نتایج جستجو برای: congenital myasthenia

تعداد نتایج: 126576  

Journal: :Twin research and human genetics : the official journal of the International Society for Twin Studies 2006
Kari Hemminki Xinjun Li Kristina Sundquist

Diseases of the myoneural junction and muscle are disabling and some are life-threatening. Recent successes in the identification of the underlying genetic mechanisms have had profound implication for their diagnostics, treatment and classification. We define familial risks for siblings who were hospitalized for or deceased from diseases of the myoneural junction and muscle. A nationwide databa...

Journal: :Postgraduate medical journal 2004
B R Thanvi T C N Lo

Myasthenia gravis is an autoimmune disorder caused by autoantibodies against the nicotinic acetylcholine receptor on the postsynaptic membrane at the neuromuscular junction and characterised by weakness and fatigability of the voluntary muscles. It has a bimodal peak of incidence with first peak in the third decade and the second peak in the sixth decade. It is probably underdiagnosed in the ve...

Journal: :Brain : a journal of neurology 2014
Sophie Nicole Amina Chaouch Torberg Torbergsen Stéphanie Bauché Elodie de Bruyckere Marie-Joséphine Fontenille Morten A Horn Marijke van Ghelue Sissel Løseth Yasmin Issop Daniel Cox Juliane S Müller Teresinha Evangelista Erik Stålberg Christine Ioos Annie Barois Guy Brochier Damien Sternberg Emmanuel Fournier Daniel Hantaï Angela Abicht Marina Dusl Steven H Laval Helen Griffin Bruno Eymard Hanns Lochmüller

Congenital myasthenic syndromes are a clinically and genetically heterogeneous group of rare diseases resulting from impaired neuromuscular transmission. Their clinical hallmark is fatigable muscle weakness associated with a decremental muscle response to repetitive nerve stimulation and frequently related to postsynaptic defects. Distal myopathies form another clinically and genetically hetero...

2013
Anne Becker Nicole Ludwig Andreas Keller Björn Tackenberg Christian Eienbröker Wolfgang H. Oertel Klaus Fassbender Eckart Meese Klemens Ruprecht

BACKGROUND Myasthenia gravis is a disorder of neuromuscular transmission associated with autoantibodies against the nicotinic acetylcholine receptor. We have previously developed a customized protein macroarray comprising 1827 potential human autoantigens, which permitted to discriminate sera of patients with different cancers from sera of healthy controls, but has not yet been evaluated in ant...

Journal: :Teratology 2000
A Polizzi S M Huson A Vincent

BACKGROUND Arthrogryposis multiplex congenita (AMC) is defined as nonprogressive congenital contractures that generally result from lack of fetal movement in utero. AMC is a feature of many congenital disorders caused by genetic, environmental, or other factors. One rare cause of AMC is maternal myasthenia gravis (MG). This is an autoimmune disorder, caused by antibodies to the nicotinic acetyl...

Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2015
Julien Messéant Alexandre Dobbertin Emmanuelle Girard Perrine Delers Marin Manuel Francesca Mangione Alain Schmitt Dominique Le Denmat Jordi Molgó Daniel Zytnicki Laurent Schaeffer Claire Legay Laure Strochlic

The muscle-specific kinase MuSK is one of the key molecules orchestrating neuromuscular junction (NMJ) formation. MuSK interacts with the Wnt morphogens, through its Frizzled-like domain (cysteine-rich domain [CRD]). Dysfunction of MuSK CRD in patients has been recently associated with the onset of myasthenia, common neuromuscular disorders mainly characterized by fatigable muscle weakness. How...

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