TITLE: Guillain-Barré syndrome OVERVIEW: A rare disorder called Guillain-Barré Syndrome (GBS) is a result of the immune system damaging the peripheral nerve. According to Andary et. al.(2022), GBS is a type of clinical condition that manifests an acute inflammatory polyradiculoneuropathy resulting in reduced reflexes and frailty. The damage leads to muscle weakness, and sometimes when the damage is fatal it leads to paralysis. This syndrome affects the nerve that controls the muscle of our body that transmits a message to our brain, creating dysfunction in the touch sensation that our body has (World Health Organization, 2023). This dysfunction blocks the ability of an individual to feel pain and temperature. The World Health Organization (2023), added that the most affected age is among the adults, specifically male adults. But people should not be careless since it can affect all ages and all genders. Additionally, this syndrome has a possible lethal risk, and the main reason behind this syndrome is still unknown to the medical experts resulting in no known cure or vaccine, yet experts highly recommend medications and treatments that could be used as an alternative to ease syndromes and eventually reduces the duration of this illness (Mayo Clinic, 2023). Since the reason is unknown, experts' hypothesis prove that 2/3 of the patients have reported symptoms of infection due to viruses. Namely COVID-19, Respiratory Infection, Gastrointestinal Infection, and Zika Virus. All patients require feasible care; some may even require critical care, such as supportive care and some immunological treatments as part of the treatment. According to the report suggested by the National Health Service in the year 2023, the treatment that serves as an alternative to the cure is successful and brings full recovery to the patients. According to the Centers for Disease Control and Prevention (2023), each year, the United States of America documented an increase in patients that ranges from 3000-6000 American patients with GBS. About 85% of patients fully recover after 6 months to 1 year. After recovering from GBS, Robinson (2022) reveals that there is just a small chance of suffering from the same infection again. SYMPTOMS: The typical GBS patient typically presents 2-4 weeks after a relatively benign respiratory or gastrointestinal illness with complaints of finger dysesthesias and proximal muscle weakness of the lower extremities. GBS patients typically present as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). The cranial nerves, breathing muscles, truncal muscles, and arms may all get weaker over hours or days. 1. Muscle Weakness and Paralysis: Muscle weakness, which frequently begins in the legs and spreads to the arms and upper torso, is one of the main signs and symptoms of GBS. This weakening can advance quickly and is often symmetrical. In extreme circumstances, people may become paralyzed, which would impair their movement and everyday functioning. Usually, this paralysis increases and peaks two to four weeks following the beginning of symptoms. 2. Ascending Symptom Progression: An escalating pattern of weakness and paralysis is typical in GBS patients. As the immune system attacks the peripheral nerves, it causes this distinctive trend, which begins in the extremities and progresses toward the trunk. An important diagnostic characteristic of GBS is its ascending nature, which aids in distinguishing it from other neurological disorders. 3. Sensory Abnormalities: In GBS, sensory symptoms usually coexist with motor symptoms. Patients may have pain, tingling, and numbness; these symptoms frequently start in the fingers and toes and extend throughout the body. The degree of the sensory abnormalities might vary, and they could be one of the first indications that require medical intervention. 4. Autonomic Dysfunction: An influence on the autonomic nerve system by GBS might result in a range of symptoms associated with involuntary body functions. Variations in heart rate, blood pressure, and breathing can all be signs of autonomic dysfunction. Patients may have breathing problems and heart rate variations that range from tachycardia to bradycardia, which complicates the clinical presentation. 