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Guillain-barre Syndrome

Disease Details

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Description: Guillain-Barré syndrome is a rare neurological disorder where the body's immune system mistakenly attacks the peripheral nerves, leading to muscle weakness and potentially paralysis.
Type: Guillain-Barré Syndrome (GBS) is primarily an autoimmune disorder that affects the peripheral nervous system. It is not typically associated with genetic transmission and is usually triggered by an infection or, in rare cases, by vaccinations. There is no known hereditary pattern for GBS.
Signs And Symptoms: The first symptoms of Guillain–Barré syndrome are numbness, tingling, and pain, alone or in combination. This is followed by weakness of the legs and arms that affects both sides equally and worsens over time. The weakness can take half a day to over two weeks to reach maximum severity, and then becomes steady. In one in five people, the weakness continues to progress for as long as four weeks. The muscles of the neck may also be affected, and about half experience involvement of the cranial nerves that supply the head and face; this may lead to weakness of the muscles of the face, swallowing difficulties and sometimes weakness of the eye muscles. In 8%, the weakness affects only the legs (paraplegia or paraparesis). Involvement of the muscles that control the bladder and anus is unusual. In total, about a third of people with Guillain–Barré syndrome continue to be able to walk. Once the weakness has stopped progressing, it persists at a stable level ("plateau phase") before improvement occurs. The plateau phase can take between two days and six months, but the most common duration is a week. Pain-related symptoms affect more than half, and include back pain, painful tingling, muscle pain, and pain in the head and neck relating to irritation of the lining of the brain.Many people with Guillain–Barré syndrome have experienced the signs and symptoms of an infection in the 3–6 weeks before the onset of the neurological symptoms. This may consist of upper respiratory tract infection (rhinitis, sore throat), or diarrhea.
In children, particularly those younger than six years old, the diagnosis can be difficult and the condition is often initially mistaken (sometimes for up to two weeks) for other causes of pains and difficulty walking, such as viral infections, or bone and joint problems.On neurological examination, characteristic features are the reduced strength of muscles and reduced or absent tendon reflexes (hypo- or areflexia, respectively). However, a small proportion have normal reflexes in affected limbs before developing areflexia, and some may have exaggerated reflexes. In the Miller Fisher variant of Guillain–Barré syndrome (see below), a triad of weakness of the eye muscles, abnormalities in coordination, as well as absent reflexes can be found. The level of consciousness is normally unaffected in Guillain–Barré syndrome, but the Bickerstaff brainstem encephalitis subtype may feature drowsiness, sleepiness, or coma.
Prognosis: Guillain–Barré syndrome can lead to death as a result of many complications: severe infections, blood clots, and cardiac arrest likely due to autonomic neuropathy. Despite optimum care, this occurs in about 5% of cases.There is a variation in the rate and extent of recovery. The prognosis of Guillain–Barré syndrome is determined mainly by age (those over 40 may have a poorer outcome), and by the severity of symptoms after two weeks. Furthermore, those who experienced diarrhea before the onset of the disease have a worse prognosis. On the nerve conduction study, the presence of conduction block predicts poorer outcome at 6 months. In those who have received intravenous immunoglobulins, a smaller increase in IgG in the blood two weeks after administration is associated with poorer mobility outcomes at six months than those whose IgG level increased substantially. If the disease continues to progress beyond four weeks, or there are multiple fluctuations in the severity (more than two in eight weeks), the diagnosis may be chronic inflammatory demyelinating polyneuropathy, which is treated differently.In research studies, the outcome from an episode of Guillain–Barré syndrome is recorded on a scale from 0 to 6, where 0 denotes completely healthy; 1 very minor symptoms but able to run; 2 able to walk but not to run; 3 requiring a stick or other support; 4 confined to bed or chair; 5 requiring long-term respiratory support; 6 death.The health-related quality of life (HRQL) after an attack of Guillain–Barré syndrome can be significantly impaired. About a fifth are unable to walk unaided after six months, and many experience chronic pain, fatigue and difficulty with work, education, hobbies and social activities. HRQL improves significantly in the first year.
Onset: Guillain-Barré Syndrome (GBS) typically has an acute onset, often occurring over the course of days to a few weeks. It generally begins with weakness and tingling in the legs and can progress to muscle weakness or paralysis, potentially affecting the arms and upper body. The precise cause of the onset is unknown (nan), but it is often triggered by an infection such as respiratory or gastrointestinal illness.
Prevalence: The prevalence of Guillain-Barré Syndrome (GBS) is relatively low, with an estimated range of 1 to 2 cases per 100,000 people annually.
