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{{Myasthenia gravis}}
{{Myasthenia gravis}}
{{CMG}}
{{CMG}} ; {{AE}} {{Fs}}


==Overview==
==Overview==
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 muscles weakness.


==Physical Examination==
==Physical Examination==
Line 9: Line 10:


==== Appearance of the Patient: ====
==== Appearance of the Patient: ====
the characteristic facial expression of a MG patient is downward lip corners and depress face.<ref>{{Howard JF, Jr. Myasthenia gravis in the elderly. In: Mark MH, Sage JI (eds.) Practical Neurology of the Elderly. 1st ed. Marcel Dekker, New York, 1996, pp. 189–236}}</ref>(40) The other characteristics of myasthenia gravis is asymmetrical ptosis, worsen with sustained activity. to compensate this symptom the frontalis muscle will contract unilaterally. the other sign which we can see in the face of MG patients are incomplete eye closure.(30-31) Cogan's lid twitch is seen in this patient when they want to elevate their eyelid after rest. the eyelid will elevate and quickly drops.(32) They also have lid retraction in normal eyelid to compensate ptosis.(38) The peek sign in present in these patients when they try to close their eyes and the white sclera appears and eyes start to open slowly.(39)
* The characteristic facial expression of a MG patient is downward lip corners and depress face.<ref>{{Howard JF, Jr. Myasthenia gravis in the elderly. In: Mark MH, Sage JI (eds.) Practical Neurology of the Elderly. 1st ed. Marcel Dekker, New York, 1996, pp. 189–236}}</ref>
* The other characteristics of myasthenia gravis is asymmetrical [[ptosis]], worsen with sustained activity. to compensate this [[symptom]] the [[frontalis muscle]] will [[Contraction|contract]] unilaterally.  
* The other [[Medical sign|sign]] which we can see in the face of MG patients are incomplete eye closure.<ref name="pmid974056">{{cite journal |vauthors=Yamazaki A, Ishikawa S |title=Abnormal pupillary responses in myasthenia gravis. A pupillographic study |journal=Br J Ophthalmol |volume=60 |issue=8 |pages=575–80 |date=August 1976 |pmid=974056 |pmc=1042752 |doi= |url=}}</ref><ref name="pmid228589">{{cite journal |vauthors=Lepore FE, Sanborn GE, Slevin JT |title=Pupillary dysfunction in myasthenia gravis |journal=Ann. Neurol. |volume=6 |issue=1 |pages=29–33 |date=July 1979 |pmid=228589 |doi=10.1002/ana.410060107 |url=}}</ref>
* Cogan's lid twitch is seen in this patient when they want to elevate their eyelid after rest. the eyelid will elevate and quickly drops. They also have lid retraction in normal eyelid to compensate [[ptosis]].<ref name="pmid14318498">{{cite journal |vauthors=COGAN DG |title=MYASTHENIA GRAVIS: A REVIEW OF THE DISEASE AND A DESCRIPTION OF LID TWITCH AS A CHARACTERISTIC SIGN |journal=Arch. Ophthalmol. |volume=74 |issue= |pages=217–21 |date=August 1965 |pmid=14318498 |doi= |url=}}</ref><ref name="pmid6019006">{{cite journal |vauthors=Gay AJ, Salmon ML, Windsor CE |title=Hering's law, the levators, and their relationship in disease states |journal=Arch. Ophthalmol. |volume=77 |issue=2 |pages=157–60 |date=February 1967 |pmid=6019006 |doi= |url=}}</ref>
* The peek sign in present in these patients when they try to close their eyes and the white [[sclera]] appears and eyes start to open slowly.<ref name="pmid426681">{{cite journal |vauthors=Osher RH, Griggs RC |title=Orbicularis fatigue: the 'peek' sign of myasthenia gravis |journal=Arch. Ophthalmol. |volume=97 |issue=4 |pages=677–9 |date=April 1979 |pmid=426681 |doi= |url=}}</ref>


==== Neck: ====
==== Neck: ====
Weakness of oropharyngeal muscles causes nasal voice, regurgitation of water from nose, difficulty in swallowing and chewing. laryngeal muscle weakness causes hoarseness.(40)  
* Weakness of [[oropharyngeal]] muscles causes [[nasal voice]], regurgitation of water from nose, difficulty in [[swallowing]] and [[chewing]]. [[laryngeal]] muscle weakness causes [[hoarseness]].<ref>{{Howard JF, Jr. Myasthenia gravis in the elderly. In: Mark MH, Sage JI (eds.) Practical Neurology of the Elderly. 1st ed. Marcel Dekker, New York, 1996, pp. 189–236}}</ref>