5. Paresthesias and Neuropathic Pain: In addition to motor and sensory symptoms, paresthesias—abnormal feelings such as tingling or prickling—are a common presentation of GBS. Another typical aspect is neuropathic pain, which can be uncomfortable and add to the overall difficulties that people with GBS endure. According to the National Institute of Neurological Disorder and Stroke (2023), GDS includes the following symptoms: Difficulty with eye muscles and vision Difficulty swallowing, speaking, or chewing Pricking or pins and needles sensations in the hands and feet Pain that can be severe, particularly at night Coordination problems and unsteadiness Abnormal heartbeat/rate or blood pressure Problems with digestion and/or bladder control Facial droop (may mimic Bell palsy) Diplopias Dysarthria Dysphagia Ophthalmoplegia Pupillary disturbances Tachycardia Bradycardia Facial flushing Paroxysmal hypertension Orthostatic hypotension Anhidrosis and/or diaphoresis Urinary retention Tachycardia Bradycardia Facial flushing Paroxysmal hypertension Orthostatic hypotension Anhidrosis and/or diaphoresis Urinary retention CAUSE: Experts believe that some issue with the immune system, the body's natural defense against disease and infection, is the origin of Guillain-Barré syndrome. The immune system typically targets any pathogens that enter the body. However, in those who have Guillain-Barré syndrome, an abnormality causes the nerves to be unintentionally attacked and damaged. The specific cause of this is unknown, although the illness frequently follows an infection—particularly one that affects the respiratory system, like the flu, or the digestive system, such as food poisoning or a stomach virus (gastroenteritis) (National Health Service, 2023 ). Here are some possible causes of GDS: Infectious Agents: A robust correlation has been shown in several investigations between GBS and prior illnesses, including those caused by bacteria and viruses. Of all the microorganisms associated with GBS, Campylobacter Bacteria can be found in food poisoning, and is a notable trigger. A Campylobacter Bacteria infection may precede up to 40% of GBS cases, according to an expert testimony. Furthermore, viruses such as the Epstein-Barr virus, CMV, and Zika virus (A tropical virus, categorized as a family with Dengue), as well as other bacterial infections like Mycoplasma pneumoniae, Flu, cytomegalovirus, and glandular fever, have been linked to the development of GBS. One theory for the mechanism of infection-related GBS is molecular mimicry. When the immune system is triggered by an infection, it might unintentionally attack parts of the nervous system that mimic the characteristics of the infectious pathogen. Gangliosides on nerve cells, for example, structurally mimic lipopolysaccharides on Campylobacter Bacteria, which can cause autoimmune injury and cross-reactivity. Vaccination as a Trigger: Historically, immunizations have been associated with a higher incidence of Guillain-Barré syndrome, especially the flu shot administered to US citizens during the 1976 swine flu pandemic. However, further study has shown that there is a very low likelihood of contracting the illness following a vaccine. For instance, research on the vaccination used during the 2009 swine flu pandemic discovered that there were less than 2 more occurrences of Guillain-Barré syndrome for every million recipients of the immunization. Furthermore, data indicates that contracting an illness, like the flu, increases your risk of developing Guillain-Barré syndrome considerably more than vaccinations meant to fend off infections, like the flu shot. Genetic Predisposition: Although genetic factors do not directly cause GBS, they do influence an individual's vulnerability to the condition. Specific genetic polymorphisms linked to GBS susceptibility have been found through genome-wide association studies (GWAS), suggesting a complex interaction between genetic and environmental variables. Surgical Trauma and Stress: Trauma and surgery have occasionally been identified as possible GBS triggers. These events' stress and inflammatory reactions may set off an aberrant immune response that results in the development of GBS. But in contrast to instances linked to infections, these are very uncommon. Guillain-Barre Syndrome is a complex illness with a wide range of possible causes. Vaccinations, genetic predisposition, surgical trauma, and infections—especially those caused by bacteria and certain viruses—have all been linked. The complicated interaction between genetic and environmental variables, as well as molecular mimicry, add to the intricacy of GBS pathogenesis. Comprehending these plausible origins is essential for propelling investigations, refining diagnostics, and creating focused treatment approaches. PATHOPHYSIOLOGY: GBS is an immune-mediated, postinfectious illness. Humoral and cellular immunological processes likely contribute to its development. In the weeks before GBS manifests, the majority of patients report having an infectious disease. It