Respiratory failure in clustered acetylcholine receptor autoantibody‐positive myasthenia gravis

2019 ◽  
Vol 60 (3) ◽  
Author(s):  
Conor Kavanagh ◽  
James Lilleker ◽  
Ronan MacDonagh
Keyword(s):  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor

THUR 213 An unusual case of cluster acetylcholine receptor antibody positive myasthenia gravis

2018 ◽  
Vol 89 (10) ◽  
pp. A30.3-A30
Author(s):  
Kavanagh Conor ◽  
Lilleker James ◽  
Mohanraj Rajiv ◽  
Smith Thomas
Keyword(s):  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
High Dose ◽  
Antibody Testing ◽  
Limb Weakness ◽  
Serological Screening ◽  
Achr Antibody ◽  
First Case ◽  
High Dependency

A 73-year-old builder presented with fatigable ptosis and ophthalmoplegia. He was started on pyridostigmine for presumed ocular myasthenia gravis (MG). Despite making an initial improvement he subsequently developed dysarthia, dysphonia and a head drop within 3 weeks. At this time, he also had mild proximal weakness and marked asymmetrical distal weakness of the upper limbs and mild distal lower limb weakness.Conventional serological screening for acetylcholine receptor (AChR) and muscle specific kinase antibodies were negative. However, extended testing using a cell-based cluster assays demonstrated AChR antibody positivity. Repetitive nerve stimulation demonstrated significant decrement more prominent in distal muscles.Despite high dose prednisolone, azathioprine, several courses of IV Immunoglobulins and plasma exchange the patient has had frequent admissions to the high dependency and intensive care setting with respiratory failure.Cluster AChR antibody positive MG has been described only within the last decade and is usually mild and treatment responsive. This is the first case reported to have required intubation and ventilation due to respiratory failure. This case highlights the importance of extending the routine panels for antibody testing in patients with ‘seronegative’ MG and the need to remain vigilant in patients with cluster-AChR positive disease.

Therapeutic Immunoadsorption of Acetylcholine Receptor Antibodies in Myasthenia Gravis

1988 ◽  
Vol 540 (1 Advances in N) ◽  
pp. 554-556 ◽  
Author(s):  
TAKESHI SATO ◽  
YASUNORI ISHIGAKI ◽  
TADATOSHI KOMIYA ◽  
HIROSHI TSUDA
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Antibodies

Subunit specificity of the acetylcholine receptor antibodies in double seropositive myasthenia gravis

2021 ◽  
Author(s):  
Vasiliki Zouvelou ◽  
Maria Michail ◽  
Maria Belimezi ◽  
Paraskevi Zisimopoulou
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Antibodies

scholarly journals Myasthenia gravis in children: analysis of 18 patients

2001 ◽  
Vol 59 (3B) ◽  
pp. 681-685 ◽  
Author(s):  
Maria da Penha A. Morita ◽  
Alberto A. Gabbai ◽  
Acary S.B. Oliveira ◽  
Audrey S. Penn
Keyword(s):  
Myasthenia Gravis ◽  
Intravenous Immunoglobulin ◽  
Acetylcholine Receptor ◽  
Nerve Stimulation ◽  
Chest Ct ◽  
Clinical Severity ◽  
Myasthenic Crisis ◽  
Repetitive Nerve Stimulation ◽  
Follicular Hyperplasia ◽  
Receptor Antibodies

Myasthenia gravis (MG) in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1). Eleven patients (61%) presented moderate or severe generalized disease and 4 (22%) had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47%) out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested) and the levels had no relation to clinical severity. Nine out of 16 patients (56%) worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia). Three patients (75%) improved markedly after thymectomy and one (25%) worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.

scholarly journals Myasthenia Gravis Presenting Initially as Acute Respiratory Failure

2014 ◽  
Vol 60 (1) ◽  
pp. e14-e16 ◽  
Author(s):  
S. J. Lee ◽  
J. Hur ◽  
T. W. Lee ◽  
S. Ju ◽  
S. H. Lee ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Acute Respiratory Failure ◽  
Myasthenia Gravis

Characterization of peripheral blood acetylcholine receptor-binding B cells in experimental myasthenia gravis

2011 ◽  
Vol 271 (2) ◽  
pp. 292-298 ◽  
Author(s):  
Windy Allman ◽  
Shamsher S. Saini ◽  
Erdem Tuzun ◽  
Premkumar Christadoss
Keyword(s):  
B Cells ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Binding ◽  
Peripheral Blood
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