Myasthenic Crisis Treatment Selection

Admit a patient with impending crisis to the hospital for close serial assessment of respiratory and bulbar function, with immediate capability to transfer to intensive care if deterioration occurs. Admit manifest crisis to an ICU or step-down unit able to manage respiratory failure and bulbar dysfunction. NeurologyNeurologyInternational consensus guidance for management of myasthenia gravis

Define the immediate airway problem clinically: respiratory insufficiency requiring mechanical ventilation or intubation for airway protection constitutes myasthenic crisis. Do not defer intubation in progressive ventilatory failure, ineffective airway protection, or rapidly worsening bulbar weakness while waiting for immunotherapy to work. JAMA+1JAMAPredictors of Extubation Failure in Myasthenic CrisisWileyMyasthenic crisis

At presentation, actively identify and correct reversible precipitants while initiating rescue treatment. Crisis care priorities include airway, respiratory, and circulatory support; confirmation of MG and crisis; evaluation and treatment of acute respiratory failure; elimination of triggers; immunomodulatory treatment; and prevention or treatment of complications. WileyWileyMyasthenic crisis

PLEX and IVIG are both appropriate short-term treatments for life-threatening respiratory insufficiency or dysphagia, significant bulbar dysfunction before surgery, need for rapid response, inadequate response to other treatments, and selected situations before corticosteroid initiation. NeurologyNeurologyInternational consensus guidance for management of myasthenia gravis PLEX has strong guideline support and typically reaches maximal efficacy after three to five treatment sessions. BMJBMJEfgartigimod following plasma exchange in the treatment of subjects ...

When physiologic urgency is extreme, PLEX is commonly favored if it can be delivered safely. Comparative literature describes PLEX as producing faster and more noticeable improvement in some studies, and a retrospective crisis cohort found PLEX superior to IVIG for 7-day severity, 2-week ventilation status, and 1-month functional outcome; these data are not definitive enough to establish universal superiority. Wiley+2WileyMyasthenic crisisWolters KluwerMyasthenic crisis : Muscle & Nerve - OvidNeurologyClinical Reasoning: Therapeutic considerations in myasthenic crisis due to COVID-19 infection

Choose IVIG when the logistics, vascular access requirements, or hemodynamic demands of PLEX are unfavorable, or when IVIG is the more deliverable option. Randomized studies in MG exacerbation found IVIG as effective as PLEX at approximately 14 to 15 days, and an earlier trial found no difference in myasthenic muscle score at day 15 among PLEX and IVIG regimens. Nature+1NatureIntravenous immunoglobulins may prevent prednisone-exacerbation in myasthenia gravis | Scientific ReportsWileyMyasthenic crisis In COVID-19-associated crisis, IVIG may be preferable because PLEX removes circulating protective as well as pathogenic antibodies, although the clinical consequence of lowering antiviral antibody titers remains uncertain. NeurologyNeurologyClinical Reasoning: Therapeutic considerations in myasthenic crisis due to COVID-19 infection

Do not interpret failure of one modality as a reason to abandon rescue therapy. If an exacerbation or crisis was not attributable to primary failure of the initial modality, retreatment may be reasonable; after exposure to both therapies, clinicians may retry the modality associated with the better prior response when there is a plausible benefit. nice org uknice org uk3 Committee discussion | Rozanolixizumab for treating antibody-positive generalised myasthenia gravis | Guidance | NICE

Corticosteroids or other immunosuppressive agents are commonly started at the time of PLEX or IVIG to establish a sustained response. Because corticosteroids can transiently worsen myasthenic weakness, it may be safer to wait several days for PLEX or IVIG benefit before initiating corticosteroids when respiratory or bulbar reserve is limited. NeurologyNeurologyInternational consensus guidance for management of myasthenia gravis

