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GuillainBarr syndrome
Classification and external resources G61.0 ICD-10 357.0 ICD-9 139393 OMIM DiseasesDB 5465 MedlinePlus 000684 eMedicine emerg/222 neuro/7 pmr/48 neuro/598 D020275 MeSH GuillainBarr syndrome (GBS) (French pronunciation: [il bae], English pronunciation: /ilnbre/), sometimes Landry's paralysis or GuillainBarrStrohl syndrome, is an acute polyneuropathy, a disorder affecting the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system. It can cause life-threatening complications, in particular if the respiratory muscles are affected or if there is autonomic nervous system involvement. The disease is usually triggered by an infection. The diagnosis is usually made by nerve conduction studies and with studies of the cerebrospinal fluid. With prompt treatment by intravenous immunoglobulins or plasmapheresis, together with supportive care, the majority will recover completely. GuillainBarr syndrome is rare, at 12 cases per 100,000 people annually, but is the most common cause of acute non-trauma-related paralysis in the world. The syndrome is named after the French physicians Georges Guillain and Jean Alexandre Barr, who described it in 1916.
Contents
1 Classification 2 Signs and symptoms 3 Cause 4 Diagnosis o 4.1 Testing o 4.2 Criteria 5 Treatment 6 Prognosis 7 Epidemiology
Classification
Six different subtypes of GuillainBarr syndrome exist:[citation needed]
Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS, and the term is often used synonymously with GBS. It is caused by an autoimmune response directed against Schwann cell membranes. Miller Fisher syndrome (MFS) is a rare variant of GBS. Accounting for approximately 5% of GBS cases, it manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS.[1] It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. The ataxia predominantly affects the gait and trunk, with the limbs relatively spared. Anti-GQ1b antibodies are present in 90% of cases. Acute motor axonal neuropathy (AMAN),[2] also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies[3] are present. Anti-GD3 antibodies are found more frequently in AMAN. Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. Like AMAN, it is probably due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.[4] Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias. Frequently occurring symptoms include impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and constipation unrelieved by laxatives or alternating with diarrhea. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils; loss of accommodation) may also be observed. Bickerstaff's brainstem encephalitis (BBE) is a further variant of GuillainBarr syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly
in the brainstem, especially in the pons, midbrain and medulla, are described in the literature. Despite severe initial presentation, BBE usually has a good prognosis. Magnetic resonance imaging (MRI) plays a critical role in the diagnosis of BBE. A considerable number of BBE patients have associated axonal GuillainBarr syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
Cause
Structure of a typical neuron Neuron
Dendrite Soma Axon Nucleus Node of Ranvier Axon terminal Schwann cell Myelin sheath All forms of GuillainBarr syndrome are autoimmune diseases, due to an immune response to foreign antigens (such as infectious agents) that is mistargeted at host nerve tissues instead, a phenomenon called molecular mimicry.[6] The targets of such immune attack are thought to be gangliosides, compounds naturally present in large quantities in human peripheral nerve tissues. The most common antecedent infection is the bacterium Campylobacter jejuni,[7][8] followed by cytomegalovirus (CMV).[9] However, 60% of cases do not have a known cause. Some cases may be triggered by the influenza virus, or by an immune reaction to the influenza virus.[10] There was increased incidence of Guillain-Barr syndrome following influenza immunization during the 1976-1977 swine flu pandemic;[11] however, epidemiological studies since then have demonstrated either an extremely small increased risk following immunization (under 1 additional case per million vaccinations) or no increased risk.[12][13] The end result of this autoimmune attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to muscle paralysis that may be accompanied by sensory or autonomic disturbances. In mild cases, nerve axon (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage occurs, and recovery depends on the regeneration of this important tissue. Approximately 80% of patients have myelin loss; in the remaining 20%, the pathological hallmark is axon loss. GuillainBarr, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's disease (ALS), is a peripheral nerve disorder and does not in general cause nerve damage to the brain or spinal cord.
Diagnosis
The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.
Testing
In cerebrospinal fluid, characteristic findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (1001000 mg/dL), without an accompanying increased cell count (absence of pleocytosis). A sustained increased white blood cell count may indicate an alternative diagnosis such as infection. Electromyography (EMG) and nerve conduction studies (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. F waves and H-reflexes may be prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced, neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle EMG if there is some axonal injury after 3 to 4 weeks. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.
