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__NOTOC__
{{Guillain-Barré syndrome}}
{{Guillain-Barré syndrome}}


{{CMG}}; '''Associate Editors-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh@perfuse.org]
{{CMG}}; {{AE}} {{Fs}}


==Overview==
==Overview==
'''Guillain-Barré syndrome''' ('''GBS''') is an acute, autoimmune, [[neuropathy|polyradiculoneuropathy]] affecting the [[peripheral nervous system]], usually triggered by an acute infectious process. With prompt treatment of [[plasmapheresis]] followed by [[immunoglobulins]] and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and [[dysautonomia]] are present.
Supportive therapy for Guillain Barre syndrome include: Respiratory assistance, [[Heart rate]] and [[blood pressure]] monitoring, prevention of [[Thromboembolic disorders|thromboembolic]] complications by [[heparin]], minimal sedation in intensive care units, [[pain]] control and early passive movements. [[Immunomodulators|Immunomodulating]] therapy for Guillain Barre syndrome include: [[Plasma]] exchange, High dose [[immunoglobulin]] and [[Corticosteroids]].


==Medical therapy==
==Medical Therapy==
 
* Treatment for Guillan Barre syndrome can be divided into two groups:
* There is no [[cure]] for [[Guillain-Barré syndrome]]. However, many [[treatments]] are available to help reduce symptoms, treat complications, and speed up recovery.
** Supportive therapy:
===Intensive care management===
*** Respiratory assistance: We measure maximal expiratory [[vital capacity]] and if [[vital capacity]] falls under 15 ml/kg, we start [[mechanical ventilation]] and [[endotracheal intubation]].  
* When symptoms are severe, the patient will need to go to the hospital for breathing help, [[treatment]], and [[physical therapy]].
*** [[Heart rate]] and [[blood pressure]] monitoring.
* Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient.
*** Prevention of [[Thromboembolic disorders|thromboembolic]] complications by [[heparin]].
* Of greatest concern is respiratory failure due to paralysis of the diaphragm.
*** Reduce respiratory infections by minimal sedation in intensive care units.  
* Early [[intubation]] should be considered in any patient with
*** [[Pain]] control by [[analgesics]].
** A [[vital capacity]] (VC) <20 ml/kg,
*** Prevention of contracture by early passive movement.
** A Negative Inspiratory Force (NIF) <-25 cmH<sub>2</sub>O
** [[Immunomodulators|Immunomodulating]] therapy
** More than 30% decrease in either VC or NIF within 24 hours
*** [[Plasma]] exchange: It is proved in so many studies that [[plasma]] exchange is an effective treatment option and can reduce recovery time.<ref name="pmid2893583">{{cite journal |vauthors= |title=Efficiency of plasma exchange in Guillain-Barré syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barré syndrome |journal=Ann. Neurol. |volume=22 |issue=6 |pages=753–61 |date=December 1987 |pmid=2893583 |doi=10.1002/ana.410220612 |url=}}</ref><ref name="pmid1642477">{{cite journal |vauthors= |title=Plasma exchange in Guillain-Barré syndrome: one-year follow-up. French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome |journal=Ann. Neurol. |volume=32 |issue=1 |pages=94–7 |date=July 1992 |pmid=1642477 |doi=10.1002/ana.410320115 |url=}}</ref>
** Rapid progression of disease
*** High dose [[immunoglobulin]]: [[IVIG]] is as effective as plasma exchange for treatment of GBS. Combination therapy with these two will not result in a netter outcome.<ref name="pmid1552913">{{cite journal |vauthors=van der Meché FG, Schmitz PI |title=A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group |journal=N. Engl. J. Med. |volume=326 |issue=17 |pages=1123–9 |date=April 1992 |pmid=1552913 |doi=10.1056/NEJM199204233261705 |url=}}</ref><ref name="pmid12499466">{{cite journal |vauthors=Dalakas MC |title=Mechanisms of action of IVIg and therapeutic considerations in the treatment of acute and chronic demyelinating neuropathies |journal=Neurology |volume=59 |issue=12 Suppl 6 |pages=S13–21 |date=December 2002 |pmid=12499466 |doi= |url=}}</ref>
** Autonomic instability
*** [[Corticosteroids]]:
 