Epidemiology: In Western countries, the number of new episodes per year has been estimated to be between 0.89 and 1.89 cases per 100,000 people. Children and young adults are less likely to be affected than the elderly: the relative risk increases by 20% for every decade of life. Men are more likely to develop Guillain–Barré syndrome than women; the relative risk for men is 1.78 compared to women.The distribution of subtypes varies between countries. In Europe and the United States, 60–80% of people with Guillain–Barré syndrome have the demyelinating subtype (AIDP), and AMAN affects only a small number (6–7%). In Asia and Central and South America, that proportion is significantly higher (30–65%). This may be related to the exposure to different kinds of infection, but also the genetic characteristics of that population. Miller Fisher variant is thought to be more common in Southeast Asia.
Intractability: Guillain-Barré Syndrome (GBS) is generally not considered intractable. It is an acute, autoimmune condition where the body's immune system attacks the peripheral nervous system. While it can cause severe muscle weakness and paralysis, many patients recover with appropriate medical treatment, including immunoglobulin therapy or plasmapheresis. However, the recovery process can vary, and some patients might experience residual weakness or other complications.
Disease Severity: Guillain-Barré Syndrome (GBS) can vary in severity from mild weakness to severe paralysis. Most patients require hospitalization, and a significant number experience debilitating effects that may necessitate intensive care, especially if respiratory muscles are involved. While many recover fully, some may have long-term residual weakness or other complications. Cases may turn life-threatening if not promptly managed.
Healthcare Professionals: Disease Ontology ID - DOID:12842
Pathophysiology: The nerve dysfunction in Guillain–Barré syndrome is caused by an immune attack on the nerve cells of the peripheral nervous system and their support structures. The nerve cells have their body (the soma) in the spinal cord and a long projection (the axon) that carries electrical nerve impulses to the neuromuscular junction, where the impulse is transferred to the muscle. Axons are wrapped in a sheath of Schwann cells that contain myelin. Between Schwann cells are gaps (nodes of Ranvier) where the axon is exposed. Different types of Guillain–Barré syndrome feature different types of immune attacks. The demyelinating variant (AIDP, see below) features damage to the myelin sheath by white blood cells (T lymphocytes and macrophages); this process is preceded by activation of a group of blood proteins known as complement. In contrast, the axonal variant is mediated by IgG antibodies and complement against the cell membrane covering the axon without direct lymphocyte involvement.Various antibodies directed at nerve cells have been reported in Guillain–Barré syndrome. In the axonal subtype, these antibodies have been shown to bind to gangliosides, a group of substances found in peripheral nerves. A ganglioside is a molecule consisting of ceramide bound to a small group of hexose-type sugars and containing various numbers of N-acetylneuraminic acid groups. The key four gangliosides against which antibodies have been described are GM1, GD1a, GT1a, and GQ1b, with different antiganglioside antibodies being associated with particular features; for instance, GQ1b antibodies have been linked with Miller Fisher variant GBS and related forms including Bickerstaff encephalitis. The production of these antibodies after an infection probably is the result of molecular mimicry, where the immune system is reacting to microbial substances, but the resultant antibodies also react with substances occurring naturally in the body. After a Campylobacter infection, the body produces antibodies of the IgA class; only a small proportion of people also produce IgG antibodies against bacterial substance cell wall substances (e.g. lipooligosaccharides) that cross react with human nerve cell gangliosides. It is not currently known how this process escapes central tolerance to gangliosides, which is meant to suppress the production of antibodies against the body's own substances. Not all antiganglioside antibodies cause disease, and it has recently been suggested that some antibodies bind to more than one type of epitope simultaneously (heterodimeric binding) and that this determines the response. Furthermore, the development of pathogenic antibodies may depend on the presence of other strains of bacteria in the bowel.It has been suggested that a poor injection technique may also cause a direct injury to the axillary nerves adjacent to the injection site in deltoid muscle that may lead to peripheral neuropathy. The consequent vaccine transfection and translation in the nerves may spur an immune response against nerve cells potentially causing an autoimmune nerve damage, leading to conditions like Guillain–Barré syndrome.
Carrier Status: Guillain-Barré Syndrome (GBS) is not a genetic disease, so there is no carrier status associated with it. It is an autoimmune condition where the body's immune system attacks the peripheral nerves, often triggered by an infection.
Mechanism: Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder where the body's immune system mistakenly attacks peripheral nerves.