Criteria
Features required for diagnosis are progressive weakness in legs and often arms and areflexia (the absence of deep tendon reflexes).[14][15] Features that strongly support diagnosis are progression of symptoms over days to 4 weeks, relative symmetry of symptoms, mild sensory symptoms or signs, cranial nerve involvement (especially bilateral weakness of facial muscles), autonomic dysfunction, pain (often present), high concentration of protein in CSF, and typical electrodiagnostic features [14][15] Features that should raise doubt about the diagnosis include severe pulmonary dysfunction with limited limb weakness at onset, severe sensory signs with limited weakness at onset, bladder or bowel dysfunction at onset, fever at onset, sharp sensory level, slow progression with limited weakness without respiratory involvement (subacute inflammatory demyelinating polyneuropathy or CIDP is more likely), marked persistent asymmetry of weakness, persistent bladder or bowel dysfunction, increased number of mononuclear cells in CSF (>50106/L), and polymorphonuclear cells in CSF.[14][15]
Treatment
Supportive care is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle most important for breathing. Intubation may be needed when there is evidence of impending failure of the muscles
of breathing when the vital capacity (VC) is less than 20 ml/kg, the negative inspiratory force (NIF) is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability. Subsequent treatment consists of attempting to reduce the body's attack on the nervous system, either by plasmapheresis, filtering antibodies out of the blood stream, or by administering intravenous immunoglobulins (IVIg), to neutralize harmful antibodies and inflammation causing disease. These two treatments are equally effective and a combination of the two is not significantly better than either alone. Glucocorticoids have not been found to be effective in GBS. [16][17] Treatment is usually begun as soon as the diagnosis is made. Plasmapheresis hastens recovery when used within 4 weeks of the onset of symptoms. [17] IVIg has equivalent efficacy to plasmapheresis when started within 2 weeks of the onset of symptoms, and has fewer complications. [17] IVIg is usually used first because of its ease of administration and safety profile. The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Following the acute phase, treatment often consists of rehabilitation with the help of a multidisciplinary team to focus on improving activities of daily living (ADLs). Occupational therapists may offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. Physiotherapists assist to correct functional movement, avoiding harmful compensations that might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with GuillainBarr syndrome regain strength, endurance, and gait quality,[18] as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties.[19] Speech and language therapists help regain speaking and swallowing ability, especially if the patient was intubated or received a tracheostomy.
Prognosis
Recovery usually starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 510% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. Despite all improvements in treatment and supportive care, the death rate is still about 23% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 510% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP). Poor prognostic factors include age over 40 years, history of preceding diarrheal illness, requiring ventilator support, high anti-GM1 titre, and poor upper-limb muscle strength.
Epidemiology
Worldwide, the annual incidence is about 0.64 occurrences per 100,000 people. Men are one and a half times more likely to be affected than women. The incidence increases with age; there are approximately 1 cases per 100,000 people aged below 30 years and about 4 cases per 100,000 in those older than 75 years.[20] The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[21] Congenital and neonatal GuillainBarr syndrome have also been reported.[22]
History
The French physician Jean Landry first described the disorder in 1859. In 1916, Georges Guillain, Jean Alexandre Barr, and Andr Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal-fluid protein production, but normal cell count.[23] GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry's ascending paralysis and LandryGuillainBarr syndrome. Canadian neurologist C. Miller Fisher described the variant that bears his name in 1956.[24]
Notable cases
American actor Andy Griffith developed Guillain-Barr syndrome in 1983. Griffith is seen here receiving an award at the White House in 2005
Jennifer Young, 2013, Prominant Canadian-English SAP Consultant Rytar Arimura, vocalist for Japanese rock band Plastic Tree. It was detected and treated early, and Arimura was able to return to touring within three months.[25] Markus Babbel, former international footballer, contracted GBS in 2001, following a period suffering from the EpsteinBarr virus. He lost almost an entire year of his footballing career between the two illnesses and never again demonstrated the same level of ability.[26]
