Myasthenic Crisis as the First Presentation of Myasthenia Gravis: A Case Report

Payus, Alvin Oliver; Hsiang, Justin Leow Wen; Leong, Jia Qian; Ibrahim, Azliza; Ali, Raymond Azman

doi:10.12659/ajcr.928419

Cited by 3 publications

(4 citation statements)

References 5 publications

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“…Although our study confirmed the beneficial role of SNX17 in AChR-MG, its role in MuSK-MG, LRP4-MG remains to be further explored. In addition, the evaluation of SNX17 in this study was mainly in the gastrocnemius muscle, and the MG crisis mainly causes weakness of the respiratory muscles (e.g., intercostal muscles, diaphragm) ( 37 ). Therefore, expanding the exploration of the role of SNX17 in respiratory muscles in the future may help find a novel method to treat the weakness of patients in crisis.…”

Section: Discussionmentioning

confidence: 97%

Sorting nexin 17 increases low-density lipoprotein receptor-related protein 4 membrane expression: A novel mechanism of acetylcholine receptor aggregation in myasthenia gravis

Zhou

et al. 2022

Front. Immunol.

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Myasthenia gravis (MG) is characterized by autoimmune damage to the postsynaptic membrane of the neuromuscular junction (NMJ) with impaired postsynaptic acetylcholine receptor (AChR) aggregation. Low-density lipoprotein receptor-related protein 4 (LRP4) plays an important role in AChR aggregation at endplate membranes via the Agrin–LRP4–muscle-specific receptor tyrosine kinase (MuSK) cascade. Sorting nexin 17 (SNX17) regulates the degradation and recycling of various internalized membrane proteins. However, whether SNX17 regulates LRP4 remains unclear. Therefore, we examined the regulatory effects of SNX17 on LRP4 and its influence on AChR aggregation in MG. We selected C2C12 myotubes and induced LRP4 internalization via stimulation with anti-LRP4 antibody and confirmed intracellular interaction between SNX17 and LRP4. SNX17 knockdown and overexpression confirmed that SNX17 promoted MuSK phosphorylation and AChR aggregation by increasing cell surface LRP4 expression. By establishing experimental autoimmune MG (EAMG) mouse models, we identified that SNX17 upregulation improved fragmentation of the AChR structure at the NMJ and alleviated leg weakness in EAMG mice. Thus, these results reveal that SNX17 may be a novel target for future MG therapy.

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Section: Discussionmentioning

confidence: 97%

Sorting nexin 17 increases low-density lipoprotein receptor-related protein 4 membrane expression: A novel mechanism of acetylcholine receptor aggregation in myasthenia gravis

Zhou

et al. 2022

Front. Immunol.

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“…There is a profound ascending paralysis commonly seen with MG, which was not observed in our patient. Another hallmark manifestation of MG is muscle weakness that is exacerbated by exertion and improves with rest [ 10 ]. This was clinically not observed nor reported by our patient during her hospital stay, effectively ruling out MG. GBS was also considered but much like MG, the pattern of paralysis is ascending which was not consistent with the patient's presentation [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Given the increased morbidity and mortality rates of this disease, a clinical diagnosis of botulism was made. Additional tests such as a Tensilon test for the confirmation of MG, a lumbar puncture to diagnose GBS, and electromyography to diagnose Eaton-Lambert were not completed due to low clinical suspicion [ 10 - 12 ]. Additionally, our patient was only at an estimated gestational age of 14 weeks and four days at the time that she left against medical advice, and she has not been to the hospital or our clinic for prenatal care since.…”

Section: Discussionmentioning

confidence: 99%

Wound Botulism in the Setting of Pregnancy: A Literature Review and Case Report

Ghantarchyan¹,

Phan²,

Hu³

et al. 2022

Cureus

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Botulism is a rare neuroparalytic illness caused by Clostridium botulinum that can manifest as a descending flaccid paralysis, progressing from cranial neuropathies to respiratory failure. Wound botulism, constituting a minority of cases, is majorly associated with the injection of black tar heroin (BTH) in the western United States. A patient population of particular concern is pregnant women, who may experience a more severe course due to the physiological changes that occur in pregnancy. Because botulism in pregnancy lacks pathognomic features, physicians should maintain a high clinical suspicion when faced with a pregnant patient with neurological symptoms and a history of BTH use. Here, we report the case of a 25-year-old G3P1A1 female with a history of BTH use who presented with cranial neuropathies and respiratory insufficiency.

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“…They typically cause fluctuating, exercise-induced muscle weakness, and fatigability. Respiratory and/or bulbar muscles are commonly involved and hypoventilation may be the first clinical sign in some patients [68,69]. With adequate therapy, most patients remain asymptomatic or stable with only minimal clinical signs, but myasthenic crisis requiring mechanical ventilation due to infections, concomitant medications, or drugs may occur in 15% to 20% [70 ▪▪ ].…”

Section: Respiratory Involvement In Neuromuscular Disordersmentioning

confidence: 99%

Hypoventilation syndrome in neuromuscular disorders

Wenninger

Jones

2021

Current Opinion in Neurology

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Purpose of reviewHypoventilation syndrome in neuromuscular disorders (NMDs) is primarily due to respiratory muscle weakness and results in increased morbidity and mortality. This article highlights current aspects of neuromuscular hypoventilation syndrome, including pathophysiology, clinical symptoms, assessment, respiratory involvement in various NMD, and causal and symptomatic treatments with an emphasis on recent research and advances.Recent findings and SummaryNew therapeutic agents have been developed within the last years, proving a positive effect on respiratory system. Symptomatic therapies, including mechanical ventilation and cough assistance approaches, are important in NMD and respiratory muscle training may have benefit in strengthening respiratory muscles and should be offered patients with respiratory muscle weakness the same way as physiotherapy. Correct respiratory assessments and their correct interpretation are hallmarks for early diagnosis of hypoventilation syndrome and treatment.

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Myasthenic Crisis as the First Presentation of Myasthenia Gravis: A Case Report

Cited by 3 publications

References 5 publications

Sorting nexin 17 increases low-density lipoprotein receptor-related protein 4 membrane expression: A novel mechanism of acetylcholine receptor aggregation in myasthenia gravis

Sorting nexin 17 increases low-density lipoprotein receptor-related protein 4 membrane expression: A novel mechanism of acetylcholine receptor aggregation in myasthenia gravis

Wound Botulism in the Setting of Pregnancy: A Literature Review and Case Report

Hypoventilation syndrome in neuromuscular disorders

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