==== Lungs: ====
==== Lungs: ====
MG patients may experience respiratory muscle weakness especially in crises.(57)
* MG patients may experience respiratory muscle weakness especially in crises.<ref name="pmid1495652">{{cite journal |vauthors=Nagappan R, Kletchko S |title=Myasthenia gravis presenting as respiratory failure |journal=N. Z. Med. J. |volume=105 |issue=932 |pages=152 |date=April 1992 |pmid=1495652 |doi= |url=}}</ref>
 
==== Neuromuscular: ====
==== Neuromuscular: ====
Dropped head syndrome is seen in these patients in the result of head weight and weakness of head extensor and flexors.<ref name="pmid28000322">{{cite journal |vauthors=Sih M, Soliven B, Mathenia N, Jacobsen J, Rezania K |title=Head-drop: A frequent feature of late-onset myasthenia gravis |journal=Muscle Nerve |volume=56 |issue=3 |pages=441–444 |date=September 2017 |pmid=28000322 |doi=10.1002/mus.25526 |url=}}</ref> To compensate this problem posterior neck muscles should work more and this result in aching neck muscles. The weakness in this disease is more prominent in proximal muscles and in arms. Other muscles such as wrist and finger extensors and foot dorsiflexors can be affected too.<ref name="pmid12911465">{{cite journal |vauthors=Werner P, Kiechl S, Löscher W, Poewe W, Willeit J |title=Distal myasthenia gravis frequency and clinical course in a large prospective series |journal=Acta Neurol. Scand. |volume=108 |issue=3 |pages=209–11 |date=September 2003 |pmid=12911465 |doi= |url=}}</ref>
* Dropped head syndrome is seen in these patients in the result of head weight and weakness of head [[extensor]] and [[Flexor|flexors]].<ref name="pmid28000322">{{cite journal |vauthors=Sih M, Soliven B, Mathenia N, Jacobsen J, Rezania K |title=Head-drop: A frequent feature of late-onset myasthenia gravis |journal=Muscle Nerve |volume=56 |issue=3 |pages=441–444 |date=September 2017 |pmid=28000322 |doi=10.1002/mus.25526 |url=}}</ref> To compensate this problem posterior neck muscles should work more and this result in aching neck muscles.
* The weakness in this disease is more prominent in proximal muscles and in arms. Other muscles such as wrist and finger [[Extensor|extensors]] and foot [[Dorsiflexion|dorsiflexors]] can be affected too.<ref name="pmid12911465">{{cite journal |vauthors=Werner P, Kiechl S, Löscher W, Poewe W, Willeit J |title=Distal myasthenia gravis frequency and clinical course in a large prospective series |journal=Acta Neurol. Scand. |volume=108 |issue=3 |pages=209–11 |date=September 2003 |pmid=12911465 |doi= |url=}}</ref>


It has been suggested that ice pack and edrophonium test are parts of neurological examination of a suspected patient.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref>
* It has been suggested that ice pack and [[edrophonium]] test are parts of neurological examination of a suspected patient.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref>


Ice pack test: Based on the fact that this test was designed by the fact that cooling can improve NMJ transmission, it can be used only in patients with ptosis since the eyelid muscle is the most accessible muscle to be cooled. We place the ice pack on closed lid for a couple of minutes and the assess the degree of ptosis in the patient. The sensitivity of this test is almost 80 percent.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref><ref name="pmid10406606">{{cite journal |vauthors=Golnik KC, Pena R, Lee AG, Eggenberger ER |title=An ice test for the diagnosis of myasthenia gravis |journal=Ophthalmology |volume=106 |issue=7 |pages=1282–6 |date=July 1999 |pmid=10406606 |doi=10.1016/S0161-6420(99)00709-5 |url=}}</ref><ref name="pmid15529523">{{cite journal |vauthors=Larner AJ |title=The place of the ice pack test in the diagnosis of myasthenia gravis |journal=Int. J. Clin. Pract. |volume=58 |issue=9 |pages=887–8 |date=September 2004 |pmid=15529523 |doi= |url=}}</ref>
* Ice pack test: Based on the fact that this test was designed by the fact that cooling can improve [[Neuromuscular junction|NMJ]] transmission, it can be used only in patients with [[ptosis]] since the [[eyelid]] muscle is the most accessible muscle to be cooled. We place the ice pack on closed lid for a couple of minutes and the assess the degree of [[ptosis]] in the patient. The sensitivity of this test is almost 80 percent.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref><ref name="pmid10406606">{{cite journal |vauthors=Golnik KC, Pena R, Lee AG, Eggenberger ER |title=An ice test for the diagnosis of myasthenia gravis |journal=Ophthalmology |volume=106 |issue=7 |pages=1282–6 |date=July 1999 |pmid=10406606 |doi=10.1016/S0161-6420(99)00709-5 |url=}}</ref><ref name="pmid15529523">{{cite journal |vauthors=Larner AJ |title=The place of the ice pack test in the diagnosis of myasthenia gravis |journal=Int. J. Clin. Pract. |volume=58 |issue=9 |pages=887–8 |date=September 2004 |pmid=15529523 |doi= |url=}}</ref>


