scholarly journals A challenging case of hypercapnic respiratory failure during pregnancy

2020 ◽  
pp. 1753495X2094470
Author(s):  
B Vijayakumar ◽  
M Cao ◽  
L Mackillop ◽  
N Sarangmat ◽  
MI Leite ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Early Recognition ◽  
Respiratory Muscles ◽  
Normal Pregnancy ◽  
Term Infant ◽  
Limb Weakness ◽  
Hypercapnic Respiratory Failure ◽  
Life Threatening ◽  
Muscle Specific Kinase

We describe a 40-year-old female who presented with progressive breathlessness and hypercapnic respiratory failure during pregnancy secondary to undiagnosed muscle-specific kinase myasthenia gravis. Her presentation was progressive and protracted, having over five contacts with healthcare professionals over nine months, many of these predating her pregnancy. Her atypical presentation for myasthenia with minimal limb weakness led to consideration of other causes of hypercapnic respiratory failure. Once diagnosed, she was treated with intravenous immunoglobulin and non-invasive ventilation. She gave birth to a pre-term infant by planned caesarean section. Her insidious presentation and the progressive nature of her breathlessness were unusual and our report highlights the predominant involvement of respiratory muscles in muscle-specific kinase myasthenia. Her pregnancy may have further delayed her diagnosis due the attribution of some symptoms to normal pregnancy. Early recognition and treatment of myasthenia gravis are important to prevent life-threatening complications.

Acetylcholinesterase Inhibitors Test Confirmed Myasthenia Gravis in Psychosis Remitted by Aripiprazole

2018 ◽  
Vol 68 (12) ◽  
pp. 2952-2954
Author(s):  
Andreea Teodorescu ◽  
Petru Ifteni ◽  
Paula Petric ◽  
Sebastian Toma ◽  
Adrian Baracan ◽  
...  
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Receptor Tyrosine Kinase ◽  
Skeletal Muscles ◽  
Early Recognition ◽  
Respiratory Muscles ◽  
Acetylcholinesterase Inhibitors ◽  
Psychotic Symptoms ◽  
Life Threatening ◽  
Myastenia Gravis

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease affecting the neuromuscular junction and causes weakness in the skeletal muscles. The acetylcholine receptor is usually attacked in skeletal muscles, but other components of neuromuscular junction, such as muscle-specific receptor tyrosine kinase, may be affected. MG can be life-threatening when the respiratory muscles are involved. The first symptom in about 2 out of 3 cases is the damage of the extrinsic eye muscles. The condition is treatable, so an early recognition is needed. Although there have been reports of associations between psychosis and myasthenia gravis it is unclear if psychotic symptoms in MG are an integral part of the various manifestations of this disease, or are due to another co-occurring distinct disorder. Sometimes psychotic episodes could disguise the simptoms of myastenia gravis, and delay the diagnosis.

scholarly journals Coexistence of myasthenia gravis with hypokalemic periodic paralysis: a rare presentation

2019 ◽  
Vol 12 (10) ◽  
pp. e231241 ◽  
Author(s):  
Farah Gul Khan ◽  
Sidra Namran
Keyword(s):  
Myasthenia Gravis ◽  
Young Female ◽  
Periodic Paralysis ◽  
Acute Onset ◽  
Hypokalemic Periodic Paralysis ◽  
Rare Presentation ◽  
Limb Weakness ◽  
Oral Steroids ◽  
Life Threatening ◽  
Neurological Illnesses

Bilateral symmetrical weakness of acute onset is not very uncommon and the differential varies widely from life-threatening neurological illnesses to metabolic and electrolyte derangements. We report the case of a young female with severe muscle weakness, respiratory distress and hypokalemia who required immediate intubation on arrival to emergency department. During hospital course, even after normalisation of serum potassium and some improvement in limb weakness, patient failed multiple attempts of extubation because of type II respiratory failure. Subsequently, acetyl cholinesterase antibodies were checked which came out positive, and diagnosis of myasthenia gravis and hypokalemic periodic paralysis was made. She was successfully extubated after intravenous pulse steroids, pyridostigmine and plasmapheresis. Patient was finally discharged home on oral steroids, pyridostigmine and azathioprine. In a patient presenting with hypokalemic weakness, the suspicion of a second disorder should be very high if weakness fails to resolve following correction of hypokalemia.

scholarly journals Neuroleptic Malignant Syndrome: Diagnostic and Therapeutic Dilemmas

2005 ◽  
Vol 16 (1) ◽  
pp. 9-13 ◽  
Author(s):  
Abebaw Fekadu ◽  
Jonathan I. Bisson
Keyword(s):  
Renal Failure ◽  
Respiratory Failure ◽  
Neuroleptic Malignant Syndrome ◽  
Dopaminergic System ◽  
Early Recognition ◽  
Delayed Diagnosis ◽  
Antipsychotic Medications ◽  
Comprehensive Management ◽  
Life Threatening

Neuroleptic malignant syndrome (NMS) is a life threatening medical state complicating the use of antipsychotic medications and other drugs that affect the dopaminergic system on administration or withdrawal. The condition was recognised nearly half a century ago, shortly after the discovery of antipsychotic medications. However, there are still no systematic studies about NMS. There are no definitive guidelines on its treatment. Although early recognition is emphasised and usually possible, delayed diagnosis is not rare. We here report on a case of NMS complicated by renal failure, and possibly respiratory failure. The report underscores the seriousness of delayed diagnosis and puts forward a comprehensive management recommendation based on our experience and the existing literature.

