrens.org]
  • Paralysis & Pain Treatment Center : 9819297772 Careers Our Locations Andheri Center Home About From Founders Desk Corporate Profile Vision Mission Gallery Photo Gallery Video Testimonial Virtual Tour News Articles Management Network Tribute FAQ Page Single[physioline.in]
  • The most common treatments for arthrogryposis are: Open Reduction Open reduction is a surgery that returns a dislocated or misshapen joint to its proper place.[gillettechildrens.org]
  • Part one discusses the approach to neuromuscular disorders, covering principles and basics, neuromuscular investigations, and assessment and treatment of neurological disorders.[books.google.es]

Prognosis

  • Prognosis Ventilator dependence in the neonatal period is associated with a poor prognosis. Prognosis also depends on natural history and the patient’s response to therapy.[boneandspine.com]
  • Prognosis depends on the specific etiology of the contractures The incidence of abnormal joint contractures and other accompanying malformations in eight patients observed with three-dimensional ultrasonography are described[elsevier.es]
  • Prognosis Prognosis depends on the underlying cause but most have a normal lifespan. If, however, there is a central nervous system problem in addition, about half of patients die in the first year.[patient.info]
  • Prognosis The prognosis for spinal muscular atrophy is variable. Life expectancy is dependent on the degree of respiratory impairment present.[healthofchildren.com]

Etiology

  • Abstract Arthrogryposis, or multiple congenital contractures, is the occurrence of joint contractures of diverse etiology in the prenatal period.[elsevier.es]
  • The etiology of arthrogryposis (multiple congenital contracture). Clin Orthop Relat Res. 1985;(194):15-29. Kowalczyk B, Feluś J. Arthrogryposis: an update on clinical aspects, etiology, and treatment strategies. Arch Med Sci. 2016 Feb 1;12(1):10-24.[now.aapmr.org]
  • We treat all levels of SCI including ventilator dependence, due to traumatic or non-traumatic etiology, complete or incomplete at any level, and comorbidities including brain injury, polytrauma, fractures, halo fixation, neurogenic bowel/bladder, DVT,[seattlechildrens.org]
  • Arthrogryposis (Multiple Congenital Contractures): Diagnostic Approach to Etiology, Classification, Genetics, and General Principles. Eur J Med Genet 2014;57:464-472. Filges I, Hall JG.[rarediseases.org]
  • Table 674-1 ASSOCIATED ETIOLOGIES OF ARTHROGRYPOSIS ARTHROGRYPOSIS DUE TO NERVOUS SYSTEM DISORDERS DISTAL ARTHROGRYPOSIS SYNDROMES PTERYGIUM SYNDROMES MYOPATHIES ABNORMALITIES OF JOINTS AND CONTIGUOUS TISSUE SKELETAL DISORDERS INTRAUTERINE AND MATERNAL[clinicalgate.com]

Epidemiology

  • Introduction Nonprogressive congenital disorder involving multiple rigid joints (usually symmetric) leading to severe limitation in motion Epidemiology incidence 1:3000 live births Mechanism symmetry of contractures due to immobilization in utero neurogenic[orthobullets.com]
  • Arthrogryposis Epidemiology The occurrence of AMC is rather rare. Only 1 out of 3000 people gets affected by this disorder. Amyoplasia is the most common form of this disease and is observed in nearly 43% of all reported cases.[primehealthchannel.com]
  • Epidemiology of depression with psychotic experiences and its association with chronic physical conditions in 47 low- and middle-income countries . Psychol Med 2017. 47: 531-542.[fsjd.org]
  • Epidemiology It may occur to some extent in 1 in 3,000 to 1 in 5,000 live births. [ 3 ] The condition is usually detected at birth or before by ultrasound examination. It is often secondary to other conditions.[patient.info]
  • Epidemiology Arthrogryposis is a rare condition. Some authors say the overall prevalence is one in 3000 and others say it is one in 11000-12000 among European live births.[monsterologist.blogspot.com]
Sex distribution
Age distribution

Pathophysiology

  • Amyoplasia, the most common type of arthrogryposis: the pathophysiology for good outcome. Pediatr 1996:97(2):225–31. [ Pubmed ] Brown LM, Robson MJ, Sharrard WJ. The pathophysiology of arthrogryposis multiplex congenita neurologica.[ijcasereportsandimages.com]
  • […] congenital disorder involving multiple rigid joints (usually symmetric) leading to severe limitation in motion Epidemiology incidence 1:3000 live births Mechanism symmetry of contractures due to immobilization in utero neurogenic (90%) myopathic (10%) Pathophysiology[orthobullets.com]
  • Fibrosis What is basic pathophysiological mechanism for AMC? Multiple joint contractures - due to lack of fetal movement mobility and self-care skills Type 1 AMC: clinical manifestations? 1. Flexed and dislocated hips 2. Extended knees 3.[quizlet.com]
  • Pathophysiology of Arthrogryposis Decreased fetal movements or fetal akinesis has been suggested to be the major contributory cause. The decrease in activity could be due to fetal abnormalities or maternal disorders like.[boneandspine.com]
  • Patho-anatomy/physiology The pathophysiology is related to the specific type of arthrogryposis. Exact mechanisms are not well understood in some subtypes of arthrogryposis.[now.aapmr.org]

Prevention

  • However some reason prevents the fetus movement leading to contracture of joints and muscles.[diseasespictures.com]
  • SMA cannot be prevented, but prospective parents can request genetic testing if they may be carriers. Types There are different types of SMA.[medicalnewstoday.com]
  • However, it is necessary to seek treatment to prevent further impediments of the joints. What causes Arthrogryposis Multiplex Congenita? There are many causes of the disease, some of which are inherited.[aanem.org]
  • Prevention There is no way to prevent spinal muscular atrophy. However, genetic counseling is crucial so that parents can make informed decisions about having children.[healthofchildren.com]
  • Anything that prevents normal movement of a baby in the womb will lead to contracture, where a joint does not have a full range of movement.[ipass.org.uk]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!