Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “ARTHROGRYPOSIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 271 records · Page 15Linked to original sources
PubMed1980

The pathophysiology of arthrogryposis multiplex congenita neurologica.

Eleven patients with arthrogryposis multiplex congenita neurologica have been reviewed. Distinct patterns of deformity and muscle activity were identified which have been correlated with specific levels of segmental neurological motor deficit without sensory loss. The clinical picture was consistent with localised lesions of the anterior horn cell cell columns. This finding agreed with the recorded pathological lesions. Orthopaedic treatment should take account of the paralytic nature of the deformities.

Adolescent↗
PubMed1984

Talectomy for arthrogryposis multiplex congenita.

Eighteen patients (34 feet) with arthrogryposis multiplex congenita treated by talectomy for rigid equinovarus deformity were reviewed. The average follow-up was 11 years. Twenty-four feet (71%) were considered satisfactory; the remainder were improved. Seven feet required further operations to correct recurrence of the deformity, but finally all could be fitted with boots or shoes and all patients could walk. The history of talectomy is reviewed and the operative details described.

Arthrogryposis↗
PubMed1984

Talectomy for club foot in arthrogryposis.

Talectomy was performed on 10 patients (15 feet) for club foot deformity in arthrogryposis multiplex congenita. These were reviewed after an average follow-up of eight years. At follow-up nine feet were plantigrade, and six had less than 15 degrees residual equinus at the ankle. All the feet were asymptomatic but had mild residual adduction of the forefoot and marked stiffness of the hindfoot. Seven feet developed spontaneous bony ankylosis in the tibiotarsal joint. The common technical errors were incomplete removal of the talus and incorrect positioning of the calcaneus in the ankle mortise.

Arthrogryposis↗
PubMed1978

Arthrogryposis multiplex congenita as part of an inherited symptom complex: two case reports and a review of the literature.

Two first cousins appear to have an autosomal recessive disorder consisting of arthrogryposis multiplex congenita (AMC), pterygium coli, congenital cervical spine fusions, and some features of the Eagle-Barrett syndrome. A review of the literature is summarized including the broad concepts of AMC as well as some syndromes in which AMC is but one manifestation.

Arthrogryposis↗
PubMed1988

Arthrogryposis multiplex congenita, Pena-Shokeir phenotype, with gastroschisis and agenesis of the leg.

A young mother had a stillborn infant at 33 weeks' gestation, the pregnancy complicated by polyhydramnios. The parents were unrelated, healthy, and both had a normal karyotype. The infant had multiple malformations such as ankylosis, facial anomalies, and pulmonary hypoplasia. A severe gastroschisis and agenesis of the right leg were also present. The neuropathologic findings were those of marked atrophy of anterior horn motor cells in the spinal cord and neurogenic muscle atrophy. This is a case of Pena-Shokeir syndrome with two additional features: gastroschisis and agenesis of the right leg. This syndrome represents a lethal form of arthrogryposis multiplex congenita, and the essential neuropathologic findings are marked reduction in the number of spinal motor cells and neurogenic muscle atrophy.

Abdominal Muscles↗
PubMedDate not supplied

Lethal congenital muscular dystrophy with arthrogryposis multiplex congenita: three new cases and review of the literature.

Congenital muscular dystrophy (CMD) comprises a heterogeneous group of muscle disorders. We report on two stillborn sibs with early lethal CMD and a prematurely born boy who died within minutes after birth. The pregnancies were complicated by polyhydramnios. All presented with arthrogryposis multiplex congenita, severe muscle wasting, lung hypoplasia, and hydrops. The muscle biopsies showed fibrosis, variation in fiber size, and extensive fat replacement compatible with muscular dystrophy. Fatal CMD seems to be distinct from CMD with survival after birth and is probably autosomal recessively inherited.

Arthrogryposis↗
PubMedDate not supplied

Lethal arthrogryposis multiplex congenital (fetal akinesia deformation sequence, FADS).

Arthrogryposis multiplex congenital (AMC) is the presence at birth of multiple congenital contractures in an intact skeleton. The severity of the condition is highly variable and the possible underlying causes are numerous. Fetal immobility and lesions of the brain, spinal cord, peripheral nerves and muscle, along with mechanical restriction of the fetus in utero are the pathogenic mechanisms that need to be considered. Etiological factors that have been implicated in the development of AMC include genetic conditions, infections, drugs, toxins, maternal hyperthermia, and maternal illness. This review will concentrate on the severe end of the spectrum of AMC that results in disease that is lethal pre- or postnatally, and will discuss the pathology, pathogenesis, etiology, and practical approach to this diversely expressed condition.

