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{{WikiDoc CMG}}; {{AE}}, {{Fs}}, [[User:MoisesRomo|Moises Romo, M.D.]]


{{SK}}
{{SK}}<nowiki/>''Myasthenia gravis'' ''management, Myasthenia gravis workup, Myasthenia gravis approach, approach to Myasthenia gravis, Myasthenia gravis treatment''
==Overview==
==Overview==
'''[[Myasthenia gravis]]''' is a [https://www.wikidoc.org/index.php/Neuromuscular_disease neuromuscular disease] leading to fluctuating [[muscle weakness]] and [https://www.wikidoc.org/index.php/Fatigue_(physical) fatiguability]. It is an [https://www.wikidoc.org/index.php/Autoimmunity autoimmune disorder], in which [[weakness]] is caused by circulating [https://www.wikidoc.org/index.php/Antibody antibodies] that block [https://www.wikidoc.org/index.php/Acetylcholine_receptor acetylcholine receptors] at the [[post-synaptic]] [https://www.wikidoc.org/index.php/Neuromuscular_junction neuromuscular junction], inhibiting the stimulative effect of the [https://www.wikidoc.org/index.php/Neurotransmitter neurotransmitter] [https://www.wikidoc.org/index.php/Acetylcholine acetylcholine]. The diagnosis is maily clinical, although ordering [[acetylcholine]] [[autoantibodies]] may sometimes be necessary. [[Physical examination]] of patients with [[myasthenia gravis]] is usually remarkable for: downward lip corners and depress face, asymmetrical [[ptosis]], incomplete eye closure, Cogan's lid twitch, peek sign, [[weakness]] of [[oropharyngeal]] [[muscles]], [[respiratory]] [[muscle]] [[weakness]], dropped head syndrome, and proximal [[muscle weakness]]. [[Thoracic]] immaging may reveal a mediastinal mass ([[thymoma]]). Edrophonium test is used in patients with obvious [[ptosis]] or [[ophthalmoparesis]]. [[Myasthenia]] is treated medically with [https://www.wikidoc.org/index.php/Cholinesterase_inhibitor cholinesterase inhibitors] or [https://www.wikidoc.org/index.php/Immunosuppressants immunosuppressants] and in selected cases with [https://www.wikidoc.org/index.php/Thymectomy thymectomy].


==Causes==
==Causes==
===Life Threatening Causes===
===Life Threatening Causes===
Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.
Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.
* [[Life threatening cause 1]]
 
* [[Life threatening cause 2]]
* [[Myasthenia gravis]] is not a life-threatening condition that may result in death or permanent disability within 24 hours if left untreated.
* [[Life threatening cause 3]]


===Common Causes===
===Common Causes===
* [[Common cause 1]]
 
