Myasthenia gravis natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
The [[age of onset]] in Myasthenia gravis follows a bimodal distribution. The early type (before age of 50) is [[female]] predominant and the late type (after age of 60) is [[male]] predominant. Between the age of 50-60 there is no significant different between [[male]] and [[female]].<ref name="pmid23893883">{{cite journal |vauthors=Alkhawajah NM, Oger J |title=Late-onset myasthenia gravis: a review when incidence in older adults keeps increasing |journal=Muscle Nerve |volume=48 |issue=5 |pages=705–10 |date=November 2013 |pmid=23893883 |doi=10.1002/mus.23964 |url=}}</ref> About 50 percent of patiens have [[ptosis]] and [[diplopia]] as their presenting sign. [[Ptosis]] is usually asymmetrical. The direction and degree of [[diplopia]] may change in a [[Myasthenia gravis|MG]] patients because of [[Fatigue|fatigable]] [[ocular]] motor [[paresis]].<ref name="pmid7369304">{{cite journal |vauthors=Osher RH, Glaser JS |title=Myasthenic sustained gaze fatigue |journal=Am. J. Ophthalmol. |volume=89 |issue=3 |pages=443–5 |date=March 1980 |pmid=7369304 |doi= |url=}}</ref> The hallmark of myasthenia symptoms is Fluctuating [[weakness]] of specific [[Muscle|muscles]] in [[limbs]], [[bulbar]], [[ocular]] and [[respiratory system]] which get worse with activity or using the muscle.<ref name="pmid23117946">{{cite journal |vauthors=Silvestri NJ, Wolfe GI |title=Myasthenia gravis |journal=Semin Neurol |volume=32 |issue=3 |pages=215–26 |date=July 2012 |pmid=23117946 |doi=10.1055/s-0032-1329200 |url=}}</ref><ref name="pmid15052614">{{cite journal |vauthors=Keesey JC |title=Clinical evaluation and management of myasthenia gravis |journal=Muscle Nerve |volume=29 |issue=4 |pages=484–505 |date=April 2004 |pmid=15052614 |doi=10.1002/mus.20030 |url=}}</ref><ref name="pmid6951497">{{cite journal |vauthors=Keesey J, Buffkin D, Kebo D, Ho W, Herrmann C |title=Plasma exchange alone as therapy for myasthenia gravis |journal=Ann. N. Y. Acad. Sci. |volume=377 |issue= |pages=729–43 |date=1981 |pmid=6951497 |doi= |url=}}</ref> Other signs and symptoms include: Gaze paralysis <ref name="pmid570673">{{cite journal |vauthors=Spooner JW, Baloh RW |title=Eye movement fatigue in myasthenia gravis |journal=Neurology |volume=29 |issue=1 |pages=29–33 |date=January 1979 |pmid=570673 |doi= |url=}}</ref>, Photophobia <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>, Facial weakness <ref name="pmid86952">{{cite journal |vauthors=Patten BM |title=Myasthenia gravis: review of diagnosis and management |journal=Muscle Nerve |volume=1 |issue=3 |pages=190–205 |date=1978 |pmid=86952 |doi=10.1002/mus.880010304 |url=}}</ref>, Orbicularis oculi weakness <ref name="pmid11781428">{{cite journal |vauthors=Roberts ME, Steiger MJ, Hart IK |title=Presentation of myasthenia gravis mimicking blepharospasm |journal=Neurology |volume=58 |issue=1 |pages=150–1 |date=January 2002 |pmid=11781428 |doi= |url=}}</ref>, tongue weakness, chewing problems and dysphagia <ref name="pmid7779022">{{cite journal |vauthors=De Assis JL, Marchiori PE, Scaff M |title=Atrophy of the tongue with persistent articulation disorder in myasthenia gravis: report of 10 patients |journal=Auris Nasus Larynx |volume=21 |issue=4 |pages=215–8 |date=1994 |pmid=7779022 |doi= |url=}}</ref><ref name="pmid4708458">{{cite journal |vauthors=Oosterhuis H, Bethlem J |title=Neurogenic muscle involvement in myasthenia gravis. A clinical and histopathological study |journal=J. Neurol. Neurosurg. Psychiatry |volume=36 |issue=2 |pages=244–54 |date=April 1973 |pmid=4708458 |pmc=1083560 |doi= |url=}}</ref>, Respiratory problems <ref name="pmid10561522">{{cite journal |vauthors=Keesey JC |title=Does myasthenia gravis affect the brain? |journal=J. Neurol. Sci. |volume=170 |issue=2 |pages=77–89 |date=November 1999 |pmid=10561522 |doi= |url=}}</ref>, Limbs muscles weakness <ref name="pmid6290881">{{cite journal |vauthors=Jablecki C, Benton A |title=The frequency of muscle involvement in myasthenia gravis correlates with mean muscle temperature |journal=Muscle Nerve |volume=5 |issue=6 |pages=491–2 |date=1982 |pmid=6290881 |doi= |url=}}</ref><ref name="pmid6287911">{{cite journal |vauthors=Engel AG, Lambert EH, Mulder DM, Torres CF, Sahashi K, Bertorini TE, Whitaker JN |title=A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine-induced ion channel |journal=Ann. Neurol. |volume=11 |issue=6 |pages=553–69 |date=June 1982 |pmid=6287911 |doi=10.1002/ana.410110603 |url=}}</ref><ref name="pmid1603341">{{cite journal |vauthors=Oh SJ, Kuruoglu R |title=Chronic limb-girdle myasthenia gravis |journal=Neurology |volume=42 |issue=6 |pages=1153–6 |date=June 1992 |pmid=1603341 |doi= |url=}}</ref><ref name="pmid10025802">{{cite journal |vauthors=Nations SP, Wolfe GI, Amato AA, Jackson CE, Bryan WW, Barohn RJ |title=Distal myasthenia gravis |journal=Neurology |volume=52 |issue=3 |pages=632–4 |date=February 1999 |pmid=10025802 |doi= |url=}}</ref>, Fatigue <ref name="pmid9704288">{{cite journal |vauthors=Ochs CW, Bradley RJ, Katholi CR, Byl NN, Brown VM, Jones LL, Shohet JS |title=Symptoms of patients with myasthenia gravis receiving treatment |journal=J Med |volume=29 |issue=1-2 |pages=1–12 |date=1998 |pmid=9704288 |doi= |url=}}</ref>, Pelvic floor weakness <ref name="pmid4843338">{{cite journal |vauthors=Greene LF, Ghosh MK, Howard FM |title=Transurethral prostatic resection in patients with myasthenia gravis |journal=J. Urol. |volume=112 |issue=2 |pages=226–7 |date=August 1974 |pmid=4843338 |doi= |url=}}</ref><ref name="pmid6180793">{{cite journal |vauthors=Wise GJ, Gerstenfeld JN, Brunner N, Grob D |title=Urinary incontinence following prostatectomy in patients with myasthenia gravis |journal=Br J Urol |volume=54 |issue=4 |pages=369–71 |date=August 1982 |pmid=6180793 |doi= |url=}}</ref>. | The [[age of onset]] in Myasthenia gravis follows a bimodal distribution. The early type (before age of 50) is [[female]] predominant and the late type (after age of 60) is [[male]] predominant. Between the age of 50-60 there is no significant different between [[male]] and [[female]].<ref name="pmid23893883">{{cite journal |vauthors=Alkhawajah NM, Oger J |title=Late-onset myasthenia gravis: a review when incidence in older adults keeps increasing |journal=Muscle Nerve |volume=48 |issue=5 |pages=705–10 |date=November 2013 |pmid=23893883 |doi=10.1002/mus.23964 |url=}}</ref> About 50 percent of patiens have [[ptosis]] and [[diplopia]] as their presenting sign. [[Ptosis]] is usually asymmetrical. The direction and degree of [[diplopia]] may change in a [[Myasthenia gravis|MG]] patients because of [[Fatigue|fatigable]] [[ocular]] motor [[paresis]].<ref name="pmid7369304">{{cite journal |vauthors=Osher RH, Glaser JS |title=Myasthenic sustained gaze fatigue |journal=Am. J. Ophthalmol. |volume=89 |issue=3 |pages=443–5 |date=March 1980 |pmid=7369304 |doi= |url=}}</ref> The hallmark of myasthenia symptoms is Fluctuating [[weakness]] of specific [[Muscle|muscles]] in [[limbs]], [[bulbar]], [[ocular]] and [[respiratory system]] which get worse with activity or using the muscle.