**Mechanism:**
1. **Immune Response Malfunction**: Typically follows an infection (bacterial or viral) that triggers an abnormal immune response.
2. **Demyelination**: The immune system targets the myelin sheath (protective covering of nerve fibers) or the nerve axons themselves, leading to impaired nerve signal transmission.
3. **Nerve Damage**: This results in muscle weakness, numbness, and potential paralysis.

**Molecular Mechanisms:**
1. **Molecular Mimicry**: Pathogens (like Campylobacter jejuni) share epitopes similar to components of peripheral nerves, leading to cross-reactivity where the immune system attacks both the pathogen and myelin/axons.
2. **Antiganglioside Antibodies**: In GBS, the immune system produces antibodies against gangliosides (components of nerve membranes). These antibodies can activate complement pathways and macrophages, leading to nerve damage.
3. **Inflammatory Cytokines**: Release of cytokines in response to infection contributes to inflammation and further damage to peripheral nerves.

These mechanisms combine to produce the clinical manifestation of Guillain-Barré Syndrome, which can vary in severity and duration.
Treatment: There appears to be an error in the input "nan." If you meant "not applicable" or something else, please clarify.

If you simply want information about the treatment of Guillain-Barré syndrome, here is the relevant information:

- **Plasma Exchange (Plasmapheresis)**: This process involves removing and replacing the liquid portion of blood (plasma). It can help reduce the severity and duration of Guillain-Barré syndrome symptoms.

- **Intravenous Immunoglobulin (IVIg)**: High doses of immunoglobulin can help block the damaging antibodies that may contribute to this syndrome.

- **Pain Management**: Pain relievers, such as analgesics, may be used to manage pain associated with Guillain-Barré syndrome.

- **Physical Therapy**: To help maintain muscle strength and flexibility, physical therapy is often recommended.

- **Respiratory Support**: In severe cases, patients may require mechanical ventilation to assist with breathing.

- **Supportive Care**: This includes monitoring vital functions, preventing complications like infections or blood clots, and providing nutrition and hydration support.

Please clarify if you need information on another specific aspect of Guillain-Barré syndrome.
Compassionate Use Treatment: Guillain-Barré Syndrome (GBS) is an acute illness affecting the peripheral nervous system, often leading to muscle weakness and paralysis. For compassionate use or off-label/experimental treatments, several options have been explored:

1. **Eculizumab:** This monoclonal antibody targeting the complement system has been investigated for GBS due to its potential role in reducing the immune system's attack on nerves.

2. **Beta-Interferons:** These are immunomodulatory agents primarily used in multiple sclerosis, explored for their potential to modulate the immune response in GBS.

3. **Stem Cell Therapy:** Experimental studies are assessing the potential of autologous stem cell transplants to promote nerve repair and regeneration in severe cases of GBS.

4. **Rituximab:** A monoclonal antibody against CD20, typically used in B-cell malignancies and autoimmune diseases, has been investigated for its potential to decrease autoantibody production in GBS.