Tony Benn, British politician.[27] Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela Brimmer, a notable disabilities activist.[28] Tom Edlefsen, American tennis player. Made the fourth round of Wimbledon in 1968, a year after he developed GBS.[29] Rowdy Gaines, a former American swimmer and Olympic three-time gold medalist, was temporarily paralyzed with GBS.[when?] Samuel Goldstein, American athlete and Paralympian.[30] Andy Griffith, an American actor best known for The Andy Griffith Show and Matlock, developed GBS in 1983.[31] Joseph Heller, author, contracted GBS in 1981. This episode in his life is recounted in the autobiographical No Laughing Matter.[32] Luci Baines Johnson, daughter of President Lyndon Johnson and Lady Bird Johnson. Diagnosed and under treatment for GBS in April 2010.[33] Hugh McElhenny, former professional American football player with the San Francisco 49ers.[34] Scott McKenzie (born Philip Wallach Blondheim), an American singer and songwriter most notable for his hit single and hippie anthem "San Francisco (Be Sure to Wear Flowers in Your Hair)", died August 18, 2012, at the age of 73 from GBS. [35] Jim Moss, Canadian professional lacrosse player.[citation needed] Lucky Oceans, Grammy Award-winning musician with Asleep at the Wheel, was diagnosed with GBS in 2008.[36] Len Pasquarelli, sports writer and analyst for ESPN and resident of the Pro Football Writers of America, was diagnosed in 2008.[37] Serge Payer, Canadian-born professional hockey player. After battling and overcoming the syndrome, he set up the Serge Payer Foundation, which is dedicated to raising money for research into new treatments and cures for GBS.[38] William The Refrigerator Perry, former professional American football player with the Chicago Bears, was diagnosed with GBS in 2008.[39] Georgena Sil, Canadian physicist and writer. After recovery, Sil established Tuum Est, a non-profit site devoted to legal rights of the physically disabled.[40] Norton Simon, American industrialist and philanthropist.[41] Kelly-Marie Stewart, British actress.[42] Hans Vonk, Dutch conductor.[43] Morten Wieghorst, Danish former footballer and football coach. [44] Danny Wuerffel, 1996 Heisman Trophy winner from the University of Florida.[45]
References
1. ^ Davids, H. "Guillain-Barre Syndrome". Medscape Reference. Retrieved 3 Jan 2012. 2. ^ McKhann GM, Cornblath DR, Ho T, et al (1991). "Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China". Lancet 338 (8767): 5937. doi:10.1016/0140-6736(91)90606-P. PMID 1679153. 3. ^ Ho TW, Mishu B, Li CY, et al (1995). "Guillain-Barr syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies". Brain 118 (3): 597605. doi:10.1093/brain/118.3.597. PMID 7600081.
4. ^ Griffin JW, Li CY, Ho TW, et al (1995). "GuillainBarr syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases". Brain 118 (3): 57795. doi:10.1093/brain/118.3.577. PMID 7600080. 5. ^ Burt, Christiana C.; Arrowsmith, Joseph E. (1 November 2009). "Respiratory failure". Surgery (Oxford) 27 (11): 475479. doi:10.1016/j.mpsur.2009.09.007. 6. ^ CW, Ang; Jacobs BC, Laman JD (1). "Guillain-Barr syndrome: a true case of molecular mimicry.". Trends in Immunoology 25 (2): 6166. doi:10.1016/j.it.2003.12.004. 7. ^ Yuki N (June 2008). "[Campylobacter genes responsible for the development and determinant of clinical features of Guillain-Barr syndrome]" (in Japanese). Nippon Rinsho. Japanese Journal of Clinical Medicine 66 (6): 120510. PMID 18540372. 8. ^ Kuwabara S. et al. (2004-08-10). "Does Campylobacter jejuni infection elicit "demyelinating" Guillain-Barr syndrome?". Neurology (Lippincott Williams & Wilkins) 63 (3): 52933. doi:10.1212/01.WNL.0000133205.05169.04. PMID 15304587. 9. ^ Orlikowski D; Porcher, R.; Sivadon-Tardy, V.; Quincampoix, J.-C.; Raphael, J.-C.; Durand, M.-C.; Sharshar, T.; Roussi, J. et al. (April 2011). "GuillainBarr Syndrome following Primary Cytomegalovirus Infection: A Prospective Cohort Study". Clin Infect Dis. 52 (7): 837844. doi:10.1093/cid/cir074. PMID 21427390. 10. ^ Sivadon-Tardy V. et al. (Jan. 1 2009). "Guillain-Barr syndrome and influenza virus infection". Clinical Infectious Diseases (The University of Chicago Press) 48 (1): 4856. doi:10.1086/594124. PMID 19025491. 11. ^ Haber P et al. (Nov 24, 2004). "Guillain-Barr syndrome following influenza vaccination". JAMA 292 (20): 247881. doi:10.1001/jama.292.20.2478. PMID 15562126. 12. ^ Lehmann HC P et al. (Sept 2010). "Guillain-Barr syndrome following influenza vaccination". Lancet Infect Dis 10 (9): 64351. doi:10.1016/S1473-3099(10)70140-7. PMID 20797646. 13. ^ Liang et al; Li, Li; Liu, Da-Wei; Li, Ke-Li; Wu, Wen-Di; Zhu, Bao-Ping; Wang, HuaQing; Luo, Hui-Ming et al. (February 2011). "Safety of Influenza A (H1N1) Vaccine in Postmarketing Surveillance in China". New England Journal of Medicine 364 (7): 638 647. doi:10.1056/NEJMoa1008553. PMID 21288090. 