Presentation
Although rare, neonatal weakness can present in utero, and mothers may comment on reduced fetal movements. Polyhydramnios can be present as a result of poor fetal swallowing. [mhmedical.com]
Anti-acetylcholine receptor (anti-AChR) antibodies were present in the serum of both mother and child, in umbilical cord serum and in the amniotic fluid. The presence of anti-AChR antibodies in the amniotic fluid may be one of the causes of the NMG. [ncbi.nlm.nih.gov]
Entire Body System
- Intermittent Claudication
claudication. myasthenia gastrica weakness and loss of tone in the muscular coats of the stomach; atony of the stomach. myasthenia gravis a syndrome of muscular weakness that is aggravated by activity and relieved by rest. [medical-dictionary.thefreedictionary.com]
Gastrointestinal
- Intestinal Perforation
Key words: Myastenia gravis, neonatal, intestinal perforation Advertisement Journal of Apitherapy SUBMIT YOUR ARTICLE NOW [scopemed.org]
Skin
- Hypoplastic Nails
Another drug, mycophenolate mofetil (MMF), is thought to be teratogenic, causing a clinical syndrome which includes hypoplastic nails, shortened fifth fingers, oral cleft, microtia, diaphragmatic hernia, and micrognathia [ 19 ]. [intechopen.com]
Face, Head & Neck
- Hypomimia
The dissociated pattern of neurological disorders (refusal to drink, axial hypotonia, hypomimia, but good contact and normal alertness) led to search for neuromuscular causes or poison. [ncbi.nlm.nih.gov]
Neurologic
- Akinesia
Major akinesia with arthrogryposis and craniosynostosis at birth mimics irreversible disorders of the nervous system of pejorative outcome. [ncbi.nlm.nih.gov]
The principal mechanism leading to the development of AMC is decreased fetal movements (fetal akinesia), which can result from a large number of both fetal and maternal disorders [ 4 ]. [link.springer.com]
Workup
Subsequent workup of the mother revealed myasthenia gravis with muscle-specific kinase antibodies. The infant responded to intravenous immunoglobulin and symptoms normalized. [journals.lww.com]
Edrophonium is primarily but rarely used as a diagnostic tool to predict the response to longer-acting cholinesterase inhibitors (see Workup). [43] AChE inhibitors have a wide variability in the effective dose, depending on the severity and current activity [emedicine.medscape.com]
Treatment
Selection criteria: We considered randomised controlled trials ( RCTs ) and quasi- RCTs comparing ephedrine as a single or add-on treatment with any other active treatment, placebo, or no treatment in adults or children with autoimmune MG, NMG, or CMSs [cochrane.org]
Reports tend to encourage surgical treatment for the latter group. [emedicine.medscape.com]
Anticholinesterases are used in the treatment. In this study, we present a case diagnosed with transient neonatal MG that has developed intestinal perforation after neostigmine treatment. Key words: Myastenia gravis, neonatal, intestinal perforation [scopemed.org]
It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. [myastheniagravisnews.com]
Prognosis
In this context, the early detection of anti-acetylcholine fetal receptor antibodies in the mother may allow rapid diagnosis of transient neonatal myasthenia of favorable prognosis. [ncbi.nlm.nih.gov]
False positive rate Muscle specific tyrosine kinase (MuSK-Ab): present in 38-50% of those negative for AchR-Ab. 8-12% of patients are 'seronegative' and have a better prognosis. [pedclerk.bsd.uchicago.edu]
Treatment and prognosis Babies with transient neonatal myasthenia gravis often recover with treatment in a few weeks or months, as maternal autoantibodies disappear on their own. [myastheniagravisnews.com]
The prognosis has improved very much lately due to timely thymus removal and a lot of young women suffering from MG is in clinical remission without medicaments. [myastheniagravis.cz]
"Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis". Nature Reviews Neurology. 10 (8): 469–482. doi : 10.1038/nrneurol.2014.121. [en.wikipedia.org]