is believed that a large number of the infectious agents that have been discovered cause the formation of antibodies that react with certain gangliosides and glycolipids, such as GD1b and GM1, that are found throughout the peripheral nervous system's myelin. Campylobacter jejuni infections can be used to characterize the pathophysiologic mechanism of both GBS and an antecedent disease. It's believed that C jejuni's virulence stems from the presence of certain antigens in its capsule that are shared by nerves. Peripheral nerve injury is caused by antibodies that cross-react with ganglioside GM1 in myelin as a result of immune reactions against lipopolysaccharide antigens in the C. jejuni capsule. We refer to this mechanism as molecular mimicry. Pathologic features of GBS include multifocal myelin stripping mediated by macrophages, which is followed by lymphocytic infiltration of peripheral and spinal neurons (cranial nerves may also be affected). This syndrome causes flaccid paralysis by impairing the transmission of electrical nerve signals, which eventually leads to their absence or in their significant delay. Remyelination is usually linked to recovery. Axonal disruption and loss is a subsequent effect of acute inflammation in certain people with advanced illness. A subset of individuals may have myelin sparing after an initial immune response directed directly against nerve axons. These patients' clinical presentations resemble those of the primary type. SUBTYPE OF GBS ACCORDING TO Andary et. al. (2022): Acute inflammatory demyelinating polyneuropathy: Acute inflammatory demyelinating polyneuropathy (AIDP) is the most frequently diagnosed subtype in the US; it usually follows a bacterial or viral infection; approximately 40% of patients have a positive seropositive result for C jejuni; there is lymphocytic infiltration and peripheral nerve demyelination mediated by macrophages; symptoms usually go away with remyelination. Acute motor axonal neuropathy: A solely motor condition, acute motor axonal neuropathy (AMAN) subtype is more common in pediatric age groups. Rapidly developing symmetrical weakness and subsequent respiratory failure are the hallmarks of AMAN. Approximately 70–75% of AMAN patients have positive Campylobacter seropositivity, and most AMAN cases are linked to prior C jejuni diarrhea. Patients usually have elevated ganglioside antibody titers (GM1, GD1a, GD1b). The blood-CNS barrier may become disrupted as a result of inflammation of the spinal anterior roots. Wallerian-like degeneration without a noticeable lymphocytic inflammation is seen in biopsies. In China's rural areas, a lot of cases have been documented, particularly in the summer months involving children and young adults. Pure axonal cases may happen more commonly outside of North America and Europe. Additionally, AMAN instances can differ from axonal GBS cases reported in the West. The prognosis is frequently very good. While many individuals with AMAN recover quickly, severely impaired patients may take years to see improvement. One-third of AMAN patients are likely to be hyperreflexic. Although the cause of this hyperreflexia is unknown, malfunction of the inhibitory system via spinal interneurons may increase motor neuron excitability. The presence of anti-GM1 antibodies is substantially related to hyperreflexia. Acute motor-sensory axonal neuropathy: Acute motor-sensory axonal neuropathy (AMSAN) is a severe acute sickness that, like AMAN, damages sensory nerves and roots. Most patients are adults. AMSAN frequently manifests as sudden and severe motor and sensory impairment. Muscle wasting is common, and recovery is slower than in electrophysiologically similar cases of AMAN. AMSAN, like AMAN, is frequently associated with antecedent C jejuni diarrhea. Pathologic examination reveals significant axonal degeneration of motor and sensory nerve fibers with minimal demyelination. Miller Fish Syndrome: Miller-Fisher syndrome (MFS), which affects roughly 5% of all GBS cases, is characterized by a triad of ataxia, areflexia, and ophthalmoplegia. A key hallmark is the sudden onset of external ophthalmoplegia. Ataxia is often disproportionate to the degree of sensory loss. Mild limb weakness, ptosis, facial palsy, or bulbar palsy may also occur in patients. Patients have reduced or absent sensory nerve action potentials and absent tibial H reflex. Anti-GQ1b antibodies are common in MFS and have a high specificity and sensitivity for the condition. GQ1b ganglioside concentrations are high in the oculomotor, trochlear, and abducens nerves, which may explain the link between anti-GQ1b antibodies and ophthalmoplegia. Anti-GQ1b/GT1a