14. ^ a b c van Doorn PA, Ruts L, Jacobs BC (October 2008). "Clinical features, pathogenesis, and treatment of Guillain-Barr syndrome". Lancet Neurol 7 (10): 93950. doi:10.1016/S1474-4422(08)70215-1. PMID 18848313. 15. ^ a b c Lerner AJ. Diagnostic Criteria in Neurology (Current Clinical Neurology). Totowa, NJ: Humana Press. p. 186. ISBN 1-61737-594-2. 16. ^ Merck Manual [Online]. Peripheral Neuropathy, Treatment. Retrieved 8-22-2009. 17. ^ a b c Hughes RA, Wijdicks EF, Barohn R, et al. (September 2003). "Practice parameter: immunotherapy for Guillain-Barr syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology". Neurology 61 (6): 73640. doi:10.1212/WNL.61.6.736. PMID 14504313. 18. ^ Davidson, I., Wilson, C., Walton, T., & Brissenden, S. (2009). Physiotherapy and guillain-barre syndrome: Results of a national survey. Physiotherapy, 95(3), 157163.Davidson, I.; Wilson, C.; Walton, T.; Brissenden, S. (2009). "Physiotherapy and GuillainBarr syndrome: Results of a national survey". Physiotherapy 95 (3): 157163. doi:10.1016/j.physio.2009.04.001. PMID 19635334. edit
19. ^ Karavatas, S. G. (2005). The role of neurodevelopmental sequencing in the physical therapy management of a geriatric patient with guillain-barre syndrome. Topics in Geriatric Rehabilitation, 21(2), 133-135. 20. ^ Pithadia AB, Kakadia N. (March-April 2010). "Guillain-Barr syndrome (GBS)". Pharmacol Rep 62 (2): 22032. PMID 20508277. 21. ^ Brooks, H; Christian AS; May AE (2000). "Pregnancy, anaesthesia and Guillain-Barr syndrome". Anaesthesia 55 (9): 8948. doi:10.1046/j.1365-2044.2000.01367.x. PMID 10947755. 22. ^ Iannello, S (2004). GuillainBarr syndrome: Pathological, clinical and therapeutical aspects. Nova Publishers. ISBN 1-59454-170-1. 23. ^ Guillain-Barr-Strohl syndrome and Miller Fisher's syndrome at Who Named It? 24. ^ Fisher CM (1956). "An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmolplegia, ataxia and areflexia)". N. Engl. J. Med. 255 (2): 5765. doi:10.1056/NEJM195607122550201. PMID 13334797. 25. ^ "Plastic Trees vocalist, Arimura Ryutaro recovers from "Guillain-Barre syndrome"". Tokyohive.com. 2011-01-20. Retrieved 2012-10-20. 26. ^ Wallace, Sam (2002-08-10). "Grateful Babbel a tower of strength again". London: Telegraph. Retrieved 2009-11-23. 27. ^ Lea, Robert (2002-10-17). "Relative Values: Tony and Josh Benn". London: The Times. Retrieved 2009-01-15. 28. ^ "Gaby, A True Story (1987)". Films involving Disabilities. 29. ^ Los Angeles Times, "Tom Edlefsen Beats Virus", 30 June 1968 30. ^ The case of Sam Goldstein and the swine flu vaccine, jta.org, May 5, 2009 31. ^ "Andy in Guideposts Magazine". 32. ^ Vogel, Speed; Heller, Joseph (2004). No Laughing Matter. New York: Simon & Schuster. ISBN 0-7432-4717-5. 33. ^ "Luci Baines Johnson hospitalized with nervous system disorder". 34. ^ Raley, Dan (2004-09-02). "The untold story of Hugh McElhenny, the King of Montlake". Seattle PI. Retrieved 2010-01-07. 35. ^ "Scott McKenzie Dead". Celebritydiagnosis.com. 2012-08-21. Retrieved 2012-10-20. 36. ^ "Lucky Oceans in hospital". The Australian. 2008-10-13. Retrieved 2008-10-28. 37. ^ "Chris Mortensen on Len Pasquarelli's comeback". ESPN.com. 2009-01-26. Retrieved 2009-01-26. 38. ^ Serge Payer Foundation, Serge Payer Foundation Mission. 39. ^ (2008-09-08). YumaSun.com http://www.yumasun.com/sports/tatum_44249___article.html/perry_night.html. Retrieved 2008-10-28. 40. ^ Georgena Sil (1993). "Coming to Grips with Guillain-Barr Syndrome". The Medical Post 29 (5): 32. 41. ^ "Norton Simon Biography". Retrieved 13 October 2009. 42. ^ Sutton, Jessica. "Kelly-Marie Stewart: disability and motherhood". Retrieved 3 January 2013. 43. ^ Kozinn, Allan (2004-08-31). "Hans Vonk, 63, Conductor Of the St. Louis Symphony". The New York Times. Retrieved 2009-08-26. 44. ^ http://news.bbc.co.uk/sport1/hi/football/teams/c/celtic/1002183.stm
45. ^ Dooley, Pat. "Wuerffel hospitalized to treat nervous system disorder". Gatorsports.com. Retrieved 16 June 2011. 2. Guillain-Barre (ghee-YA-buh-RAY) syndrome is a disorder in which your body's immune system attacks your nerves. Weakness and tingling in your extremities are usually the first symptoms. These sensations can quickly spread, eventually paralyzing your whole body. In its most severe form, Guillain-Barre syndrome is a medical emergency requiring hospitalization. 3. The exact cause of Guillain-Barre syndrome is unknown, but it is often preceded by an infectious illness such as a respiratory infection or the stomach flu. Luckily, GuillainBarre syndrome is uncommon, affecting only 1 or 2 people per 100,000. 4. There's no known cure for Guillain-Barre syndrome, but several treatments can ease symptoms and reduce the duration of the illness. Most people recover from GuillainBarre syndrome, though some may experience lingering effects from it, such as weakness, numbness or fatigue.