* [[Common cause 2]]
*[[Thymus]] abnormalities:<ref name="pmid8190158">{{cite journal |vauthors=Drachman DB |title=Myasthenia gravis |journal=N. Engl. J. Med. |volume=330 |issue=25 |pages=1797–810 |date=June 1994 |pmid=8190158 |doi=10.1056/NEJM199406233302507 |url=}}</ref><ref name="pmid12360217">{{cite journal |vauthors=Vincent A |title=Unravelling the pathogenesis of myasthenia gravis |journal=Nat. Rev. Immunol. |volume=2 |issue=10 |pages=797–804 |date=October 2002 |pmid=12360217 |doi=10.1038/nri916 |url=}}</ref><ref name="pmid18567866">{{cite journal |vauthors=Willcox N, Leite MI, Kadota Y, Jones M, Meager A, Subrahmanyam P, Dasgupta B, Morgan BP, Vincent A |title=Autoimmunizing mechanisms in thymoma and thymus |journal=Ann. N. Y. Acad. Sci. |volume=1132 |issue= |pages=163–73 |date=2008 |pmid=18567866 |doi=10.1196/annals.1405.021 |url=}}</ref><ref name="pmid17675582">{{cite journal |vauthors=Leite MI, Jones M, Ströbel P, Marx A, Gold R, Niks E, Verschuuren JJ, Berrih-Aknin S, Scaravilli F, Canelhas A, Morgan BP, Vincent A, Willcox N |title=Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status |journal=Am. J. Pathol. |volume=171 |issue=3 |pages=893–905 |date=September 2007 |pmid=17675582 |pmc=1959483 |doi=10.2353/ajpath.2007.070240 |url=}}</ref><ref name="pmid18644632">{{cite journal |vauthors=Hohlfeld R, Wekerle H |title=Reflections on the "intrathymic pathogenesis" of myasthenia gravis |journal=J. Neuroimmunol. |volume=201-202 |issue= |pages=21–7 |date=September 2008 |pmid=18644632 |doi=10.1016/j.jneuroim.2008.05.020 |url=}}</ref>
* [[Common cause 3]]
**[[Thymic]] [[hyperplasia]] 
* [[Common cause 4]]
**[https://www.wikidoc.org/index.php/Thymoma Thymoma]
* [[Common cause 5]]
*Genetic:<ref name="pmid4544224">{{cite journal |vauthors=Feltkamp TE, van den Berg-Loonen PM, Nijenhuis LE, Engelfriet CP, van Rossum AL, van Loghem JJ, Oosterhuis HJ |title=Myasthenia gravis, autoantibodies, and HL-A antigens |journal=Br Med J |volume=1 |issue=5899 |pages=131–3 |date=January 1974 |pmid=4544224 |pmc=1633001 |doi= |url=}}</ref><ref name="pmid15004560">{{cite journal |vauthors=Bottini N, Musumeci L, Alonso A, Rahmouni S, Nika K, Rostamkhani M, MacMurray J, Meloni GF, Lucarelli P, Pellecchia M, Eisenbarth GS, Comings D, Mustelin T |title=A functional variant of lymphoid tyrosine phosphatase is associated with type I diabetes |journal=Nat. Genet. |volume=36 |issue=4 |pages=337–8 |date=April 2004 |pmid=15004560 |doi=10.1038/ng1323 |url=}}</ref><ref name="pmid17277778">{{cite journal |vauthors=Yamanouchi J, Rainbow D, Serra P, Howlett S, Hunter K, Garner VE, Gonzalez-Munoz A, Clark J, Veijola R, Cubbon R, Chen SL, Rosa R, Cumiskey AM, Serreze DV, Gregory S, Rogers J, Lyons PA, Healy B, Smink LJ, Todd JA, Peterson LB, Wicker LS, Santamaria P |title=Interleukin-2 gene variation impairs regulatory T cell function and causes autoimmunity |journal=Nat. Genet. |volume=39 |issue=3 |pages=329–37 |date=March 2007 |pmid=17277778 |pmc=2886969 |doi=10.1038/ng1958 |url=}}</ref><ref name="pmid9521619">{{cite journal |vauthors=Raknes G, Skeie GO, Gilhus NE, Aadland S, Vedeler C |title=FcgammaRIIA and FcgammaRIIIB polymorphisms in myasthenia gravis |journal=J. Neuroimmunol. |volume=81 |issue=1-2 |pages=173–6 |date=January 1998 |pmid=9521619 |doi= |url=}}</ref><ref name="pmid14597109">{{cite journal |vauthors=van der Pol WL, Jansen MD, Kuks JB, de Baets M, Leppers-van de Straat FG, Wokke JH, van de Winkel JG, van den Berg LH |title=Association of the Fc gamma receptor IIA-R/R131 genotype with myasthenia gravis in Dutch patients |journal=J. Neuroimmunol. |volume=144 |issue=1-2 |pages=143–7 |date=November 2003 |pmid=14597109 |doi= |url=}}</ref><ref name="pmid11196709">{{cite journal |vauthors=Kristiansen OP, Larsen ZM, Pociot F |title=CTLA-4 in autoimmune diseases--a general susceptibility gene to autoimmunity? |journal=Genes Immun. |volume=1 |issue=3 |pages=170–84 |date=February 2000 |pmid=11196709 |doi=10.1038/sj.gene.6363655 |url=}}</ref>