IgG antibodies are seen in patients with acute oropharyngeal palsy. Recovery often takes 1-3 months. Acute panautonomic neuropathy: The rarest form of GBS, acute panautonomic neuropathy, affects both the sympathetic and parasympathetic neural systems. The patients exhibit aberrant pupil size, reduced salivation and lacrimation, anhidrosis, bowel and bladder retention, and severe postural hypotension. Cardiovascular involvement is prevalent, and a major cause of death is dysrhythmias. There is not much motor or sensory engagement. Recovery happens gradually and frequently falls short. Pure Sensory GBS: In the literature, a pure sensory type of GBS has been reported. It is characterized by a symmetrical and extensive pattern of rapidly developing sensory ataxia, areflexia, and sensory loss. Albuminocytologic dissociation is shown in the CSF by lumbar puncture tests, and electromyography (EMG) results reveal typical symptoms of a demyelinating process in the peripheral nerves. For pure GBS, the prognosis is usually favorable. Patients with severe illness or a sluggish rate of recovery may be candidates for immunotherapies such as IVIGs and plasma exchange. Other variants: Isolated weakness in the face, oropharynx, cervical region, and upper limbs without involvement of the lower limbs characterize the pharyngeal-cervicalbrachial type of gastrointestinal block syndrome. Virtually any combination of nerve damage can occur, as can combinations of any of the aforementioned categories. Mild instances that cause transient symptoms, resolve on their own, and never receive a clear diagnosis are probably present. Seldom are other uncommon clinical variations with constrained patterns of weakness seen. TREATMENT: Guillain-Barré syndrome (GBS) is a rare but serious neurological disorder where the body's immune system mistakenly attacks the peripheral nerves. The exact cause of GBS is not fully understood, but it often occurs after an infection, such as a respiratory or gastrointestinal infection. The syndrome can progress rapidly and lead to muscle weakness or paralysis. 1. Treatment for Guillain-Barré syndrome typically involves supportive care and medical interventions to manage symptoms, provide relief, and promote recovery. Here are some key components of GBS treatment: 2. Hospitalization: Many individuals with GBS require hospitalization, especially if the symptoms are severe. This allows close monitoring of respiratory function and other vital signs. 3. Intravenous Immunoglobulin (IVIG): One of the main treatments for GBS is the administration of intravenous immunoglobulin (IVIG). IVIG is a blood product that contains antibodies pooled from multiple donors. It helps reduce the immune system's attack on the nerves and may speed up recovery. The standard dose is usually given over a few days. 4. Plasma Exchange (Plasmapheresis): Another treatment option is plasma exchange, where the liquid portion of the blood (plasma) is removed and replaced with a substitute. This helps remove the antibodies causing the immune system to attack the nerves. 5. Pain Management: Pain and discomfort are common symptoms of GBS. Pain medications, such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs), may be used to alleviate pain. 6. Respiratory Support: Since GBS can affect the muscles responsible for breathing, individuals with severe cases may require mechanical ventilation to assist with breathing. Respiratory therapy and close monitoring of respiratory function are crucial components of care. 7. Physical Therapy: Physical therapy plays a significant role in the recovery process. It helps maintain muscle strength, prevent joint stiffness, and improve overall mobility. Rehabilitation services may include exercises, stretching, and other therapeutic interventions. 8. Occupational Therapy: Occupational therapy focuses on helping individuals regain the ability to perform daily activities, such as dressing, bathing, and eating. It aims to enhance independence and improve the quality of life. 9. Nutritional Support: In some cases, nutritional support may be necessary, especially if swallowing is affected. This can involve a feeding tube to ensure that individuals receive the necessary nutrients. It's important to note that the severity and course of GBS can vary among individuals, and the specific treatment plan may be tailored to each person's needs. Early diagnosis and intervention are crucial for a better prognosis, and individuals with suspected GBS should seek prompt medical attention. Treatment decisions are typically made by healthcare professionals based on the specific circumstances of each case.