Guillain-Barre syndrome
Email this page to a friend Share on facebook Share on twitter Bookmark & Share Printerfriendly version Guillain-Barre syndrome is a serious disorder that occurs when the body's defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes muscle weakness and other symptoms.
Causes
Guillain-Barre syndrome is an autoimmune disorder (the body's immune system attacks itself). Exactly what triggers Guillain-Barre syndrome is unknown. The syndrome may occur at any age, but is most common in people of both sexes between ages 30 and 50. It often follows a minor infection, such as a lung infection or gastrointestinal infection. Most of the time, signs of the original infection have disappeared before the symptoms of Guillain-Barre begin. It may also appear a few days or weeks after a surgery. The swine flu vaccination in 1976 may have caused rare cases of Guillain-Barre syndrome. However, the swine flu and the regular flu vaccines used today have not resulted in more cases of the illness. Guillain-Barre syndrome damages parts of nerves. This nerve damage causes tingling, muscle weakness, and paralysis. Guillain-Barre syndrome most often affects the nerve's covering (myelin sheath). Such damage is called demyelination, and it causes nerve signals to move more slowly. Damage to other parts of the nerve can cause the nerve to stop working altogether. Guillain-Barre syndrome may occur along with viral infections such as:
It may also occur with other medical conditions such as systemic lupus erythematosus or Hodgkin's disease. Some people may get Guillain-Barre syndrome after a bacterial infection. A similar syndrome may occur after surgery, or when someone is critically ill (neuropathy of critical illness). This syndrome can occur at any age, but is rare in children under age 2.
Symptoms
Symptoms of Guillain-Barre can get worse very quickly. It may take only a few hours to reach the most severe symptoms, but weakness that increases over several days is also common. Muscle weakness or the loss of muscle function (paralysis) affects both sides of the body. In most cases, the muscle weakness starts in the legs and then spreads to the arms. This is called ascending paralysis. Patients may notice tingling, foot or hand pain, and clumsiness. If the inflammation affects the nerves to the diaphragm and chest and there is weakness in those muscles, the person may need breathing assistance. Typical symptoms include:
Loss of reflexes in the arms and legs Low blood pressure or poor blood pressure control Muscle weakness or loss of muscle function (paralysis) o In mild cases, there may be weakness instead of paralysis o May begin in the arms and legs at the same time o May get worse over 24 to 72 hours o May occur in the nerves of the head only o May start in the arms and move downward o May start in the feet and legs and move up to the arms and head Numbness Sensation changes, including pain and tingling Tenderness or muscle pain (may be a cramp-like pain) Uncoordinated movement (cannot walk without help)
Blurred vision and double vision Clumsiness and falling Difficulty moving face muscles
Breathing temporarily stops Can't take a deep breath Difficulty breathing Difficulty swallowing Drooling Fainting Feeling light-headed when standing
Cerebrospinal fluid sample ("spinal tap") ECG Electromyography (EMG) tests the electrical activity in muscles Nerve conduction velocity test Pulmonary function tests
Treatment
There is no cure for Guillain-Barre syndrome. However, many treatments are available to help reduce symptoms, treat complications, and speed up recovery. When symptoms are severe, the patient will need to go to the hospital for treatment, which may include artificial breathing support. In the early stages of the illness, treatments that remove or block the proteins that attack the nerve cells, called antibodies, may reduce the severity and duration of Guillain-Barre symptoms.
