Guidelines for Neuroprognostication in Adults with Guillain–Barré Syndrome

Abstract

Background Guillain–Barré syndrome (GBS) often carries a favorable prognosis. Of adult patients with GBS, 10–30% require mechanical ventilation during the acute phase of the disease. After the acute phase, the focus shifts to restoration of motor strength, ambulation, and neurological function, with variable speed and degree of recovery. The objective of these guidelines is to provide recommendations on the reliability of select clinical predictors that serve as the basis of neuroprognostication and provide guidance to clinicians counseling adult patients with GBS and/or their surrogates. Methods A narrative systematic review was completed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Candidate predictors, including clinical variables and prediction models, were selected based on clinical relevance and presence of appropriate body of evidence. The Population/Intervention/Comparator/Outcome/Time frame/Setting (PICOTS) question was framed as follows: “When counseling patients or surrogates of critically ill patients with Guillain–Barré syndrome, should [predictor, with time of assessment if appropriate] be considered a reliable predictor of [outcome, with time frame of assessment]?” Additional full-text screening criteria were used to exclude small and lower quality studies. Following construction of an evidence profile and summary of findings, recommendations were based on four GRADE criteria: quality of evidence, balance of desirable and undesirable consequences, values and preferences, and resource use. In addition, good practice recommendations addressed essential principles of neuroprognostication that could not be framed in PICOTS format. Results Eight candidate clinical variables and six prediction models were selected. A total of 45 articles met our eligibility criteria to guide recommendations. We recommend bulbar weakness (the degree of motor weakness at disease nadir) and the Erasmus GBS Respiratory Insufficiency Score as moderately reliable for prediction of the need for mechanical ventilation. The Erasmus GBS Outcome Score (EGOS) and modified EGOS were identified as moderately reliable predictors of independent ambulation at 3 months and beyond. Good practice recommendations include consideration of both acute and recovery phases of the disease during prognostication, discussion of the possible need for mechanical ventilation and enteral nutrition during counseling, and consideration of the complete clinical condition as opposed to a single variable during prognostication. Conclusions These guidelines provide recommendations on the reliability of predictors of the need for mechanical ventilation, poor functional outcome, and independent ambulation following GBS in the context of counseling patients and/or surrogates and suggest broad principles of neuroprognostication. Few predictors were considered moderately reliable based on the available body of evidence, and higher quality data are needed.

Neuromuscular Emergencies

“…30,31 In a guideline published in 2023, this score was graded as a moderately reliable predictor of the need for intubation that can be used in conjunction with grading of bulbar weakness. 32 Other predictors of respiratory failure include short time from symptom onset to hospital admission (less than 7 days) and severe muscle weakness at hospital admission. 29 Patients with GBS are also at risk of dysphagia, corneal abrasions, and autonomic complications, such as urinary retention, ileus, and blood pressure and heart rate lability; these occur in up to 42% of patients, with constipation being the most frequent manifestation.…”

“…33 Strategies to treat dysphagia should be discussed with the patient and surrogate early in the course. 32 In patients with significant facial weakness, attention should be given to proper eye care, with ocular lubricant and taping of the eyelids during rest.…”

“…The Erasmus GBS Respiratory Insufficiency Score is another validated system to calculate the probability that a patient with GBS will require mechanical ventilation within 1 week of the assessment ( table 10-1 ). 30,31 In a guideline published in 2023, this score was graded as a moderately reliable predictor of the need for intubation that can be used in conjunction with grading of bulbar weakness 32 . Other predictors of respiratory failure include short time from symptom onset to hospital admission (less than 7 days) and severe muscle weakness at hospital admission 29 .…”

Prognostication in Neurocritical Care

“…It would also provide a grounding framework for less experienced clinicians. 67 Because of the profound impact of neuroprognostication on families who face high-stakes decisions severe acute brain injuries, several guidelines and expert consensus documents have begun to offer recommendations on neuroprognostication (TABLE 12-3 [68][69][70][71][72][73] ). A collaborative practice guideline on disorders of consciousness, published by the American Academy of Neurology; American Congress of Rehabilitation Medicine; and the National Institute on Disability, Independent Living, and Rehabilitation Research in 2018, provided recommendations regarding what clinicians should avoid when making prognostications for patients with disorders of consciousness.…”

“…After this gap analysis, an international multidisciplinary author panel (including physicians from neurocritical care, neurosurgery, ethics, and neuropalliative care; nurses; pharmacists; and family or patient representatives) from the same societies has embarked on publishing evidencebased guidelines on the neuroprognostication of the eight most common diseases in neurocritical care: ischemic encephalopathy after cardiac arrest, ICH, acute ischemic stroke, subarachnoid hemorrhage, TBI, traumatic spinal cord injury, status epilepticus, and Guillain-Barré syndrome. At the time of the writing of this article, the guidelines for neuroprognostication after cardiac arrest, Guillain-Barré syndrome, traumatic spinal cord injury, ICH, and traumatic brain injury have been published, [69][70][71][72][73] and others are under review, with the anticipation that all of them will be published by late 2024 or early 2025. These neuroprognostication guidelines are unique because of their inclusion of the most extensive and up-to-date systematic review conducted thus far on the quality of the existing literature in eight diseases.…”