These treatments remain largely experimental and are generally considered when standard therapies, such as intravenous immunoglobulin (IVIg) and plasmapheresis, are ineffective. Clinical trials and careful medical oversight are crucial for their use.
Lifestyle Recommendations: Guillain-Barré Syndrome (GBS) is a rare neurological disorder where the body's immune system mistakenly attacks the peripheral nerves. Lifestyle recommendations for someone recovering from GBS include:

1. **Physical Therapy**: Engage in physical therapy to regain strength and improve mobility. Tailored exercises can help restore muscle function.

2. **Occupational Therapy**: Work with an occupational therapist to relearn daily activities and improve fine motor skills.

3. **Balanced Diet**: Maintain a nutritious diet to support overall health and recovery. Adequate protein and vitamins are crucial for healing.

4. **Adequate Rest**: Ensure plenty of rest to help the body recover. Avoid overexertion and listen to your body’s needs.

5. **Hydration**: Stay well-hydrated to support bodily functions and recuperation.

6. **Stress Management**: Practice stress-reducing techniques such as yoga, meditation, or gentle breathing exercises.

7. **Adaptive Equipment**: Use adaptive tools if necessary to aid in daily activities, such as braces, canes, or wheelchairs.

8. **Follow-Up Care**: Keep up with regular medical appointments to monitor progress and adjust treatment as needed.

9. **Support System**: Stay connected with family, friends, or support groups to get emotional and practical support during recovery.
Medication: For Guillain-Barré Syndrome (GBS), there is no specific "medication" that cures the disorder, but there are treatments that can help manage the condition and improve outcomes:

1. **Intravenous Immunoglobulin (IVIG):** This treatment involves injecting antibodies from donated blood to help reduce the immune attack on the nervous system.
2. **Plasma Exchange (Plasmapheresis):** This procedure involves removing and treating the blood plasma to remove the antibodies that are attacking the nervous system.

Supportive care, such as physical therapy, pain management, and sometimes respiratory support, may also be required.
Repurposable Drugs: Current research on Guillain-Barré Syndrome (GBS) explores repurposing several drugs. These include:

1. **IV Immunoglobulin (IVIG)** - Commonly used for immune disorders, it helps reduce the immune system's attack on the nervous system.
2. **Corticosteroids** - Though not consistently effective, they are sometimes used to reduce inflammation.
3. **Plasmapheresis** - Not a drug, but a procedure often used with repurposed drugs to remove antibodies from the blood.

Research is ongoing, but these therapies aim to mitigate the immune response and support recovery. Always consult with a healthcare professional for personalized medical advice.
Metabolites: Guillain-Barré Syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. It's typically preceded by an infection.

Regarding metabolites in the context of GBS, alterations in certain metabolites might occur due to the disease’s effect on the body and the immune response. Some research has identified changes in metabolite profiles, such as elevated glycolysis intermediates, amino acids, and lipid metabolites, which can reflect the metabolic stress and tissue damage.

"NAN" is not a standard abbreviation related to Guillain-Barré Syndrome, metabolites, or any biological concept I’m aware of in this context. Please provide more details or clarify if you need information about something specific.
Nutraceuticals: There are no specific nutraceuticals (dietary supplements) recommended for the treatment of Guillain-Barré Syndrome (GBS). The primary treatments for GBS typically include intravenous immunoglobulin (IVIG) or plasmapheresis (plasma exchange). GBS is a serious autoimmune disorder, and its management should be supervised by healthcare professionals. Always consult with a medical professional before starting any new supplement or treatment.
Peptides: Guillain-Barré syndrome (GBS) is an autoimmune disorder where the body's immune system mistakenly attacks the peripheral nerves. It typically results in muscle weakness and sometimes paralysis. Research into peptides for GBS is ongoing, aiming to modify immune responses or promote nerve repair. Nanotechnology-based approaches are also being investigated for their potential to deliver targeted treatments, improve drug efficacy, and reduce side effects. These innovative therapies are still largely in experimental phases.