One method is called plasmapheresis, and it is used to remove the antibodies from the blood. The process involves taking blood from the body, usually from the arm, pumping it into a machine that removes the antibodies, and then sending it back into the body. A second method is to block the antibodies using high-dose immunoglobulin therapy (IVIG). In this case, the immunoglobulins are added to the blood in large quantities, blocking the antibodies that cause inflammation. Other treatments are directed at preventing complications.
Blood thinners may be used to prevent blood clots. If the diaphragm is weak, breathing support or even a breathing tube and ventilator may be needed. Pain is treated with anti-inflammatory medicines and narcotics, if needed. Proper body positioning or a feeding tube may be used to prevent choking during feeding if the muscles used for swallowing are weak.
Support Groups
Guillain-Barre Syndrome Foundation International - www.gbs-cidp.org
Outlook (Prognosis)
Recovery can take weeks, months, or years. Most people survive and recover completely. According to the National Institute of Neurological Disorders and Stroke, about 30% of patients still have some weakness after 3 years. Mild weakness may persist for some people. A patient's outcome is most likely to be very good when the symptoms go away within 3 weeks after they first started.
Possible Complications
Breathing difficulty (respiratory failure) Contractures of joints or other deformity Deep vein thrombosis (blood clots that form when someone is inactive or confined to bed) Increased risk of infections Low or unstable blood pressure Paralysis that is permanent Pneumonia Skin damage (ulcers) Sucking food or fluids into the lungs (aspiration)
Seek immediate medical help if you have any of the following symptoms:
Can't take a deep breath Decreased feeling (sensation) o Difficulty breathing o Difficulty swallowing o Fainting o Loss of movement
Alternative Names
Landry-Guillain-Barre syndrome; Acute idiopathic polyneuritis; Infectious polyneuritis; Acute inflammatory polyneuropathy; Acute inflammatory demyelinating polyneuropathy
References
Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for GuillainBarre syndrome. Cochrane Database Syst Rev. 2009;(1):CD002063. Ryszard M. Pluta, Cassio Lynm, Robert M. Golub. Guillain-Barr Syndrome. JAMA. 2011;305(3):319-319. Shy ME. Peripheral neuropathies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 446.
Guillain-Barr Syndrome
(GBS) In this factsheet:
The Facts on Guillain-Barr Syndrome Causes of Guillain-Barr Syndrome Symptoms and Complications of Guillain-Barr Syndrome Diagnosing Guillain-Barr Syndrome Treating and Preventing Guillain-Barr Syndrome
People with GBS suffer from a wide range of symptoms that may include:
weakness in leg, arm, and facial muscles problems with speech and swallowing pain in the muscles of the back shortness of breath decreased ability to move the eyes
Symptoms typically progress over a period of a few days and are usually at their worst 2 weeks after onset. A person experiencing a mild case of GBS may only have slight weakness or numbness and may not require hospitalization or treatment. In more severe cases, profound weakness, respiratory difficulties, or unstable blood pressure may develop, requiring admission to a hospital or an intensive care unit and possibly assistance from a breathing machine called a ventilator. The majority of people with GBS recover completely, but some people may experience residual symptoms. Sometimes GBS can recur.
Guillain-Barr Syndrome
(GBS) In this factsheet:
The Facts on Guillain-Barr Syndrome Causes of Guillain-Barr Syndrome Symptoms and Complications of Guillain-Barr Syndrome Diagnosing Guillain-Barr Syndrome Treating and Preventing Guillain-Barr Syndrome
To test how well your nerves transmit signals to muscles, doctors use a test called electromyography (EMG) and nerve conduction studies. Using small electrodes placed in and on the skin, the technician can measure the speed and strength of electrical messages being sent along the nerve. Nerves damaged by GBS conduct signals at a slower speed. To examine the cerebrospinal fluid (CSF), doctors may perform a spinal tap (also called a lumbar puncture). This fluid surrounds the brain and spinal cord. Examining your CSF may give your doctor clues to help with the diagnosis.
Guillain-Barr Syndrome
(GBS) In this factsheet:
The Facts on Guillain-Barr Syndrome Causes of Guillain-Barr Syndrome Symptoms and Complications of Guillain-Barr Syndrome Diagnosing Guillain-Barr Syndrome Treating and Preventing Guillain-Barr Syndrome
To test how well your nerves transmit signals to muscles, doctors use a test called electromyography (EMG) and nerve conduction studies. Using small electrodes placed in and on the skin, the technician can measure the speed and strength of electrical messages being sent along the nerve. Nerves damaged by GBS conduct signals at a slower speed. To examine the cerebrospinal fluid (CSF), doctors may perform a spinal tap (also called a lumbar puncture). This fluid surrounds the brain and spinal cord. Examining your CSF may give your doctor clues to help with the diagnosis.
