Disease Profile

Guillain-Barre syndrome

Prevalence ?
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000

3,310 - 29,790

US Estimated

1-9 / 100 000

46,215 - 5,135

Europe Estimated

Age of Onset

All ages

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ICD-10

G61.0

Inheritance

Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other Names (AKA)

Acute autoimmune peripheral neuropathy; GBS; Acute immune-mediated polyneuropathy;

Categories

Nervous System Diseases

Summary

Guillain-Barré syndrome (GBS) is a rare syndrome in which the body’s immune system attacks part of the peripheral nervous system. The peripheral nervous system carries signals from the brain to the muscles. Symptoms of GBS include muscle weakness, numbness, and tingling sensations, which can increase in intensity until the muscles cannot be used at all (paralysis).[1]

The exact cause of Guillain-Barré syndrome is unknown. In most cases, GBS occurs a few days or weeks after symptoms of a viral infection. In rare cases, GBS may run in families.[2] A diagnosis of GBS is suspected when a person has symptoms suggestive of the syndrome. A variety of tests, including a spinal tap, may be completed to confirm the diagnosis. Treatment options may include plasma exchange (plasmapheresis) and immunoglobulin therapy.[1][3]

 

Symptoms

Signs and symptoms of Guillain-Barré syndrome (GBS) include muscle weakness, muscle pain, numbness, and tingling sensations. GBS can affect people of any age. The first symptoms of GBS typically begin in the lower legs. The symptoms can then spread to the muscles of the upper body. Typically, the symptoms continue to worsen over the first 2-3 weeks after they first begin. In some cases, the symptoms of GBS can increase in intensity until the muscles cannot be used at all (paralysis).[1]

Other symptoms of GBS can include difficulty with bladder control or bowel function, difficulty breathing, blood pressure, and heart rate. Some people with GBS have facial droop, double vision, difficulty speaking or swallowing, and changes in eye movements. As the disease progresses, the muscle weakness may worsen to affect the muscles that are important for breathing.[3][4]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Acute demyelinating polyneuropathy
0007131
Autosomal dominant inheritance
0000006

Cause

The exact cause of Guillain-Barré syndrome (GBS) is not well-understood in all cases of the syndrome. GBS can occur at any age, but it is especially common in people older than 50-years-old. GBS is considered to be an autoimmune disease, meaning that it occurs when the immune system mistakenly attacks tissues of its own body. Specifically, the immune system of people with GBS targets the nerves of the peripheral nervous system. The peripheral nervous system is the part of the nervous system that is outside of the brain and spinal cord. It is responsible for carrying signals from the brain to the muscles of the body. When the immune system attacks the peripheral nervous system, signals cannot be sent from the brain to the muscles as quickly as they should. This causes the signs and symptoms associated with GBS.[1]

In some cases, people who develop GBS report that they were sick with a gastrointestinal or upper respiratory illness a few days or weeks before the symptoms of GBS began. These infections can be caused by viruses such as cytomegalovirus, Epstein-Barr virus, flu virus, or Zika virus or bacteria such as Campylobacter jejuni.[3][5] However, only a small number of people who have these infections develop GBS.[6]

On very rare occasions, people have developed GBS within a few days or weeks after receiving a vaccination. For example, in 1976, there were several cases in which people developed GBS after getting the swine flu vaccine. The link between GBS and the flu vaccine in other years is unclear. It is believed that the risk of developing GBS after seasonal flu vaccines is about one in a million. Studies suggest that a person is more likely to develop GBS after having the flu than after getting a vaccine.[5][7]

There are also case reports that suggest a link between GBS and certain medications or surgical procedures. However, a definitive association between medications or surgery and GBS has not been established.[3]

Diagnosis

A diagnosis of Guillain-Barré syndrome (GBS) is suspected when a person has signs and symptoms consistent with the syndrome. Doctors may identify symptoms such as muscle weakness on both sides of the body that starts from the lower leg muscles and spreads upwards. Doctors may also look for other signs of GBS such as relatively rapid progression of symptoms and loss of muscle reflexes.[1]

Tests can be completed to confirm a diagnosis of GBS and rule out other diseases that can cause muscle weakness. These tests may include nerve conduction velocity tests to measure how quickly signals are being sent through the peripheral nervous system. An electromyogram (EMG) may be ordered to determine if the muscles are responding to signals correctly. A lumbar puncture (spinal tap) may also be ordered, as people with GBS have high levels of protein in the cerebral spinal fluid (CSF) that surrounds the spine.[3] 

Treatment

Unfortunately, there is no cure for Guillain-Barré syndrome (GBS). However, symptoms of the syndrome can be improved with treatments including plasma exchange therapy and immunoglobulin therapy. Both of these therapies can help prevent the immune system from continuing to attack the peripheral nervous system.[1][3] In some cases, corticosteroids have been used to try to alleviate symptoms of GBS. However, it has not been proven that steroids can help speed recovery.[3]

People who have GBS may be admitted to the hospital for treatment and to reduce the risk of complications. In some cases, other treatments may be necessary to prevent complications of GBS including pain medications and heparin to reduce the risk of blood clots. Some people with GBS may require help breathing with a ventilator.[3] Physical, occupational, and speech therapy may be recommended to help people fully recover from GBS.[3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn More

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • Genetics Home Reference (GHR) contains information on Guillain-Barre syndrome. This website is maintained by the National Library of Medicine.
      • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
      • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Guillain-Barre syndrome. Click on the link to view a sample search on this topic.

          References

          1. Guillain-Barré Syndrome Fact Sheet. National Institute of Neurological Disorders and Stroke. July 2011; https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet.
          2. Guillain-Barre Syndrome, Familial. Online Mendelian Inheritance in Man (OMIM). April 8, 2009; https://www.omim.org/entry/139393.
          3. Andary MT, Oleszek JL, Maurelus K, and White-McCrimmon RY. Guillain-Barre Syndrome. Medscape Reference. October 6, 2017; https://emedicine.medscape.com/article/315632-overview.
          4. Guillain-Barre syndrome. Mayo Clinic. May 18, 2018; https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793.
          5. Zika and Guillain-Barré Syndrome. Centers for Disease Control and Prevention (CDC). August 9, 2016; https://www.cdc.gov/zika/healtheffects/gbs-qa.html.
          6. Guillain-Barré syndrome. Genetics Home Reference. September 2011; https://ghr.nlm.nih.gov/condition/guillain-barre-syndrome.
          7. Guillain-Barré Syndrome. Centers for Disease Control and Prevention. August 28, 2015; https://www.cdc.gov/vaccinesafety/concerns/guillain-barre-syndrome.html.
          8. Vriesendorp FJ. Guillain-Barré syndrome in adults: Treatment and prognosis. UpToDate. April 19, 2017; https://www.uptodate.com/contents/guillain-barre-syndrome-in-adults-treatment-and-prognosis.
          9. Rubin M. Guillain-Barré Syndrome (GBS). Merck Manual. September 2016; https://www.merckmanuals.com/professional/neurologic-disorders/peripheral-nervous-system-and-motor-unit-disorders/guillain-barr%C3%A9-syndrome-gbs.
          10. Ramakrishnan S, Mustare V, Philip M, Thennarasu K, and Perivavan S. Treatment-related Fluctuations in Guillain Barre Syndrome and the Conundrum of Additional Cycles of Plasmapharesis. Annals of Indian Academy of Neurology. October-December 2017; 20(4):372-377. https://www.ncbi.nlm.nih.gov/pubmed/29184340.