Gullian barre syndrome


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autoimmune disease

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Gullian barre syndrome

  2. 2. INTRODUCTION o Popularly known as “French polio” is an acute inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia. o All forms of Guillain–Barré syndrome are autoimmune disease, due to an immune response to foreign antigens. o It has an annual incidence of 0.6 to 2.4 cases per 100,000 population and occurs at all ages and in both sexes. o With the marked decline in the incidence of polio, GuillainBarré syndrome is now the most common cause of acute flaccid paralysis in healthy people.
  3. 3. TYPES o ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY(AIDP)- autoimmune response directed against Schwann cell membranes. o MILLER FISHER SYNDROME (MFS)- Anti-GQ1b antibodies are present in 90% of cases. o ACUTE MOTOR AXONAL NEUROPATHY (AMAN) also known as Chinese paralytic syndrome- Anti-GD3 antibodies are found more frequently in AMAN. o ACUTE PANAUTONOMIC NEUROPATHY- associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias.
  4. 4. CAMPYLOBACTER INFECTION o Campylobacter infection is the most commonly identified precipitant of Guillain-Barré syndrome. o A case-control study involving 103 patients with the disease found that 26% of affected individuals had evidence of recent C. jejuni infection compared with 2% of household and 1% of age-matched controls. o Seventy percent of those infected with C. jejuni reported a diarrheal illness within 12 weeks before the onset of the neurologic illness.
  5. 5. o The main lesions are acute inflammatory demyelinating neuropathy and, particularly in patients with Campylobacter-associated disease, acute axonal degeneration. o These changes may be caused by cross-reacting antibodies to GM1 ganglioside (present in high concentrations in peripheral nerve myelin) formed in response to similar epitopes expressed by the infecting Campylobacter strain. o However, mechanisms other than molecular mimicry may be associated with the production of antibodies to GM1 ganglioside.
  6. 6. ANTIBODIES AGAINST GANGLIOSIDES o Anti-GD3-Anti-GD3 antibodies have been found in association with specific forms of Guillain-Barré syndrome. o In vivo studies of isolated anti-GM1 and GD3 antibodies indicate the antibodies can interfere with motor neuron function. o Anti-GD1a antibodies were highly associated acute motor axonal neuropathy while high titers of anti-GM1 were more frequent indicating that GD1a possibly targets the axolemma and nodes of ranvier. .
  7. 7. o Anti-GM1-Levels of anti-GM1 are elevated in patients with various forms of dementia & correlate with more severe Guillain-Barré syndrome. o Titers to GM1 in other diseases (rheumatoid arthritis, primary Sjögren's syndrome and systemic lupus erythematosus) was also elevated. o The autoimmune role of anti-GM1 is still unclear.
  8. 8. o Anti-GQ1-Anti-GQ1b are found in MillerFisher syndrome. o Studies of these antibodies reveal large disruption of the Schwann cells. o Anti-GQ1b IgG levels were elevated in patients with ophthalmoplegia in GuillainBarré syndrome.
  9. 9. PATHOGENESIS o In Guillain-Barré syndrome, the myelin sheath surrounding the axon is lost. o Demyelination is a common response of neural tissue to many agents and conditions, including physical trauma, hypoxemia, toxic chemicals, vascular insufficiency, and immunological reactions. o Loss of the myelin sheath in Guillain-Barré syndrome makes nerve impulse transmission is aborted.
  10. 10. CLINICAL MANIFESTATION o The syndrome may develop rapidly over the course of hours or days, or may take up to 3 to 4 weeks to develop. o Most patients demonstrate the greatest weakness in the first weeks of the disorder. o Patients are at their weakest point by the third week of the illness. o In the beginning, a flaccid, ascending paralysis develops quickly.
  11. 11. o The patient may first notice weakness in the lower extremities that may quickly extend to include weakness and abnormal sensations in the arms. o Deep tendon reflexes are usually lost, even in the earliest stages. o The trunk and cranial nerves may become involved. o Respiratory muscles can become affected, resulting in respiratory compromise.
  12. 12. DIAGNOSIS o The history of the onset of symptoms can be revealing because symptoms of Guillain-Barré syndrome usually begin with weakness or paresthesias of the lower extremities and ascend in a symmetrical pattern. o A lumbar puncture may be performed and reveal increased protein. o Also, nerve conduction studies record impulse transmission along the nerve fiber.
  13. 13. TREATMENT o The main modalities of therapy for Guillain-Barré syndrome include – Plasmapheresis – Administration of intravenous immune globulin o The first therapy proven to benefit patients with Guillain-Barré syndrome is plasmapheresis. o This procedure mechanically removes humoral factors.
  14. 14. o Plasma exchange is recommended for patients who – Are unable to walk unaided – Demonstrate worsening vital capacities – Require mechanical ventilation – Have significant bulbar weakness o Intravenous immunoglobulin (IVIG) is also useful in managing Guillain-Barré syndrome.
  15. 15. REFERENCE o Davids, H. "Guillain-Barre Syndrome". Medscape Reference. Retrieved 3 Jan 2012. o Jump up Mori, M; Kuwabara, S; Fukutake, T; Hattori, T (2002). "Plasmapheresis and Miller Fisher syndrome: analysis of 50 consecutive cases". Journal of neurology, neurosurgery, and psychiatry 72 (5). o Thomas J.Kindt,Barabara A.Osborne,Richard A.Goldsby.Kuby Immunology 6th edition.
  16. 16. American actor Andy Griffith developed GuillainBarré syndrome in 1983. Griffith is seen here receiving an award at the White House in 2005.