scholarly journals Biliary-Pleural Fistula following Portal Vein Embolization for Perihilar Cholangiocarcinoma

2017 ◽  
Vol 11 (2) ◽  
pp. 277-283 ◽  
Author(s):  
Mujtaba Mohammed ◽  
Katsuhiro Kobayashi ◽  
Mohammed Jawed
Keyword(s):  
Respiratory Failure ◽  
Pleural Effusion ◽  
Portal Vein ◽  
Portal Vein Embolization ◽  
Biliary Obstruction ◽  
Early Recognition ◽  
Pleural Space ◽  
Perihilar Cholangiocarcinoma ◽  
Life Threatening ◽  
Life Threatening Complication

Biliary-pleural fistula (BPF), an abnormal communication between the biliary tract and pleural space, is a rare but potentially life-threatening complication following percutaneous biliary intervention. We report a case of BPF following portal vein embolization (PVE) in a 79-year-old woman with obstructive jaundice secondary to perihilar cholangiocarcinoma. The patient successfully underwent right-sided PVE; however, the patient developed a symptomatic right-sided bilious pleural effusion the following day. Despite aggressive drainage of the pleural effusion with a large-bore chest tube and maximal medical management, the patient died from respiratory failure and pneumonia. Although rare, knowledge of this complication is important when performing PVE in patients with biliary obstruction because it can be life-threatening. Early recognition and management of this complication are crucial to avoid a poor outcome.

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.

scholarly journals MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation

2021 ◽  
Author(s):  
Nicole König ◽  
◽  
Henning R. Stetefeld ◽  
Christian Dohmen ◽  
Philipp Mergenthaler ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Myasthenia Gravis ◽  
Hospital Length ◽  
Hospital Length Of Stay ◽  
Myasthenic Crisis ◽  
Specific Therapy ◽  
Multiple Chronic Disease ◽  
Threatening Condition ◽  
Life Threatening ◽  
Muscle Specific Kinase

AbstractMyasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in ~ 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 ± 53.1 vs. 17.4 ± 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 ± 49.5 vs. 21.2 ± 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 ± 47.6 vs. 28.8 ± 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients’ outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.

Update on acute flaccid myelitis: recognition, reporting, aetiology and outcomes

2020 ◽  
Vol 105 (9) ◽  
pp. 842-847
Author(s):  
Duriel Hardy ◽  
Sarah Hopkins
Keyword(s):  
Spinal Cord ◽  
Grey Matter ◽  
Early Recognition ◽  
Respiratory Muscles ◽  
Febrile Illness ◽  
Late Summer ◽  
Close Monitoring ◽  
Limb Weakness ◽  
Surgical Interventions ◽  
Acute Flaccid Myelitis

Acute flaccid myelitis, defined by acute flaccid limb weakness in the setting of grey matter lesions of the spinal cord, became increasingly recognised in 2014 following outbreaks in Colorado and California, temporally associated with an outbreak of enterovirus D68 respiratory disease. Since then, there have been biennial increases in late summer/early fall. A viral infectious aetiology, most likely enteroviral, is strongly suspected, but a definitive connection has yet to be established. Patients typically present with asymmetric weakness, maximal proximally, in the setting of a febrile illness. MRI demonstrates T2/FLAIR abnormalities in the central grey matter of the spinal cord, and cerebrospinal fluid typically shows a lymphocytic pleocytosis with variable elevation in protein. The weakness may be progressive over several days and involve respiratory muscles, making early recognition and close monitoring essential. Other complications in the acute period may include autonomic instability and bowel/bladder involvement. There is no clear recommended treatment at this time, although intravenous immunoglobulin, steroids and plasma exchange have been used. Intensive therapies and rehab services have shown benefit in maximising function, and surgical interventions may be considered in cases without optimal response to therapies. Close attention should also be paid to psychosocial factors. Prognosis is generally guarded, and additional factors that predict final outcome, including host factors and treatment effects, have yet to be elucidated. Multicentre collaborative efforts will be required to provide answers about this rare but serious disorder.

One- to Three-Year Outcome for 14 Neonatal Survivors of Extracorporeal Membrane Oxygenation

PEDIATRICS ◽  
1986 ◽  
Vol 78 (4) ◽  
pp. 692-698
Author(s):  
Alice French Andrews ◽  
Cynthia A. Nixon ◽  
Robert E. Cilley ◽  
Dietrich W. Roloff ◽  
Robert H. Bartlett
Keyword(s):  
Respiratory Failure ◽  
Extracorporeal Membrane Oxygenation ◽  
Growth And Development ◽  
Normal Growth ◽  
Term Infant ◽  
Control Group ◽  
Mental Ability ◽  
Membrane Oxygenation ◽  
Life Threatening ◽  
Near Term

Extracorporeal membrane oxygenation, using venoarterial or venovenous perfusion, is a safe and effective procedure in the term or near-term infant with life-threatening respiratory failure. Without extracorporeal membrane oxygenation, due to the severity of their disease, these children are at high risk for neurologic damage, chronic lung disease, and death. Because survival is not expected without extracorporeal membrane oxygenation therapy, there is no corresponding control group to which these survivors may be compared. In this report, we reviewed the outcome at 1 to 3 years in the first 14 survivors of extracorporeal membrane oxygenation treated at our institution. Seven of 14 neonatal extracorporeal membrane oxygenation survivors (50%) were normal or near normal at between 1 and 3 years of age. Ten (71%) had normal mental ability. We conclude that in neonates with high mortality risk from respiratory failure, near-normal growth and development can be expected in the majority who survive with extracorporeal membrane oxygenation treatment.

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