<ref name="pmid23117946">{{cite journal |vauthors=Silvestri NJ, Wolfe GI |title=Myasthenia gravis |journal=Semin Neurol |volume=32 |issue=3 |pages=215–26 |date=July 2012 |pmid=23117946 |doi=10.1055/s-0032-1329200 |url=}}</ref><ref name="pmid15052614">{{cite journal |vauthors=Keesey JC |title=Clinical evaluation and management of myasthenia gravis |journal=Muscle Nerve |volume=29 |issue=4 |pages=484–505 |date=April 2004 |pmid=15052614 |doi=10.1002/mus.20030 |url=}}</ref><ref name="pmid6951497">{{cite journal |vauthors=Keesey J, Buffkin D, Kebo D, Ho W, Herrmann C |title=Plasma exchange alone as therapy for myasthenia gravis |journal=Ann. N. Y. Acad. Sci. |volume=377 |issue= |pages=729–43 |date=1981 |pmid=6951497 |doi= |url=}}</ref> Other signs and symptoms include: Gaze paralysis <ref name="pmid570673">{{cite journal |vauthors=Spooner JW, Baloh RW |title=Eye movement fatigue in myasthenia gravis |journal=Neurology |volume=29 |issue=1 |pages=29–33 |date=January 1979 |pmid=570673 |doi= |url=}}</ref>, Photophobia <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>, Facial weakness <ref name="pmid86952">{{cite journal |vauthors=Patten BM |title=Myasthenia gravis: review of diagnosis and management |journal=Muscle Nerve |volume=1 |issue=3 |pages=190–205 |date=1978 |pmid=86952 |doi=10.1002/mus.880010304 |url=}}</ref>, Orbicularis oculi weakness <ref name="pmid11781428">{{cite journal |vauthors=Roberts ME, Steiger MJ, Hart IK |title=Presentation of myasthenia gravis mimicking blepharospasm |journal=Neurology |volume=58 |issue=1 |pages=150–1 |date=January 2002 |pmid=11781428 |doi= |url=}}</ref>, tongue weakness, chewing problems and dysphagia <ref name="pmid7779022">{{cite journal |vauthors=De Assis JL, Marchiori PE, Scaff M |title=Atrophy of the tongue with persistent articulation disorder in myasthenia gravis: report of 10 patients |journal=Auris Nasus Larynx |volume=21 |issue=4 |pages=215–8 |date=1994 |pmid=7779022 |doi= |url=}}</ref><ref name="pmid4708458">{{cite journal |vauthors=Oosterhuis H, Bethlem J |title=Neurogenic muscle involvement in myasthenia gravis. A clinical and histopathological study |journal=J. Neurol. Neurosurg. Psychiatry |volume=36 |issue=2 |pages=244–54 |date=April 1973 |pmid=4708458 |pmc=1083560 |doi= |url=}}</ref>, Respiratory problems <ref name="pmid10561522">{{cite journal |vauthors=Keesey JC |title=Does myasthenia gravis affect the brain? |journal=J. Neurol. Sci. |volume=170 |issue=2 |pages=77–89 |date=November 1999 |pmid=10561522 |doi= |url=}}</ref>, Limbs muscles weakness <ref name="pmid6290881">{{cite journal |vauthors=Jablecki C, Benton A |title=The frequency of muscle involvement in myasthenia gravis correlates with mean muscle temperature |journal=Muscle Nerve |volume=5 |issue=6 |pages=491–2 |date=1982 |pmid=6290881 |doi= |url=}}</ref><ref name="pmid6287911">{{cite journal |vauthors=Engel AG, Lambert EH, Mulder DM, Torres CF, Sahashi K, Bertorini TE, Whitaker JN |title=A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine-induced ion channel |journal=Ann. Neurol. |volume=11 |issue=6 |pages=553–69 |date=June 1982 |pmid=6287911 |doi=10.1002/ana.410110603 |url=}}</ref><ref name="pmid1603341">{{cite journal |vauthors=Oh SJ, Kuruoglu R |title=Chronic limb-girdle myasthenia gravis |journal=Neurology |volume=42 |issue=6 |pages=1153–6 |date=June 1992 |pmid=1603341 |doi= |url=}}</ref><ref name="pmid10025802">{{cite journal |vauthors=Nations SP, Wolfe GI, Amato AA, Jackson CE, Bryan WW, Barohn RJ |title=Distal myasthenia gravis |journal=Neurology |volume=52 |issue=3 |pages=632–4 |date=February 1999 |pmid=10025802 |doi= |url=}}</ref>, Fatigue <ref name="pmid9704288">{{cite journal |vauthors=Ochs CW, Bradley RJ, Katholi CR, Byl NN, Brown VM, Jones LL, Shohet JS |title=Symptoms of patients with myasthenia gravis receiving treatment |journal=J Med |volume=29 |issue=1-2 |pages=1–12 |date=1998 |pmid=9704288 |doi= |url=}}</ref>, Pelvic floor weakness <ref name="pmid4843338">{{cite journal |vauthors=Greene LF, Ghosh MK, Howard FM |title=Transurethral prostatic resection in patients with myasthenia gravis |journal=J. Urol. |volume=112 |issue=2 |pages=226–7 |date=August 1974 |pmid=4843338 |doi= |url=}}</ref><ref name="pmid6180793">{{cite journal |vauthors=Wise GJ, Gerstenfeld JN, Brunner N, Grob D |title=Urinary incontinence following prostatectomy in patients with myasthenia gravis |journal=Br J Urol |volume=54 |issue=4 |pages=369–71 |date=August 1982 |pmid=6180793 |doi= |url=}}</ref>. | ||
