by Although the reported outcomes of MuSK MG patients with respiratory insufficiency show substantial heterogeneity, long-term prognosis appears favorable. Keywords:Myasthenia gravis, Respiratory insufficiency, Immunosuppressive brokers, Prognosis, Therapeutics == Introduction == Although no epidemiological studies on myasthenia gravis (MG) have been done in Lebanon, it has been established Rabbit polyclonal to ADRA1B that this prevalence of muscle-specific tyrosine kinase (MuSK) antibody-positive MG shows a distinct geographical pattern, with a higher occurrence in Difluprednate Mediterranean countries compared to northern regions [1,2]. therapy. == Conclusion == A review of the literature revealed varied clinical presentations and treatment methods among reported cases. Long-term prognosis appears favorable, requiring ongoing immunosuppressive management. Although the reported outcomes of MuSK MG patients with respiratory insufficiency show substantial heterogeneity, long-term prognosis appears favorable. Keywords:Myasthenia gravis, Respiratory insufficiency, Immunosuppressive brokers, Prognosis, Therapeutics == Introduction == Although no epidemiological studies on myasthenia gravis (MG) have been carried out in Lebanon, it has been established that this prevalence of muscle-specific tyrosine kinase (MuSK) antibody-positive MG shows a distinct geographical pattern, with a higher occurrence in Mediterranean countries compared to northern regions [1,2]. As such, we must become more familiar with this subtype of MG since it may differ from other more common subtypes. Pathophysiologically, anti-MuSK antibodies obstruct the binding sites on MuSK responsible for its conversation with essential binding proteins such as low density lipoprotein receptor-related protein 4 (LRP4) and collagen Q (ColQ) [3]. This obstruction results in the inactivation of MuSK, which subsequently leads to a decrease in the postsynaptic density of acetylcholine receptors (AChRs) and disrupts their Difluprednate proper alignment within the postsynaptic membrane [4]. The unique pathophysiology observed in MuSK MG patients could account for the distinct clinical presentation they exhibit. These patients tend to have a heightened incidence of bulbar involvement [59] and respiratory crises [5,7,9,10], setting them apart from other MG subtypes. A case series by Deymeer et al. [9] showed patients with MuSK MG also have a more unfavorable prognosis as they tend to have higher Myasthenia Gravis Foundation of America (MGFA) scales in comparison with anti-AChR-positive subtype, but there was no statistically significant difference from those with seronegative MG. As for treatment, the case-control by Deymeer et al. [9] showed that MuSK MG patients needed higher maintenance steroid doses, required treatment Difluprednate with azathioprine more often, and experienced poorer outcomes overall in comparison with the other subtypes [9]. In this paper, we present a patient that exhibited progressive dyspnea as his main and only symptom prior to presenting to the emergency department (ED). == Case Statement == The CARE Checklist has been completed by the authors for this case statement, attached as online supplementary material (for all those online suppl. material, seehttps://doi.org/10.1159/000540916). A 51-year-old male with a history of gastroesophageal reflux disease and generalized anxiety disorder on paroxetine offered to the ED complaining of acute dyspnea that had been progressing over the past week. History goes back to 2 months prior when the patient started going through worsening dyspnea in the beginning upon exertion which developed into dyspnea that limited his Difluprednate ability to speak to getting a few words in between breaths. To note, the patient had been complaining of a progressive weight decline of almost 20 kg over the past year; however, gastroenterology and endocrinology visits were unfavorable. In the ED, the patients oxygen saturation reached 63% prompting administration of bilevel positive airway pressure (BiPAP). The patient continued to deteriorate, Difluprednate becoming minimally responsive to painful stimuli, and was thus intubated. Arterial blood gas showed severe respiratory acidosis. The patient was then transferred to the intensive care unit around the eve of day 1 pending further workup. CT brain ruled out hemorrhagic stroke, and CT stomach was not significant. Infectious workup showed bilateral lower lobe pneumonia on CT scan; however, the infectious disease team remained unconvinced of this as the cause of his respiratory failure, since the patient was afebrile, experienced no history of cough, experienced no hypotension, and his laboratories and inflammatory markers were unimpressive. He was started on piperacillin-tazobactam 4.5 g for 7 days and was given 1 g vancomycin and amikacin. The neurology team was consulted and examined the patient thoroughly. On physical exam,.