What is Guillain-Barr syndrome (GBS)? What causes GBS? Who is at risk for developing GBS? How common is GBS, and how common is it among people who have been vaccinated against seasonal influenza? What happened in 1976 with GBS and the swine flu vaccine? How do public health authorities investigate cases of GBS?
Guillain-Barr syndrome (GBS) is a rare disorder in which a persons own immune system damages their nerve cells, causing muscle weakness and sometimes paralysis. GBS can cause symptoms that last for a few weeks. Most people recover fully from GBS, but some people have permanent nerve damage. In very rare cases, people have died of GBS, usually from difficulty breathing. In the United States, for example, an estimated 3,000 to 6,000 people develop GBS each year on average, whether or not they received a vaccination.
How common is GBS, and how common is it among people who have been vaccinated against seasonal influenza?
GBS is rare. Medical events occur regardless of vaccination, and background rates are used to assess vaccine safety by comparing the expected rate of disease or death to the actual or observed rate in any given timeframe. The background rate for GBS in the U.S. is about 80 to 160 cases of GBS each week, regardless of vaccination.
What happened in 1976 with GBS and the swine flu vaccine?
In 1976 there was a small increased risk of GBS following vaccination with an influenza vaccine made to protect against a swine flu virus. The increased risk was approximately 1 additional case of GBS per 100,000 people who got the swine flu vaccine. The Institute of Medicine (IOM) conducted a thorough scientific review of this issue in 2003 and concluded that people who received the 1976 swine influenza vaccine had an increased risk for developing GBS. Scientists have multiple theories on why this increased risk may have occurred, but the exact reason for this association remains unknown. It is important to keep in mind that severe illness and death are associated with influenza, and vaccination is the best way to prevent influenza infection and its complications.
Ensuring the safety of vaccines is a high priority for CDC. Several systems are in place to monitor vaccine safety. One of these systems is the Vaccine Adverse Event Reporting System (VAERS) . CDC and the U.S. Food and Drug Administration (FDA) co-manage VAERS, which serves as an early warning system to collect voluntary reports about possible side effects that people experience following vaccinations. CDC and FDA scientists regularly review all VAERS reports and store the information in a computerized database that is monitored to detect new, unusual, or rare health events that could be possible side effects of vaccines. In addition to the normal vaccine safety monitoring systems, CDC has proactively put additional monitoring systems in place to ensure safety after licensing. Some of these systems include: actively observing persons in defined geographic areas, collaborating with professional organizations for reports of any adverse events after vaccination, and conducting thorough investigations when severe adverse events occur to determine whether they may have been associated with the vaccine. Through these numerous approaches, we are able to detect any possible risk of GBS that might be associated with the 2012-2013 flu vaccines as early as possible and take appropriate action.
Guillain-Barre syndrome
Dr Trisha Macnair
Guillain-Barre syndrome (GBS) - also known as acute post-infective polyneuritis - is a disease of the peripheral nervous system, affecting the nerves to the arms, legs, head and trunk, but not the brain or spinal cord. This article was last reviewed by Patricia Macnair in February 2013.
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Causes
Typically, Guillain-Barre develops as an autoimmune reaction following an acute infection. It's not inherited, although it's thought that genetic factors may make some people more likely to develop autoimmune conditions. Top
Symptoms
An initial episode of a headache, vomiting, fever and back and limb pain is followed by paralysis, which starts as tingling and numbness followed by increasing weakness. The paralysis is often progressive and ascending (starting with the feet and moving upwards), but the condition may come on suddenly and affect all four limbs at once. The degree of paralysis varies. There may also be a loss of sensation. Half of patients suffer pain, usually nerve pain or deep muscle pain. In about one in four cases the paralysis spreads to the respiratory nerves supplying the chest wall muscles, which are used in breathing. The person then needs to be put on a ventilator. Some patients have a similar, but longer-lasting illness called chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). It was once known as chronic Guillain-Barre but now usually regarded as a related condition. Top
Who's affected?
Guillain-Barre affects about 1,500 people every year in the UK, and about 150 develop CIDP. The exact mechanisms which cause the conditions aren't clear, but about 60 per cent of those affected will have had a throat or intestinal infection, flu or major stress within the previous two weeks. Bacteria such as Campylobacter and mycoplasma pneumonia, and viruses such as Epstein Barr virus and cytomegalovirus are some of the organisms that have been reported. This triggers the immune system, which then attacks the nerves. It rarely occurs in first-degree relatives, but familial cases have been reported and genetic similarities noted. For example, a study of Japanese people with Guillain-Barre following an intestinal infection with the bacteria Campylobacter jejuni found they were more likely to have a rare version of the gene for an immune system chemical known as tumour necrosis factor.
