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Guillain–Barré Syndrome Update Webinar
The much-awaited Neuromuscular Series: Guillain-Barre Syndrome Update Webinar was finally conducted on Wednesday, December 9, 2020, under the sponsorship of Biotest AG, a global company that supplies plasma protein products and biotherapeutic drugs, and managed by Medetarian Conferences Organizing (MCO), the leading healthcare events management in the UAE. With 730+ attendees, the one-hour and 30-minutes virtual event was a remarkable feat in its entirety, traversing various professional participants not only in the United Arab Emirates but all across the globe, sharing a commonality of interest in this particular field of study.
By a popular medical definition, Guillain–Barré Syndrome is a rare disorder in which your body’s immune system attacks your nerves leading to a whole-body paralysis. It has been said that the causes for this disease are still unknown, ergo, considered under the clinical diagnosis with the medication of intravenous immunoglobulin (IVIG), the most commonly used treatment for patients with GBS.
Prominent Webinar Chairman Dr. Abubaker Al Madani, Senior Neurologist of United Medical Center, Dubai, UAE has expressed his gratitude to all participating delegates for joining the GBS Syndrome Update Webinar. He officially commenced the agenda by sharing a very brief biographical introduction to our line of esteemed speakers.
Sharing her expertise specifically on the topic of “Update in Diagnostic Criteria of GBS”, Dr. Aysha Alshareef, Neuromuscular Consultant and a respected Associate Professor Neurology of King Abdulaziz University, Jeddah, KSA, has discussed a condensed historical background in which in 1859 Landry, the first death associated with this syndrome was reported. She further elaborated her research by presenting a clinical picture in which GBS is recognized as a diverse disorder divided into several patterns based on the predominant mode of fiber injury (demyelinating vs. axonal). And most recently, the Zika virus epidemics in French Polynesia in 2013 and Latin America and the Caribbean in 2015–2016 were linked to an increase in individuals being diagnosed with GBS.
According to a Kolb report in 2018, Guillain-Barré syndrome is increasingly reported in cancer patients treated with immune checkpoint inhibitors and represents the second most common immune checkpoint inhibitors-related neuromuscular complication, after myasthenia, occurring in about 0.1% of patients. It was also studied that Acute Motor Axonal Neuropathy (AMAN) presents as an acute, flaccid, symmetrical ascending paralysis with increased cerebral spinal fluid protein suggests Guillain-Barré syndrome.
In the Electrodiagnostic update, Dr. Aysha also explained that electrophysiology plays a determinant role in GBS diagnosis, classification of the subtypes, and in establishing the prognosis. Various clinical pieces of evidence are recently published in the hopes to provide updates in GBS diagnosis. One example pertains to a prospective evaluation of MRI lumbosacral nerve root enhancement in acute GBS syndrome in which as quote, “Twenty of 24 patients had cauda equina nerve root enhancement, which was mild in 6 and prominent in 14; GBS disability was higher in patients with prominent enhancement, and significantly fewer patients with prominent nerve root enhancement could walk independently by 2 months.”
In a nutshell, the diagnosis of GBS is based on clinical history and examination, supported by ancillary investigations such as CSF examination and electrodiagnostic studies. GBS variants and subtypes have variable atypical presentation which requires a high degree of suspicion.
Dr. Deeb Maxwell Kayed, Consultant Neurologist & Clinical Neurophysiologist of IRAC/ MCIT / AHD – Dubai, UAE, has deliberated a comprehensive “GBS: Therapy Update” in which he addressed the importance of monitoring the respiratory function by considering as quote, “Routine measurement of respiratory function is advised, as not all patients with respiratory insufficiency will have clinical signs of dyspnea.”
He also argues that Respiratory measurements can include:
- Use of accessory respiratory muscles
- Counting during expiration of one full-capacity inspiratory breath (a single breath count of ≤19 predicts a requirement for mechanical ventilation)
- Vital capacity and maximum inspiratory and expiratory pressure
As an essential side note, Dr. Deeb advised that clinicians should consider using the ‘20/30/40 rule’, whereby the patient is deemed at risk of respiratory failure if the vital capacity is <20ml/kg, the maximum inspiratory pressure is <30cmH2O or the maximum expiratory pressure is <40cmH2O. Based on a clinical evaluation, up to two-thirds of the deaths of patients with GBS occur during the recovery phase and are mostly caused by cardiovascular and respiratory dysfunction, thus, through constant monitoring, one must remain alert during this phase and monitor the patient for potential arrhythmias, blood pressure shifts, or respiratory distress caused by mucus plugs. The call to treatment for patients with GBS must include Immunomodulatory therapy and should be started if patients are unable to walk independently for 10 m especially if these patients display rapidly progressive weakness or other severe symptoms such as autonomic dysfunction, bulbar failure, or respiratory insufficiency.
Dr. Deeb also mused that clinical trials have demonstrated a treatment effect for intravenous immunoglobulins (IVIg) when started within 2 weeks of the onset of weakness and for plasma exchange when started within 4 weeks. As a response to therapy, it was found out that about 40% of patients treated with PE / IVIg do NOT improve in the first 4 weeks following treatment in which Dr. Deeb suggests considering repeating the treatment or changing to an alternative treatment; however, at present no evidence exists that this approach will improve the outcome.
According to Walgaard, C. et al. Second IVIg course in Guillain-Barré syndrome, the efficacy of complement inhibitors, IgG-cleaving enzymes, and the second course of IVIg is being investigated for upcoming therapy trials. Other treatment factors include Rehabilitation: PT/OT. The intensity of exercise must be monitored as overwork can cause fatigue.
With these updates on therapy, GBS patients are very much likely to experience depression. Why? Citing Dr. Deeb’s presented analysis on psychological factors, a patient’s rapid loss of physical function, often in a previously healthy lifestyle (sic), can be severely traumatic and may cause anxiety &/or depression. Thus, as a preventive measure, early recognition and management of psychological distress should be important in patients with GBS, especially as mental status can influence physical recovery and vice versa; providing accurate information to patients on the relatively good chance of recovery and low recurrence risk can help reduce their fear, and connecting patients with others who have had GBS can also help guide them through the rehabilitation process. *The GBS/CIDP Foundation International — the international patient association for GBS — and other national organizations can help establish these networks.*
During these uncertain times of COVID-19 pandemic, patients with GBS are also considered at risk, thus, it’s fitting to note that many vaccines carry a warning about GBS. However prior GBS is not a strict contraindication for vaccination and no definite causal relation has been established, except for Rabies vaccine (prepared from infected brain tissue). Further, as discussed thoroughly by Dr. Deeb, other studies have called the GBS/vaccine link into question, finding no evidence of an increased risk of GBS after the seasonal influenza vaccine or after the 2009 H1N1 mass vaccination program. A review of all postvaccination cases of GBS from 1990-2005 did not reveal any increase in mortality with postvaccination cases of GBS compared with cases resulting from other causes.
Complementing this very interesting webinar, a live Q and A session have kept the virtual floor exciting drawing a generous amount of inquiries all related to GBS. This event managed by MCO is CME accredited and is a certified member of the International Congress and Convention Association (ICCA).