Etiology
Etiology The disorder is related to passive transplacental transfer of maternal anti-acetylcholine receptorantibodies (anti-AChR) or anti-muscle-specific tyrosine kinase antibodies (anti-MuSK) to the neonate. [orpha.net]
Hall JG (1997) Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach and general aspects. J Pediatr Ortop B 6:159–166 CrossRef Google Scholar 2. [link.springer.com]
Preeclampsia In 1979, Duff described an association between myasthenia gravis and preeclampsia. [17] He observed preeclampsia in 3 patients and reasoned that altered immune status could be an etiologic factor in preeclampsia. [emedicine.medscape.com]
Congenital myasthenia presenting in an infant of non-myasthenic mother is rare (1% of cases). [3] Simpson [10] has postulated an auto-immune etiology of adult myasthenia gravis; but there is no association with thymoma or antibodies in congenital myasthenia [jpgmonline.com]
Ongoing controversies regarding etiology, diagnosis, treatment Although little controversy exists regarding the pathogenesis and diagnosis of MG, there remain questions regarding the benefits of thymectomy beyond medical treatment alone in non-thymomatous [clinicaladvisor.com]
Epidemiology
Summary Epidemiology Exact incidence data are not available. The disorder occurs in 10% to 20% of children born to mothers with myasthenia gravis. Clinical description In some cases, the mother may be asymptomatic. [orpha.net]
Sommerfield, T, Chalmers, J, Youngson, G The changing epidemiology of infantile hypertrophic pyloric stenosis in Scotland. Arch Dis Child 2008 ; 93: 1007 – 1007. Google Scholar | Crossref | Medline 4. Sivitz, AB, Tejani, C, Cohen, SG. [journals.sagepub.com]
Friedman, CR, Neimann, J., Wegener, HC, and Tauxe, RV Epidemiology of Campylobacter jejuni infections in the United States and other industrialized nations. [books.google.com]
Hoff JM, Loane M, Gilhus NE, Rasmussen S, Daltveit AK (2011) Arthrogryposis multiplexa congenita: an epidemiological study of nearly 9 million births in 24 EUROCAT registers. [link.springer.com]
Pathophysiology
Epidemiology Most common disorder of neuromuscular transmission Prevalence 5-15 / 100,000 in adults, 1.1 / 1 Million in childhood Related to HLA-DR3, -A1, -B8 and DQw2 Pathophysiology http://jessicagimeno.com/myasthenia-gravis-online-resources/ Autoimmune [pedclerk.bsd.uchicago.edu]
A major understanding of the underlying pathophysiology of autoimmune diseases has been the application of genome wide association scans that have identified a degree of genetic sharing among the autoimmune diseases. [10] Autoimmunity, on the other hand [en.wikipedia.org]
This finding indicates a shared pathophysiology for the two conditions. [link.springer.com]
PATHOPHYSIOLOGY • It is uncertain why ocular muscles are frequently involved in myasthenia and why the disease stays localized to the ocular muscles in 15 percent of cases. • It has been proposed that subtle alterations in the function of extraocular [slideshare.net]
Pathophysiology Depolarization of a motor neuron propagates to the axon terminal, where acetylcholine is released into the synapse. [aao.org]
Prevention
Knowledge of the pathogenesis, genetics, and molecular biology of neuromuscular disorders is essential both in developing and applying new therapies and preventive measures, and in formulating genetic and prognostic advice. [books.google.com]
The goal of treatment is to increase muscle function and prevent swallowing and breathing problems. Most people with this condition can improve their muscle strength and lead normal or near normal lives. [hopkinsmedicine.org]
Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. [books.google.es]
Persons with Myasthenia Gravis have antibodies that block, alter, or destroy their receptors for acetylcholine at the neuromuscular junction, preventing their muscles from contracting. [disabled-world.com]