There is no way to detect those who may be more vulnerable to Guillain-Barre. Top
Diagnosis
The clinical picture, usually of ascending bilateral weakness, absent reflexes and mild sensory abnormality indicates the diagnosis. Other tests include:
Cerebrospinal fluid samples will show high protein levels, but few white cells. Electrodiagnostic tests will show the typical abnormal nerve electrical conduction pattern of GBS. Blood tests, electrocardiogram (ECG) and spirometry may be performed to measure the extent of the disease but are not diagnostic.
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Treatment
The symptoms usually peak about four weeks after the start of the illness. Most people with Guillain-Barre recover within three to six months, but it may take longer and some are left with a permanent disability. The main treatment is supportive, such as physiotherapy, fluids, DVT avoidance with surgical stockings and heparin, pain relief, and nursing care are vital. Immunoglobulin treatment, which probably clears the abnormal antibodies which occur as an immune response in the condition, has been shown to speed recovery. It is easier and safer than plasmphoresis, which can also be used to clear the blood of antibody. Currently steroids are not used, but some research is suggesting that, given at the same time as immunoglobulin, steroids may speed up recovery. Most people will make a full recovery, although often it can take up to a year. However 10-20 per cent of patients are left with some residual weakness, pain or reduced mobility. Unfortunately 5 per cent of patients die, usually from cardiac arrhythmias or pneumonia during the immobile phase. Guillain-Barr syndrome is an autoimmune condition in which the persons nerves are attacked by the bodys own immune defence system. As a result of the attack, the nerve insulation (myelin) and sometimes even the inner covered part of the nerve (axon) is damaged and signals are delayed or otherwise changed - this causes a spreading paralysis. The syndrome appears to be triggered by acute viral or bacterial illnesses, such as respiratory or gastrointestinal infections, occurring one to three weeks earlier. The resultant antibodies attack
the myelin sheaths that coat the nerve cells, causing paralysis, muscular weakness and strange sensations as the sensory nerves of the skin are affected. Usually the symptoms start in the feet or legs and progress up the body over a few days or weeks. The disorder can be mild, moderate or severe, with life support needed in the worst cases. The exact cause is unknown and there is no cure. Most people spontaneously recover, though some will be left with permanent disabilities. This rare illness strikes between two and eight people in every 100,000 irrespective of gender or age, although it is more common in the 30 to 50 years age group. Other names for Guillain-Barr syndrome include acute idiopathic polyneuritis, acute idiopathic polyradiculoneuritis and Landry's ascending paralysis.
Symptoms
The symptoms of Guillain-Barr syndrome include:
Muscle weakness and paralysis affecting both sides of the body. Jerky, uncoordinated movements. Numbness. Muscle aches, pains or cramps. Odd sensations such as vibrations, buzzing or crawling under the skin. Blurred vision. Dizziness. Breathing problems. The symptoms typically start in the feet or legs and progress up the body. Sometimes, the symptoms start in the arms and progress downwards. Symptoms may take a few days or weeks to progress. After the symptoms remain steady and peaked for a short time, the person starts to recover. Recovery may take six months to two years or more.
weeks prior to the onset of symptoms. The most commonly identified trigger is gastrointestinal infection with Campylobacter jejuni - one of the most common causes of food poisoning. Some of the viral infections associated with the syndrome include respiratory and gastrointestinal infections, herpes zoster, glandular fever and viral hepatitis. Other triggers may be surgery, insect bites and vaccinations. Whatever the trigger may be, the immune cells of the body are prompted to attack and destroy the insulation of the nerve cells (myelin sheath). Sometimes the nerve cell bodies are targeted too. There is no evidence to suggest that Guillain-Barr syndrome is contagious.
Diagnosis methods
Guillain-Barr syndrome can be difficult to diagnose because the symptoms may seem vague and unrelated. Diagnosis relies on a number of tests including:
Physical examination Muscle strength tests Muscle activity tests Reflex tests, such as the knee-jerk reaction Nerve conduction velocity tests Spinal tap, to check for higher than expected levels of protein in the cerebrospinal fluid.
Treatment options
The progress of the disorder is very difficult to predict. Most people diagnosed with GuillainBarr syndrome are hospitalised so that any complications which affect their vital functions can be treated promptly. There is no cure, but treatment options include:
Plasmapheresis - blood is taken from the patient. The immune cells are removed, and the remaining red blood cells are returned to the body. Gammaglobulin (IVIG) - trials have proven the effectiveness of this form of treatment. IVIG is given by infusion into a vein, usually every day for five days. Each infusion takes about two hours.
Things to remember
Guillain-Barr syndrome is a form of nerve inflammation. The cause is unknown, but most cases seem to be triggered by a bacterial or viral illness. Most people recover, but it may take six months to two years or more. There is no cure.