Edrophonium test: This test is used in patients with obvious ptosis or ophthalmoparesis. We begin with 2 mg of edriphonium and add another 2 mg every 60 second to the total dosage of 10 mg. this test can have serious muscarinic side effect so we begin with low dose edrophonium since so many patients will respond to drug in 4 to 6 mg dose and by this way we can reduce the side effects of the drug. The sensitivity of this test is 80 to 90 percent. A positive edrophonium test can be seen in other conditions such as motor neuron disease, brainstem tumors, and compressive cranial neuropathies.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref><ref name="pmid27922503">{{cite journal |vauthors=Nicolle MW |title=Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome |journal=Continuum (Minneap Minn) |volume=22 |issue=6, Muscle and Neuromuscular Junction Disorders |pages=1978–2005 |date=December 2016 |pmid=27922503 |doi=10.1212/CON.0000000000000415 |url=}}</ref><ref name="pmid12870109">{{cite journal |vauthors=Pascuzzi RM |title=The edrophonium test |journal=Semin Neurol |volume=23 |issue=1 |pages=83–8 |date=March 2003 |pmid=12870109 |doi=10.1055/s-2003-40755 |url=}}</ref>
* [[Edrophonium]] test: This test is used in patients with obvious [[ptosis]] or [[ophthalmoparesis]]. We begin with 2 mg of [[edrophonium]] and add another 2 mg every 60 second to the total dosage of 10 mg. this test can have serious [[muscarinic]] side effect so we begin with low dose [[edrophonium]] since so many patients will respond to drug in 4 to 6 mg dose and by this way we can reduce the side effects of the drug. The sensitivity of this test is 80 to 90 percent. A positive [[edrophonium]] test can be seen in other conditions such as [[motor neuron]] disease, [[Brain stem|brainstem]] tumors, and compressive cranial neuropathies.<ref name="pmid16793269">{{cite journal |vauthors=Benatar M |title=A systematic review of diagnostic studies in myasthenia gravis |journal=Neuromuscul. Disord. |volume=16 |issue=7 |pages=459–67 |date=July 2006 |pmid=16793269 |doi=10.1016/j.nmd.2006.05.006 |url=}}</ref><ref name="pmid27922503">{{cite journal |vauthors=Nicolle MW |title=Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome |journal=Continuum (Minneap Minn) |volume=22 |issue=6, Muscle and Neuromuscular Junction Disorders |pages=1978–2005 |date=December 2016 |pmid=27922503 |doi=10.1212/CON.0000000000000415 |url=}}</ref><ref name="pmid12870109">{{cite journal |vauthors=Pascuzzi RM |title=The edrophonium test |journal=Semin Neurol |volume=23 |issue=1 |pages=83–8 |date=March 2003 |pmid=12870109 |doi=10.1055/s-2003-40755 |url=}}</ref>


==References==
==References==

Latest revision as of 15:47, 15 August 2019

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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

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 muscles weakness.

Physical Examination

Physical examination of patients with myasthenia gravis is usually remarkable for:

Appearance of the Patient:

  • The characteristic facial expression of a MG patient is downward lip corners and depress face.[1]
  • The other characteristics of myasthenia gravis is asymmetrical ptosis, worsen with sustained activity. to compensate this symptom the frontalis muscle will contract unilaterally.
  • The other sign which we can see in the face of MG patients are incomplete eye closure.[2][3]
  • Cogan's lid twitch is seen in this patient when they want to elevate their eyelid after rest. the eyelid will elevate and quickly drops. They also have lid retraction in normal eyelid to compensate ptosis.[4][5]
  • The peek sign in present in these patients when they try to close their eyes and the white sclera appears and eyes start to open slowly.[6]

Neck:

Lungs:

  • MG patients may experience respiratory muscle weakness especially in crises.[8]

Neuromuscular:

