A Paralyzing Attack on Self: Guillain-Barré Syndrome
“An individual affected by this syndrome will experience progressive ascending motor weakness of the limbs, ultimately leading to paralysis of the respiratory system and eventually, the entire body.”
Brief Summary of Disease
Guillain-Barré Syndrome (GBS) is a rare neurological autoimmune disorder.2 An individual affected by this syndrome will experience progressive ascending motor weakness of the limbs, ultimately leading to paralysis of the respiratory system, and eventually, the entire body. The incidence of Guillain-Barré syndrome typically increases with age, with a prevalence of 1 in 100,000 people developing the disorder.2 While more commonly affecting elderly and male populations, all age groups and genders are susceptible to the onset of the syndrome. This disorder has grown to become the most common acute paralytic disease in Western countries.2
Etiology & Pathology
Guillain-Barré syndrome is an autoimmune disease often preceded by an infection or vaccine administration, due to an aberrant immune response to an infectious pathogen.3 This disease causes demyelination of the peripheral nervous system (PNS) and is characterized by acute symmetrical ascending motor weakness and mild sensory abnormalities.4
Campylobacter jejuni is the most commonly identified bacteria that is associated with triggering the onset of Guillain-Barré syndrome.5 In fact, two-thirds of GBS patients report an antecedent C. jejuni infection.4 This bacterium, a commensal organism of the chicken gut, is a leading cause of gastroenteritis in humans. 4 Most infections of this bacteria are acquired due to consumption of undercooked poultry, unpasteurized milk, and contaminated water.4 It is interesting to note that several studies performing experimental infections of C. jejuni on chickens have observed severe paralysis resembling a neuropathy.4 Unfortunately, despite extensive research on C. jejuni and its effects on GBS, the pathogenesis of this disease has not been completely understood.4
Scientists suggest that a molecular mimicry exists between Add lipopolysaccharide structures on the cell envelope of C. jejuni and ganglioside-like epitopes on human nerves.4 The presence of foreign pathogens in the body stimulates an immune system response that subsequently produces a cross-reactive reaction, driving a cascade of immune-mediated inflammatory responses on self-peripheral nerves. Specific immune recognition involving T-cells, monocytes and pro-inflammatory cytokines is primarily responsible for causing demyelination of nerve cells in the host peripheral nervous system.4 One week following this immune system stimulation, there is an acute progression of limb weakness with sensory and cranial nerves being primarily affected due to the blocking of nerve conduction – the rate at which electrical impulses flow through the nerve.5
Symptoms & Diagnosis
Symptoms of this disorder often peak within four weeks of infection.5 Patients demonstrate flaccid weakness and hyporeflexia, a muscle tone reduction and loss of reflex respectively, which leads to paralysis. The neuromuscular compromise that occurs causes respiratory failure in most patients, requiring mechanical ventilation.2 Due to peripheral nerve demyelination, additional autonomic symptoms can arise such as tachycardia (a decrease in blood pressure), anhidrosis (an inability to sweat normally), and urinary retention.2,6 These symptoms are often a significant source of mortality in patients with GBS. Some patients progress rapidly and can become ventilator-dependent within hours, while others have very mild progression of symptoms for several weeks.7 In extreme cases, patients can be completely paralyzed and lose the ability to move. These symptoms in addition to abnormal laboratory tests indicating elevated protein levels and abnormal nerve conduction results can be attributed to a diagnosis of GBS.2,7
“Moreover, as paralysis is common in GBS patients, individualized treatment plans can involve rehabilitation to strengthen muscles and regain movement.”
Prognosis
Currently, no cure exists for Guillain-Barré syndrome. However, treatments can be implemented to manage symptoms and reduce the severity of the illness. Treatment is essential for GBS patients due to the risks associated with respiratory failure and autonomic function, with potential implications for cardiovascular health. Due to neuromuscular deficits and respiratory failure, supportive care through mechanical ventilation is of utmost importance for many patients.6 Moreover, as paralysis is common in GBS patients, individualized treatment plans can involve rehabilitation to strengthen muscles and regain movement.6 In order to reduce the severity of the disease, GBS patients can be treated with plasma exchange and intravenous immune globulin. These methods work to remove circulating antibodies and other immune-mediators in order to terminate the auto-immune response of the host. While effective in large proportions of GBS patients, these treatments require one year to regain lost motor function.6
Many treatments for GBS have demonstrated effectiveness; however, caring for patients in a clinical setting can have its complexities as the outcomes may be poor. For instance, 10% may die, 20% have difficulties walking after six months and many patients experience residual symptoms which can include pain and fatigue.8
“Novel research is focused primarily on establishing better preventative mechanisms and screening tests.”
Current Research
Continued research in this area of study is important in order to maximize efficacy and efficiency while treating the disease. Current research investigating a potential genetic basis of GBS has identified a possible genetic heritability of the disease; Single Nucleotide Polymorphisms (SNPs) can contribute up to 25% of susceptibility of the disease in adult populations.9 Furthermore, scientists have examined GBS incidence in organ transplant recipients. The findings of these studies revealed that these recipients possess increased susceptibility to infections which can ultimately promote GBS onset due to immunosuppressive therapies that are administered in order to prevent graft rejection.10 As there currently is no cure for GBS, novel research is focused primarily on establishing better preventative mechanisms and screening tests that can reduce the risk of disease onset. While there has been significant progress in treatment development and support for afflicted individuals, there are still plenty of research avenues and therapeutic advancements to consider and explore.
Relevant Resources
Guillain-Barré Syndrome Foundation of Canada
The Guillain-Barré Foundation of Canada is a registered Canadian charity striving to connect patients of this condition and their families with dedicated volunteers. This foundation has also established a Medical Advisory Board consisting of neurologists trained in the diagnosis and treatment of GBS.
Works Cited:
1. Blau N, van Spronsen FJ, Levy HL. Phenylketonuria. Lancet, 2010;376:1417-1427. https://doi.org/10.1016/S0140-6736(10)60961-0
1. Sohara E, Saraya T, Honda K, Yamada A, Inui T, Ogawa Y, Sada M, Tsuiimoto N, Nakamura M, Tsuchiya A, Saito M, Oishi C, Chiba A, Takizawa H, Goto H. Guillain-Barré syndrome in two patients with respiratory failure and a review of the Japanese literature. J Thorac Dis. 2012;4(6):601-607.
2. Ansar V, Valadi N. Guillain-Barré Syndrome. Primary Care: Clinics in Office Practice. 2015;42(2):189-193.
3. Nyati K, Prasad K. Role of Cytokines and Toll-Like Receptors in the Immunopathogenesis of Guillain-Barré Syndrome. Mediators of Inflammation. 2014.
4. Nyati K, Nyati R. Role of Campylobacter jejuni Infection in the Pathogenesis of Guillain-Barré Syndrome: An Update. Biomed Research International. 2013.
5. Willison H, Jacobs B, van Doorn P. Guillain-Barré syndrome. The Lancet. 2016;388(10045):717-727. doi:10.1016/s0140-6736(16)00339-1
6. Meena A, Murthy J, Khadilkar S. Treatment guidelines for Guillain-Barré Syndrome. Ann Indian Acad Neurol. 2011;14(5):73.
7. Dimachkie M, Barohn R. Guillain-Barré Syndrome and Variants. Neurol Clin. 2013;31(2):491-510.
Cite This Article:
Pasumarthi K., Chan G., Palczewski K., Lewis K., Ho J. A Paralyzing Attack on Self: Guillain-Barré Syndrome. Illustrated by W. Y. Wu. Rare Disease Review. October 2019.
DOI:10.13140/RG.2.2.17732.17288.