**** In some animal models it was demonstrated that [[corticosteroids]] reduce allergic [[neuritis]].<ref name="pmid3260088">{{cite journal |vauthors=Heininger K, Schäfer B, Hartung HP, Fierz W, Linington C, Toyka KV |title=The role of macrophages in experimental autoimmune neuritis induced by a P2-specific T-cell line |journal=Ann. Neurol. |volume=23 |issue=4 |pages=326–31 |date=April 1988 |pmid=3260088 |doi=10.1002/ana.410230403 |url=}}</ref>
===Immunotherapy===
**** In another study it was demonstrated that treatment with [[corticosteroids]] alone is not effective.<ref name="pmid22786476">{{cite journal |vauthors=Hughes RA, Swan AV, van Doorn PA |title=Intravenous immunoglobulin for Guillain-Barré syndrome |journal=Cochrane Database Syst Rev |volume= |issue=7 |pages=CD002063 |date=July 2012 |pmid=22786476 |doi=10.1002/14651858.CD002063.pub5 |url=}}</ref>
* Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible.
**** In a randomized study it was demonstrated that the combination of [[IVIG]] and [[corticosteroid]] will not result in any advantage.<ref name="pmid14738791">{{cite journal |vauthors=van Koningsveld R, Schmitz PI, Meché FG, Visser LH, Meulstee J, van Doorn PA |title=Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial |journal=Lancet |volume=363 |issue=9404 |pages=192–6 |date=January 2004 |pmid=14738791 |doi= |url=}}</ref>
* Either high-dose intravenous [[immunoglobulin]]s (IVIg) at 400mg/kg for 5 days or [[plasmapheresis]] can be administered, as they are equally effective and a combination of the two is not significantly better than either alone.
* Therapy is no longer effective after 2 weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible.
* High-dose immunoglobulin therapy ([[IVIG|IVIg]]) is used to reduce the severity and length of Guillain-Barré symptoms. In this case, the [[immunoglobulins]] are added to the [[blood]] in large quantity, blocking the [[antibodies]] that cause [[inflammation]].
* IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (.4kg each day).
* 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.
* If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) is administered four times over a week. A method called [[plasmapheresis]] is used to remove [[proteins]], called [[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]], then sending it back into the body.
* [[Glucocorticoids]] have '''NOT''' been found to be effective in GBS.
 
===Other treatments for 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 aggressively 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]] for [[swallowing]] are weak.


==References==
==References==
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[[Category:Autoimmune diseases]]
[[Category:Neurological disorders]]
[[Category:Neurology]]
[[Category:Immunology]]
[[Category:Disease]]
[[Category:Emergency medicine]]

Latest revision as of 17:21, 27 December 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.

Overview

Supportive therapy for Guillain Barre syndrome include: Respiratory assistance, Heart rate and blood pressure monitoring, prevention of thromboembolic complications by heparin, minimal sedation in intensive care units, pain control and early passive movements. Immunomodulating therapy for Guillain Barre syndrome include: Plasma exchange, High dose immunoglobulin and Corticosteroids.

Medical Therapy

References

  1. "Efficiency of plasma exchange in Guillain-Barré syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barré syndrome". Ann. Neurol. 22 (6): 753–61. December 1987. doi:10.1002/ana.410220612. PMID 2893583.
  2. "Plasma exchange in Guillain-Barré syndrome: one-year follow-up. French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome". Ann. Neurol. 32 (1): 94–7. July 1992. doi:10.1002/ana.410320115. PMID 1642477.
  3. van der Meché FG, Schmitz PI (April 1992). "A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group". N. Engl. J. Med. 326 (17): 1123–9. doi:10.1056/NEJM199204233261705. PMID 1552913.
  4. Dalakas MC (December 2002). "Mechanisms of action of IVIg and therapeutic considerations in the treatment of acute and chronic demyelinating neuropathies". Neurology. 59 (12 Suppl 6): S13–21. PMID 12499466.
  5. Heininger K, Schäfer B, Hartung HP, Fierz W, Linington C, Toyka KV (April 1988). "The role of macrophages in experimental autoimmune neuritis induced by a P2-specific T-cell line". Ann. Neurol. 23 (4): 326–31. doi:10.1002/ana.410230403. PMID 3260088.
  6. Hughes RA, Swan AV, van Doorn PA (July 2012). "Intravenous immunoglobulin for Guillain-Barré syndrome". Cochrane Database Syst Rev (7): CD002063. doi:10.1002/14651858.CD002063.pub5. PMID 22786476.
  7. van Koningsveld R, Schmitz PI, Meché FG, Visser LH, Meulstee J, van Doorn PA (January 2004). "Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial". Lancet. 363 (9404): 192–6. PMID 14738791.

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