**[https://www.wikidoc.org/index.php/CHRNA1 CHRNA1] [https://www.wikidoc.org/index.php/Locus Locus]
**[https://www.wikidoc.org/index.php/PTPN22 PTPN22] [https://www.wikidoc.org/index.php/Gene Gene]
**[https://www.wikidoc.org/index.php/FCGR2A FCGR2] [https://www.wikidoc.org/index.php/Locus Locus]
**[https://www.wikidoc.org/index.php/CTLA-4 CTLA4] [https://www.wikidoc.org/index.php/Locus Locus]
*Environment:<ref name="pmid12588942">{{cite journal |vauthors=Scoppetta C, Onorati P, Eusebi F, Fini M, Evoli A, Vincent A |title=Autoimmune myasthenia gravis after cardiac surgery |journal=J. Neurol. Neurosurg. Psychiatry |volume=74 |issue=3 |pages=392–3 |date=March 2003 |pmid=12588942 |pmc=1738331 |doi= |url=}}</ref><ref name="pmid16427886">{{cite journal |vauthors=Resatoglu AG, Tok M, Yemisci M, Yener N, Yener A |title=Autoimmune myasthenia gravis after coronary artery bypass surgery |journal=Ann. Thorac. Surg. |volume=81 |issue=2 |pages=725–6 |date=February 2006 |pmid=16427886 |doi=10.1016/j.athoracsur.2004.10.027 |url=}}</ref><ref name="pmid9046891">{{cite journal |vauthors=Wittbrodt ET |title=Drugs and myasthenia gravis. An update |journal=Arch. Intern. Med. |volume=157 |issue=4 |pages=399–408 |date=February 1997 |pmid=9046891 |doi= |url=}}</ref><ref name="pmid16019566">{{cite journal |vauthors=Fujimaki K, Takasaki H, Koharazawa H, Takabayashi M, Yamaji S, Baba Y, Kanamori H, Ishigatsubo Y |title=Idiopathic thrombocytopenic purpura and myasthenia gravis after fludarabine treatment for chronic lymphocytic leukemia |journal=Leuk. Lymphoma |volume=46 |issue=7 |pages=1101–2 |date=July 2005 |pmid=16019566 |doi=10.1080/10428190500063054 |url=}}</ref><ref name="pmid10874911">{{cite journal |vauthors=Ronzière T, Auzou P, Ozsancak C, Magnier P, Sénant J, Hannequin D |title=[Myasthenic syndrome induced by lithium] |language=French |journal=Presse Med |volume=29 |issue=19 |pages=1043–4 |date=2000 |pmid=10874911 |doi= |url=}}</ref><ref name="pmid16175373">{{cite journal |vauthors=Iwase T, Iwase C |title=Systemic effect of local and small-dose botulinum toxin injection to unmask subclinical myasthenia gravis |journal=Graefes Arch. Clin. Exp. Ophthalmol. |volume=244 |issue=3 |pages=415–6 |date=March 2006 |pmid=16175373 |doi=10.1007/s00417-005-0130-4 |url=}}</ref>
**Drugs:
***[https://www.wikidoc.org/index.php/D-penicillamine D-penicillamine]
***[https://www.wikidoc.org/index.php/Fludarabine Fludarabine]
***[https://www.wikidoc.org/index.php/Lithium_carbonate Lithium carbonate]
***[https://www.wikidoc.org/index.php/Botulinum_toxin Botulinum toxin]
***[https://www.wikidoc.org/index.php/Aminoglycosides Aminoglycosides],
***[https://www.wikidoc.org/index.php/Macrolide Macrolides]
***[https://www.wikidoc.org/index.php/Fluoroquinolones Fluoroquinolones]
***[https://www.wikidoc.org/index.php/Quinine Quinine]
***[https://www.wikidoc.org/index.php/Calcium_channel_blocker Calcium channel blockers],
***[https://www.wikidoc.org/index.php/Beta_blockers Beta blockers]
***[https://www.wikidoc.org/index.php/Iodinated_contrast Iodinated contrast agents]  
**Infections:
***[https://www.wikidoc.org/index.php/Hepatitis_C Hepatitis C]
***[https://www.wikidoc.org/index.php/Herpes_simplex_virus HSV]
***[https://www.wikidoc.org/index.php/Epstein_Barr_virus EBV]
***[https://www.wikidoc.org/index.php/Cytomegalovirus_infection CMV]
***[https://www.wikidoc.org/index.php/HTLV-1 HTLV1]
***[https://www.wikidoc.org/index.php/West_nile_virus West Nile virus]
**Trauma:
***[[Cardiothoracic surgery|Cardiothoracic]] [https://www.wikidoc.org/index.php/Bypass_surgery bypass surgery]  