  • Dropped head syndrome is seen in these patients in the result of head weight and weakness of head extensor and flexors.[9] To compensate this problem posterior neck muscles should work more and this result in aching neck muscles.
  • The weakness in this disease is more prominent in proximal muscles and in arms. Other muscles such as wrist and finger extensors and foot dorsiflexors can be affected too.[10]
  • It has been suggested that ice pack and edrophonium test are parts of neurological examination of a suspected patient.[11]
  • Ice pack test: Based on the fact that this test was designed by the fact that cooling can improve NMJ transmission, it can be used only in patients with ptosis since the eyelid muscle is the most accessible muscle to be cooled. We place the ice pack on closed lid for a couple of minutes and the assess the degree of ptosis in the patient. The sensitivity of this test is almost 80 percent.[11][12][13]
  • Edrophonium test: This test is used in patients with obvious ptosis or ophthalmoparesis. We begin with 2 mg of edrophonium and add another 2 mg every 60 second to the total dosage of 10 mg. this test can have serious muscarinic side effect so we begin with low dose edrophonium since so many patients will respond to drug in 4 to 6 mg dose and by this way we can reduce the side effects of the drug. The sensitivity of this test is 80 to 90 percent. A positive edrophonium test can be seen in other conditions such as motor neuron disease, brainstem tumors, and compressive cranial neuropathies.[11][14][15]

References

  1. Template:Howard JF, Jr. Myasthenia gravis in the elderly. In: Mark MH, Sage JI (eds.) Practical Neurology of the Elderly. 1st ed. Marcel Dekker, New York, 1996, pp. 189–236
  2. Yamazaki A, Ishikawa S (August 1976). "Abnormal pupillary responses in myasthenia gravis. A pupillographic study". Br J Ophthalmol. 60 (8): 575–80. PMC 1042752. PMID 974056.
  3. Lepore FE, Sanborn GE, Slevin JT (July 1979). "Pupillary dysfunction in myasthenia gravis". Ann. Neurol. 6 (1): 29–33. doi:10.1002/ana.410060107. PMID 228589.
  4. COGAN DG (August 1965). "MYASTHENIA GRAVIS: A REVIEW OF THE DISEASE AND A DESCRIPTION OF LID TWITCH AS A CHARACTERISTIC SIGN". Arch. Ophthalmol. 74: 217–21. PMID 14318498.
  5. Gay AJ, Salmon ML, Windsor CE (February 1967). "Hering's law, the levators, and their relationship in disease states". Arch. Ophthalmol. 77 (2): 157–60. PMID 6019006.
  6. Osher RH, Griggs RC (April 1979). "Orbicularis fatigue: the 'peek' sign of myasthenia gravis". Arch. Ophthalmol. 97 (4): 677–9. PMID 426681.
  7. Template:Howard JF, Jr. Myasthenia gravis in the elderly. In: Mark MH, Sage JI (eds.) Practical Neurology of the Elderly. 1st ed. Marcel Dekker, New York, 1996, pp. 189–236
  8. Nagappan R, Kletchko S (April 1992). "Myasthenia gravis presenting as respiratory failure". N. Z. Med. J. 105 (932): 152. PMID 1495652.
  9. Sih M, Soliven B, Mathenia N, Jacobsen J, Rezania K (September 2017). "Head-drop: A frequent feature of late-onset myasthenia gravis". Muscle Nerve. 56 (3): 441–444. doi:10.1002/mus.25526. PMID 28000322.
  10. Werner P, Kiechl S, Löscher W, Poewe W, Willeit J (September 2003). "Distal myasthenia gravis frequency and clinical course in a large prospective series". Acta Neurol. Scand. 108 (3): 209–11. PMID 12911465.
  11. 11.0 11.1 11.2 Benatar M (July 2006). "A systematic review of diagnostic studies in myasthenia gravis". Neuromuscul. Disord. 16 (7): 459–67. doi:10.1016/j.nmd.2006.05.006. PMID 16793269.
  12. Golnik KC, Pena R, Lee AG, Eggenberger ER (July 1999). "An ice test for the diagnosis of myasthenia gravis". Ophthalmology. 106 (7): 1282–6. doi:10.1016/S0161-6420(99)00709-5. PMID 10406606.
  13. Larner AJ (September 2004). "The place of the ice pack test in the diagnosis of myasthenia gravis". Int. J. Clin. Pract. 58 (9): 887–8. PMID 15529523.
  14. Nicolle MW (December 2016). "Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome". Continuum (Minneap Minn). 22 (6, Muscle and Neuromuscular Junction Disorders): 1978–2005. doi:10.1212/CON.0000000000000415. PMID 27922503.
  15. Pascuzzi RM (March 2003). "The edrophonium test". Semin Neurol. 23 (1): 83–8. doi:10.1055/s-2003-40755. PMID 12870109.

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