The Neuromuscular Challenge in the Intensive Care Unit
Managing patients with acute neuromuscular disorders (NMD) in the intensive care unit (ICU) represents one of the most complex challenges in respiratory medicine. Unlike patients with primary pulmonary diseases, individuals with Guillain-Barré syndrome (GBS) or myasthenia gravis (MG) often possess healthy lung parenchyma but suffer from a failure of the ‘respiratory pump.’ This failure, driven by peripheral nerve demyelination or neuromuscular junction blockade, leads to a unique and often unpredictable weaning process. Until recently, large-scale data detailing the specific characteristics of ventilator weaning in these populations were limited, leaving clinicians to rely on small cohorts or institutional protocols.
A new nationwide multicenter study published in Intensive Care Medicine (2025) provides much-needed clarity. By analyzing nearly 900 patients over a ten-year period, researchers have mapped the distinct trajectories of GBS and MG patients, highlighting significant disparities in weaning duration, tracheostomy rates, and the persistent threat of extubation failure.
Understanding the Clinical Context: GBS vs. Myasthenia Gravis
While both GBS and MG can lead to hypercapnic respiratory failure requiring mechanical ventilation, their underlying pathophysiologies dictate different clinical courses. Guillain-Barré syndrome is an acute inflammatory polyneuropathy that often follows a monophasic but protracted course. Recovery depends on the slow process of peripheral nerve remyelination or axonal regeneration. In contrast, Myasthenia Gravis is a chronic autoimmune disorder affecting the postsynaptic membrane of the neuromuscular junction. Myasthenic crises are often triggered by infections or medication changes and can frequently be reversed more rapidly with targeted therapies like plasmapheresis or intravenous immunoglobulin (IVIG).
These biological differences suggest that the ‘readiness’ for weaning would differ significantly between the two groups. The study sought to quantify these differences to better inform ICU resource planning and bedside decision-making.
Study Design and Methodology: A Decade of French Multicenter Data
The study utilized a retrospective cohort design, encompassing 886 patients admitted to 47 different ICUs across France between January 2014 and December 2023. Inclusion criteria were strict: patients had to be intubated for at least 48 hours specifically due to complications of GBS or MG.
Of the total cohort, 513 patients (58%) were diagnosed with GBS, and 373 (42%) had MG. The primary endpoint was the proportion of patients experiencing ‘prolonged weaning,’ defined as a weaning process lasting at least seven days from the first spontaneous breathing trial or reduction in pressure support to successful extubation. Secondary endpoints included the rate of tracheostomy, reintubation rates within 48 to 72 hours, and overall ICU mortality.
Key Findings: Disparities in Weaning and Tracheostomy
The results revealed a stark contrast in the weaning experiences of patients with GBS versus those with MG.
Prolonged Weaning and Extubation Strategy
Patients with GBS were significantly more likely to experience prolonged weaning compared to those with MG (64% vs. 35%, p < 0.001). This finding aligns with the biological reality of nerve repair versus neuromuscular junction stabilization. Furthermore, the approach to extubation differed wildly. An extubation attempt was performed in only 46% of GBS patients, whereas 88% of MG patients were given an attempt at liberation from the ventilator (p < 0.001). This suggests that clinicians often anticipate weaning failure in GBS and move directly toward alternative strategies, such as tracheostomy, without an intervening extubation trial.
Tracheostomy and Resource Utilization
The most dramatic difference was seen in the rates of tracheostomy. Over half of the GBS patients (57%) underwent the procedure, compared to only 17% of patients with MG (p < 0.001). This reflects the clinical consensus that GBS-associated respiratory failure is likely to be long-term. As a result, GBS patients required a significantly longer duration of mechanical ventilation and longer ICU stays, representing a higher burden on healthcare resources.
The Reintubation Paradox: High Risk Despite Low Mortality
One of the most clinically relevant findings of the study was the high rate of reintubation. Among patients who were given an extubation attempt, 26% of GBS patients and 29% of MG patients failed and required reintubation. These rates are substantially higher than the 10-15% typically seen in a general medical-surgical ICU population.
The high failure rate in MG (nearly 30%) is particularly noteworthy. It suggests that even when clinicians believe a patient is ready—perhaps because peripheral muscle strength has improved—bulbar weakness or ‘myasthenic fatigue’ can lead to rapid post-extubation failure. This underscores the need for more specialized weaning parameters in NMD patients, such as measuring maximal expiratory pressure (MEP) or assessing swallow function, rather than relying solely on traditional weaning indices like the Rapid Shallow Breathing Index (RSBI).
Despite the high rates of prolonged ventilation and reintubation, the mortality rates remained impressively low and nearly identical between groups: 9.4% for GBS and 9.7% for MG. This indicates that while the path to recovery is long and fraught with complications, the vast majority of these patients can survive the acute phase if provided with high-quality supportive care.
Expert Commentary: Clinical Implications for the Multidisciplinary Team
This study provides a robust evidentiary base for what many neuro-intensivists have suspected: GBS and MG require vastly different respiratory management strategies. For the GBS patient, the high probability of prolonged weaning (64%) and the high frequency of tracheostomy (57%) suggest that early discussion of tracheostomy may be appropriate to improve patient comfort, facilitate mobilization, and reduce the risks associated with long-term endotracheal intubation.
For the MG patient, the high reintubation rate (29%) serves as a cautionary tale. It suggests that we may be over-optimistic in our weaning trials. The intermittent nature of myasthenic weakness means that a successful 30-minute spontaneous breathing trial may not accurately predict the patient’s ability to maintain an airway over 24 or 48 hours. Clinicians should consider a lower threshold for using non-invasive ventilation (NIV) or high-flow nasal oxygen (HFNO) immediately post-extubation as a ‘bridge’ in this population.
Furthermore, the study highlights the ‘neuromuscular weaning paradox’: patients who look clinically stable may still have significant bulbar dysfunction, leading to aspiration and subsequent reintubation. Future research should focus on whether specific ‘neuro-weaning’ protocols—incorporating cough peak flow and formal bedside swallow evaluations—can reduce these high failure rates.
Conclusion: Refining the Approach to Neuro-Respiratory Failure
The study by Thille et al. (2025) provides a landmark reference for the management of GBS and MG in the ICU. The key takeaway for clinicians is the high likelihood of a protracted course for GBS and a high risk of extubation failure for both conditions. While mortality is low, the morbidity associated with prolonged mechanical ventilation is significant. By recognizing these patterns early, multidisciplinary teams can better prepare patients and families for the ‘marathon’ of weaning, optimize the timing of tracheostomy, and exercise extreme vigilance during the critical post-extubation period.
References
1. Thille AW, Bayon C, Gauvrit M, et al. Characteristics of ventilator weaning in patients intubated for Guillain-Barré syndrome or myasthenia gravis: a nationwide multicenter study. Intensive Care Med. 2025;51(11):2054-2064. doi:10.1007/s00134-025-08159-7.
2. Rabinstein AA. Noninvasive ventilation in myasthenic crisis: appropriate but requires vigilance. Muscle Nerve. 2020;61(3):273-274.
3. Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory failure in Guillain-Barré syndrome: a risk-indicator score. Lancet Neurol. 2010;9(11):1051-1058.