==Diagnosis==
==Diagnosis==
Line 50: Line 74:


==Do's==
==Do's==
* The content in this section is in bullet points.
 
*The content in this section is in bullet points.


==Don'ts==
==Don'ts==
* The content in this section is in bullet points.
 
*The content in this section is in bullet points.


==References==
==References==

Revision as of 06:30, 28 January 2021


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

Synonyms and keywords:Myasthenia gravis management, Myasthenia gravis workup, Myasthenia gravis approach, approach to Myasthenia gravis, Myasthenia gravis treatment

Overview

Myasthenia gravis is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. It is an autoimmune disorder, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the post-synaptic neuromuscular junction, inhibiting the stimulative effect of the neurotransmitter acetylcholine. The diagnosis is maily clinical, although ordering acetylcholine autoantibodies may sometimes be necessary. Physical examination of patients with myasthenia gravis is usually remarkable for: downward lip corners and depress face, asymmetrical ptosis, incomplete eye closure, Cogan's lid twitch, peek sign, weakness of oropharyngeal muscles, respiratory muscle weakness, dropped head syndrome, and proximal muscle weakness. Thoracic immaging may reveal a mediastinal mass (thymoma). Edrophonium test is used in patients with obvious ptosis or ophthalmoparesis. Myasthenia is treated medically with cholinesterase inhibitors or immunosuppressants and in selected cases with thymectomy.

Causes

Life Threatening Causes

Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.

  • Myasthenia gravis is not a life-threatening condition that may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Diagnosis

Shown below is an algorithm summarizing the diagnosis of [[disease name]] according the the [...] guidelines.

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Treatment

Shown below is an algorithm summarizing the treatment of [[disease name]] according the the [...] guidelines.

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Do's

  • The content in this section is in bullet points.

Don'ts

  • The content in this section is in bullet points.