Corticosteroids remain an important component of crisis treatment in most patients, but they do not replace PLEX or IVIG for acute rescue because clinical response requires weeks and maximal benefit is delayed. Wolters Kluwer+1Wolters KluwerNeuromuscular Disorders in the Intensive Care Unit - ContinuumNeurologyClinical Reasoning: Therapeutic considerations in myasthenic crisis due to COVID-19 infection In a patient who is already unstable, use airway planning and rescue immunotherapy to bridge this delay rather than relying on steroid escalation alone. Neurology+1NeurologyInternational consensus guidance for management of myasthenia gravisNeurologyClinical Reasoning: Therapeutic considerations in myasthenic crisis due to COVID-19 infection

Reassess pyridostigmine and other cholinesterase inhibitors during crisis. Excess cholinesterase inhibition is an uncommon but possible contributor to deterioration, and increased airway secretions can worsen respiratory management. NeurologyNeurologyInternational consensus guidance for management of myasthenia gravis In the intubated patient with secretion burden, this tradeoff may favor withholding or reducing the agent until airway control and respiratory mechanics improve.

Mechanical ventilation is indicated for neuromuscular respiratory failure or when intubation is required for airway protection. JAMAJAMAPredictors of Extubation Failure in Myasthenic Crisis Continue aggressive respiratory support while PLEX or IVIG takes effect; intubation itself should not be viewed as rescue-treatment failure because crisis is defined by the need for ventilatory support or airway protection. JAMA+1JAMAPredictors of Extubation Failure in Myasthenic CrisisNeurologyInternational consensus guidance for management of myasthenia gravis

Before extubation, integrate bedside respiratory performance with pulmonary complication status. In a study of myasthenic crisis, male sex, prior crisis, atelectasis, and intubation longer than 10 days were associated with extubation failure. Among patients evaluated for reintubation, lower pH, lower forced vital capacity at extubation, atelectasis, and post-extubation BiPAP use predicted reintubation. JAMAJAMAPredictors of Extubation Failure in Myasthenic Crisis

Treat atelectasis and other pulmonary complications before a marginal extubation attempt whenever possible. Noninvasive ventilation may help avoid reintubation in selected patients, but the association between post-extubation BiPAP use and reintubation identifies a high-risk group rather than proof that BiPAP causes failure. JAMA+1JAMAPredictors of Extubation Failure in Myasthenic CrisisWileyMyasthenic crisis - Claytor - 2023 - Muscle & Nerve

Immune checkpoint inhibitor-associated MG can progress rapidly irrespective of initial apparent severity. In a systematic review and single-center series, 14 of 38 patients initially treated with corticosteroids alone progressed to respiratory failure, including four initially presenting with ocular symptoms; 18 of 19 patients receiving IVIG or PLEX first line improved. BMJBMJImmune checkpoint inhibitor related myasthenia gravis: single center experience and systematic review of the literature | Journal for ImmunoTherapy of Cancer Hospitalize these patients and use early IVIG or PLEX rather than waiting for steroid response when MG is suspected clinically. BMJBMJImmune checkpoint inhibitor related myasthenia gravis: single center experience and systematic review of the literature | Journal for ImmunoTherapy of Cancer

For refractory crisis after standard PLEX or IVIG, do not substitute case-report evidence for established rescue pathways. Eculizumab has been reported in refractory crisis, including successful extubation after failed PLEX and high-dose prednisone, but the evidence base consists of a case report and 19 additional reported cases. ScienceDirectScienceDirectEmergent role of complement inhibitors in myasthenic crisis: Understanding why, when and how - ScienceDirect If considered, recognize the need for prompt meningococcal vaccination and antibiotic prophylaxis described in that report; this is an exceptional rescue decision rather than routine first-line care. ScienceDirectScienceDirectEmergent role of complement inhibitors in myasthenic crisis: Understanding why, when and how - ScienceDirect

After acute stabilization, transition to long-term MG management rather than repeating rescue therapy indefinitely. The broader treatment armamentarium includes corticosteroids, nonsteroidal immunosuppressive therapy, FcRn inhibitors, complement inhibitors, B-cell-directed therapy, and thymectomy; thymectomy is required for thymoma and can benefit AChR-antibody-positive generalized nonthymomatous MG. BMJ+1BMJEfgartigimod following plasma exchange in the treatment of subjects ...BMJMyasthenia gravis - Symptoms, diagnosis and treatment | BMJ Best Practice US