Unmasking Guillain-Barré Syndrome: revelations from research at NIMHANS

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The current outbreak of Guillain–Barré syndrome (GBS), a uncommon neurological situation in Pune, Maharashtra, has led to panic among the many public in Maharashtra and different elements of India. Whereas GBS-like problems have been recognized in scientific literature for hundreds of years, this eponymous time period was first coined in 1916. Outbreaks of GBS of the magnitude reported from Pune, have been moderately unusual. A couple of examples nevertheless, have been larger incidences of GBS reported in French Polynesia in 2013-2014 and Latin America in addition to the Caribbean in 2015-2016 on account of outbreaks of Zika virus an infection. An unusually giant outbreak of GBS was reported in Peru between Could 20 and July 27, 2019, the place over a interval of two months and 7 days, 683 instances of GBS had been reported. 

The primary case of GBS in Pune was reported on January 9, 2025 and as on January 28, 111 instances and one demise have been reported. The incidence of GBS in Pune is kind of alarming given the truth that the outbreaks of GBS haven’t crossed a three-digit quantity up to now besides throughout the above two cases. 

What’s GBS? 

GBS is a neurological dysfunction that causes irregular tingling, numbness, ache and muscle weak spot. Some sufferers might expertise problem in speaking, closing eyes tightly, chewing, swallowing, and generally even respiration, in addition to irregular coronary heart price and fluctuating blood stress. The annual international incidence of GBS is roughly 1-2 per 100,000 person-years. The lifetime threat of creating GBS is lower than 1%. The analysis of GBS is established via a sequence of neurological, electrophysiological and biochemical (blood and cerebrospinal fluid) analyses. GBS progresses quickly and many of the sufferers attain the utmost incapacity inside two weeks. The present literature means that about 20% of GBS sufferers develop weak spot of respiratory muscle groups and require mechanical air flow, whereas about 3-7% sufferers succumb even with the perfect obtainable medical care. Thus, GBS is a medical emergency and may be doubtlessly deadly, albeit in a small proportion of the affected sufferers. Whereas GBS is a one-time sickness, recurrences do happen very hardly ever in about 2-5% of sufferers. 

Threat components of GBS

GBS has been categorised as a post-infectious immune-mediated dysfunction the place the immune cells and molecules assault and injury the protecting protecting of the peripheral nerves. A number of research the world over recommend an affiliation between an antecedent an infection and the danger of creating GBS in upto 70% of the instances. The signs of GBS normally start to seem after just a few days or even weeks of a gastrointestinal or respiratory an infection. It has been reported that numerous components apart from infections, resembling current surgical procedure, sure vaccinations, immunocompromised states, and so on. may additionally function triggering components. 

Among the many a number of micro-organisms, Campylobacter jejuni has been reported to be the most predominant infectious set off of GBS. Different bacterial and viral pathogens which can be related to GBS embody Cytomegalovirus, Dengue virus, Influenza virus, Japanese encephalitis virus, Chikungunya virus, Mycoplasma pneumoniae, Epstein-Barr virus, and Zika virus. Although a majority of the research recommend that 30-50% of the sufferers with GBS have antecedent Campylobacter jejuni an infection, decrease percentages of Campylobacter jejuni an infection have additionally been reported. For example, in the course of the giant GBS outbreak in Peru, solely 5.2% of the instances had Campylobacter jejuni an infection. It’s noteworthy that roughly just one in 1,000 individuals contaminated with Campylobacter jejuni go on todevelop GBS. This implies that an infection alone will not be satisfactory to induce the event of GBS. Not all sufferers with an infection develop GBS, and equally not all sufferers with GBS have a previous an infection. 

We carried out a research on the influence of antecedent infections resembling Campylobacter jejuni, Influenza virus, Dengue virus, Japanese encephalitis virus and Zika virus in 150 sufferers with GBS between July 2014 and June 2019 at NIMHANS, Bengaluru. In our cohort, 79.3% confirmed proof of prior infections, of which 32% examined optimistic for Campylobacter jejuni. Curiously, in our research, co-infection by a number of pathogens was extra widespread, seen in about 65%. This suggests a posh interaction amongst numerous micro organism and viruses that confer the danger of creating GBS. 

Etiopathology of GBS

GBS develops on account of aberrant immune features, both on account of an autoimmune response or an exaggerated inflammatory response. Infectious threat organisms can mount an autoimmune response via ‘molecular mimicry’ the place the protecting antibodies generated towards the cell floor antigens of the micro organism bind to the functionally related molecules on the floor of nerve cells, as a result of similarity between the molecules of micro organism and nerve cells. The infectious threat organisms also can result in inflammatory responses by activating the immune cells. Our analysis over the previous 10 years on the immunobiology of GBS at NIMHANS, Bengaluru, suggests the involvement of a number of forms of immune cells resembling T lymphocytes, particularly the Th1 and Th17 lineages and a novel mucosal related invariant T (MAIT) cell in GBS.

Additional, inflammatory cytokines of Th1 and Th17 pathway are additionally concerned in GBS. Probably the most notable discovering in GBS was the alteration of an alarmin, a molecule that alerts immune system to the presence of an an infection or damage. Additional, our on-going analysis suggests a pivotal function of intestine microbiota in perturbing the immune homeostasis and elevating the danger of GBS. Although a exact mechanistic foundation of GBS is but to be established, we advise a mixed, concerted and additive results of a number of cells and molecules inside the immune system. 

Who’s affected?

GBS impacts individuals of all age teams, nevertheless, the incidence will increase with age. GBS is extra widespread in males as in comparison with girls, the exact cause for the male preponderance will not be recognized. There’s a clear lack of knowledge on the epidemiology and burden of GBS at a world stage, and it’s recommended that in all probability, individuals residing in low-income and middle-income nations are extra susceptible owing to poor hygiene and excessive publicity to infections. Arboviral infections resembling Dengue, Chikungunya, Zika, and so on. are extra widespread in tropical and sub-tropical areas. Due to this fact, individuals residing in these areas are probably extra vulnerable to creating GBS. 

Remedy

There are solely two established remedy choices for GBS – plasma change and intravenous immunoglobulin (IVIg). Plasma change entails filtering out the dangerous antibodies from the physique. Alternatively, IVIg acts by neutralising the deleterious results of the immune system on the peripheral nerves. Each remedy choices are thought-about to be equally efficient. Sufferers with extreme GBS require further ICU take care of mechanical air flow and managing different inter-current problems, on a case-to-case foundation. Although a majority of the sufferers get better absolutely from GBS with remedy and rehabilitation, a small quantity are left with incapacity for months to years. 

Preventive measures 

GBS will not be a contagious illness. However, individuals ought to keep away from consuming undercooked poultry, meat and milk. It’s advisable to drink handled/ purified and/or boiled water. Meals which can be vulnerable to bacterial development on account of excessive moisture content material needs to be cooked completely earlier than consumption. One wants to take care of correct and satisfactory private in addition to meals hygiene. These measures will cut back respiratory and gastrointestinal infections and should mitigate the danger related to infection-induced GBS.

(Dr. Monojit Debnath is a Professor within the Division of Human Genetics, NIMHANS, Bengaluru. He has greater than 12 years of analysis expertise within the discipline of immune-genetics of GBS monozeet@gmail.com)

(Dr. Madhu Nagappa is a Professor within the Division of Neurology, NIMHANS, Bengaluru. She has greater than 13years of medical expertise in caring for GBS sufferers madhu_nagappa@yahoo.co.in)

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