Arthrogryposis↗
PubMed1993

Muscle ultrasonography in arthrogryposis. Comparison with clinical, neuromyographic and histologic findings in 41 cases.

We studied the ultrasonographic appearance of muscles in 41 patients with arthrogryposis multiplex congenita (AMC). The observations were compared to the findings in clinical testing, electroneuromyography, and histological examination. The strength of the muscles in AMC-patients did not always correspond to the ultrasonographic morphology of the muscles. In extension contractures of the elbow and in equinovarus adductus deformities of the foot there was a distribution of pathological changes in the muscles corresponding to the primary deformity. The sound transfer through the muscles was decreased, notably in the neurogenic form of AMC. Ultrasonography is an important complement to the clinical examination and it should always be done prior to surgery and muscle biopsy.

Adolescent↗
PubMed2000

Congenital arthrogryposis associated with atlantoaxial subluxation and dysraphic abnormalities. Case report.

The authors report the case of a 27-year-old woman with an arthrogryposis multiplex congenita (AMC) associated with atlantoaxial subluxation. To the authors' knowledge, this is the first report of its kind. The authors review the literature with reference to dysraphic abnormalities associated with atlantoaxial subluxation and with AMC. The patient presented with severe tetraparesis following a minor traffic accident. She underwent a procedure in which transoral decompression and dorsal stabilization were performed and, postoperatively, made a good clinical outcome. The authors stress the need for diagnostic neuroimaging of the craniocervical junction in patients with AMC.

Accidents, Traffic↗
PubMedDate not supplied

Ocular findings in arthrogryposis multiplex congenita.

Close ophthalmological follow-up of a case of arthrogryposis multiplex congenita disclosed a progressing paresis of the lateral recti, a gradual decrease in the corneal blinking reflex by the age of eight weeks and a clear deterioration of the visual evoked responses by the first 16 weeks of life along with preservation of normal ERG responses. These observations are believed to represent the gradual affection of the motor and sensorial cranial nerves occurring post-natally in this case of AMC.

Adult↗
PubMed2000

Treatment of the upper limb in the child with arthrogryposis.

There are good options to maximize upper limb function for the child with arthrogryposis. Preservation of available elbow and wrist motion is essential. A comprehensive plan for upper limb treatment includes operative and nonoperative care.

Arthrogryposis↗
PubMed2000

Misoprostol embryotoxicity: clinical evaluation of fifteen patients with arthrogryposis.

We report on clinical evaluations of Brazilian patients with misoprostol-induced arthrogryposis. All 15 patients had growth retardation, underdeveloped bones, short feet with equinovarus, rigidity of several joints with skin dimples and webs, decreased movement of legs stemming from neurologic impairment, bilateral symmetrical hypoplasia or atrophy of limb muscles, and absent tendon reflexes. Of the 15 patients, five had upper limb deformities in addition to lower limb involvement, and one had spinal cord disruption leading secondarily to segmental sensory loss and neurogenic bladder and bowel. Electroneuromyography of five patients indicated that the abnormalities were of neurogenic origin and suggestive of anterior horn cell defects. All of their mothers took 400-4,800 mcg of misoprostol orally or vaginally at 8 to 12 weeks of pregnancy. Our observations support a previously stated caution with regard to the embryotoxicity of misoprostol.

Abnormalities, Drug-Induced↗
PubMed2001

[Anesthesia using propofol for a patient with arthrogryposis multiplex congenita].

Arthrogryposis multiplex congenita (AMC) is a syndrome with multiple persistent limb contractures, often accompanied by associated anomalies. Pediatric patients with AMC frequently require operations, necessitating general endotracheal anesthesia. We report on the anesthesia used for a 14-year-old male with AMC, who required the surgical removal of soft tissue from both hip joints. We used propofol and vecuronium bromide to induce anesthesia, and maintained anesthesia with 40% nitrous oxide, oxygen, and propofol at 6 mg.kg-1.1 hr-1. Both induction and maintenance were smooth, and no hyperthermia occurred perioperatively. Propofol can be safely used for anesthesia in AMC patients.

Adolescent↗
PubMed2001

Maxillofacial implications and surgical treatment of arthrogryposis multiplex congenita.

A case of arthrogryposis multiplex congenita that affected a patient's temporomandibular joint is described. Preoperatively, the patient's interincisal opening was limited to 9 mm. Elective surgery was performed, which consisted of bilateral coronoidotomies, right and left meninscectomies, capsular release, and lateral pterygoid myotomies. Physical therapy was initiated postoperatively. Eighteen weeks after the surgery, the patient was able to open 18 mm and force open to 20 mm. The patient also noted significant improvement in speech and jaw function in the postoperative period.

Adult↗