References

  1. ↑ Drachman DB (June 1994). "Myasthenia gravis". N. Engl. J. Med. 330 (25): 1797–810. doi:10.1056/NEJM199406233302507. PMID 8190158.
  2. ↑ Vincent A (October 2002). "Unravelling the pathogenesis of myasthenia gravis". Nat. Rev. Immunol. 2 (10): 797–804. doi:10.1038/nri916. PMID 12360217.
  3. ↑ Willcox N, Leite MI, Kadota Y, Jones M, Meager A, Subrahmanyam P, Dasgupta B, Morgan BP, Vincent A (2008). "Autoimmunizing mechanisms in thymoma and thymus". Ann. N. Y. Acad. Sci. 1132: 163–73. doi:10.1196/annals.1405.021. PMID 18567866.
  4. ↑ Leite MI, Jones M, Ströbel P, Marx A, Gold R, Niks E, Verschuuren JJ, Berrih-Aknin S, Scaravilli F, Canelhas A, Morgan BP, Vincent A, Willcox N (September 2007). "Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status". Am. J. Pathol. 171 (3): 893–905. doi:10.2353/ajpath.2007.070240. PMC 1959483. PMID 17675582.
  5. ↑ Hohlfeld R, Wekerle H (September 2008). "Reflections on the "intrathymic pathogenesis" of myasthenia gravis". J. Neuroimmunol. 201-202: 21–7. doi:10.1016/j.jneuroim.2008.05.020. PMID 18644632.
  6. ↑ Feltkamp TE, van den Berg-Loonen PM, Nijenhuis LE, Engelfriet CP, van Rossum AL, van Loghem JJ, Oosterhuis HJ (January 1974). "Myasthenia gravis, autoantibodies, and HL-A antigens". Br Med J. 1 (5899): 131–3. PMC 1633001. PMID 4544224.
  7. ↑ Bottini N, Musumeci L, Alonso A, Rahmouni S, Nika K, Rostamkhani M, MacMurray J, Meloni GF, Lucarelli P, Pellecchia M, Eisenbarth GS, Comings D, Mustelin T (April 2004). "A functional variant of lymphoid tyrosine phosphatase is associated with type I diabetes". Nat. Genet. 36 (4): 337–8. doi:10.1038/ng1323. PMID 15004560.
  8. ↑ Yamanouchi J, Rainbow D, Serra P, Howlett S, Hunter K, Garner VE, Gonzalez-Munoz A, Clark J, Veijola R, Cubbon R, Chen SL, Rosa R, Cumiskey AM, Serreze DV, Gregory S, Rogers J, Lyons PA, Healy B, Smink LJ, Todd JA, Peterson LB, Wicker LS, Santamaria P (March 2007). "Interleukin-2 gene variation impairs regulatory T cell function and causes autoimmunity". Nat. Genet. 39 (3): 329–37. doi:10.1038/ng1958. PMC 2886969. PMID 17277778.
  9. ↑ Raknes G, Skeie GO, Gilhus NE, Aadland S, Vedeler C (January 1998). "FcgammaRIIA and FcgammaRIIIB polymorphisms in myasthenia gravis". J. Neuroimmunol. 81 (1–2): 173–6. PMID 9521619.
  10. ↑ van der Pol WL, Jansen MD, Kuks JB, de Baets M, Leppers-van de Straat FG, Wokke JH, van de Winkel JG, van den Berg LH (November 2003). "Association of the Fc gamma receptor IIA-R/R131 genotype with myasthenia gravis in Dutch patients". J. Neuroimmunol. 144 (1–2): 143–7. PMID 14597109.
  11. ↑ Kristiansen OP, Larsen ZM, Pociot F (February 2000). "CTLA-4 in autoimmune diseases--a general susceptibility gene to autoimmunity?". Genes Immun. 1 (3): 170–84. doi:10.1038/sj.gene.6363655. PMID 11196709.
  12. ↑ Scoppetta C, Onorati P, Eusebi F, Fini M, Evoli A, Vincent A (March 2003). "Autoimmune myasthenia gravis after cardiac surgery". J. Neurol. Neurosurg. Psychiatry. 74 (3): 392–3. PMC 1738331. PMID 12588942.
  13. ↑ Resatoglu AG, Tok M, Yemisci M, Yener N, Yener A (February 2006). "Autoimmune myasthenia gravis after coronary artery bypass surgery". Ann. Thorac. Surg. 81 (2): 725–6. doi:10.1016/j.athoracsur.2004.10.027. PMID 16427886.
  14. ↑ Wittbrodt ET (February 1997). "Drugs and myasthenia gravis. An update". Arch. Intern. Med. 157 (4): 399–408. PMID 9046891.
  15. ↑ Fujimaki K, Takasaki H, Koharazawa H, Takabayashi M, Yamaji S, Baba Y, Kanamori H, Ishigatsubo Y (July 2005). "Idiopathic thrombocytopenic purpura and myasthenia gravis after fludarabine treatment for chronic lymphocytic leukemia". Leuk. Lymphoma. 46 (7): 1101–2. doi:10.1080/10428190500063054. PMID 16019566.
  16. ↑ Ronzière T, Auzou P, Ozsancak C, Magnier P, Sénant J, Hannequin D (2000). "[Myasthenic syndrome induced by lithium]". Presse Med (in French). 29 (19): 1043–4. PMID 10874911.
  17. ↑ Iwase T, Iwase C (March 2006). "Systemic effect of local and small-dose botulinum toxin injection to unmask subclinical myasthenia gravis". Graefes Arch. Clin. Exp. Ophthalmol. 244 (3): 415–6. doi:10.1007/s00417-005-0130-4. PMID 16175373.


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