==Complications== | ==Complications== |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Natural History
The age of onset in Myasthenia gravis follows a bimodal distribution. The early type (before age of 50) is female predominant and the late type (after age of 60) is male predominant. Between the age of 50-60 there is no significant different between male and female.[1] About 50 percent of patiens have ptosis and diplopia as their presenting sign. Ptosis is usually asymmetrical. The direction and degree of diplopia may change in a MG patients because of fatigable ocular motor paresis.[2] The hallmark of myasthenia symptoms is Fluctuating weakness of specific muscles in limbs, bulbar, ocular and respiratory system which get worse with activity or using the muscle.[3][4][5] Other signs and symptoms include: Gaze paralysis [6], Photophobia [7], Facial weakness [8], Orbicularis oculi weakness [9], tongue weakness, chewing problems and dysphagia [10][11], Respiratory problems [12], Limbs muscles weakness [13][14][15][16], Fatigue [17], Pelvic floor weakness [18][19].
Complications
Prognosis
The prognosis of myasthenia gravis depends on:
- Disease duration at diagnosis: If the symptoms last less than 1 year, the patient has a 25 percent chance of going into remission by 10 years.[20]
- Disease severity[20]
- The age of onset: Younger patients seems to have better chance for remission than older ones.[20] Patients more than 50 years old are more prone to myasthenia crises like respiratory failure.[21]
References
- ↑ Alkhawajah NM, Oger J (November 2013). "Late-onset myasthenia gravis: a review when incidence in older adults keeps increasing". Muscle Nerve. 48 (5): 705–10. doi:10.1002/mus.23964. PMID 23893883.
- ↑ Osher RH, Glaser JS (March 1980). "Myasthenic sustained gaze fatigue". Am. J. Ophthalmol. 89 (3): 443–5. PMID 7369304.
- ↑ Silvestri NJ, Wolfe GI (July 2012). "Myasthenia gravis". Semin Neurol. 32 (3): 215–26. doi:10.1055/s-0032-1329200. PMID 23117946.
- ↑ Keesey JC (April 2004). "Clinical evaluation and management of myasthenia gravis". Muscle Nerve. 29 (4): 484–505. doi:10.1002/mus.20030. PMID 15052614.
- ↑ Keesey J, Buffkin D, Kebo D, Ho W, Herrmann C (1981). "Plasma exchange alone as therapy for myasthenia gravis". Ann. N. Y. Acad. Sci. 377: 729–43. PMID 6951497.
- ↑ Spooner JW, Baloh RW (January 1979). "Eye movement fatigue in myasthenia gravis". Neurology. 29 (1): 29–33. PMID 570673.
- ↑ 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.
- ↑ Patten BM (1978). "Myasthenia gravis: review of diagnosis and management". Muscle Nerve. 1 (3): 190–205. doi:10.1002/mus.880010304. PMID 86952.
- ↑ Roberts ME, Steiger MJ, Hart IK (January 2002). "Presentation of myasthenia gravis mimicking blepharospasm". Neurology. 58 (1): 150–1. PMID 11781428.
- ↑ De Assis JL, Marchiori PE, Scaff M (1994). "Atrophy of the tongue with persistent articulation disorder in myasthenia gravis: report of 10 patients". Auris Nasus Larynx. 21 (4): 215–8. PMID 7779022.
- ↑ Oosterhuis H, Bethlem J (April 1973). "Neurogenic muscle involvement in myasthenia gravis. A clinical and histopathological study". J. Neurol. Neurosurg. Psychiatry. 36 (2): 244–54. PMC 1083560. PMID 4708458.
- ↑ Keesey JC (November 1999). "Does myasthenia gravis affect the brain?". J. Neurol. Sci. 170 (2): 77–89. PMID 10561522.
- ↑ Jablecki C, Benton A (1982). "The frequency of muscle involvement in myasthenia gravis correlates with mean muscle temperature". Muscle Nerve. 5 (6): 491–2. PMID 6290881.
- ↑ Engel AG, Lambert EH, Mulder DM, Torres CF, Sahashi K, Bertorini TE, Whitaker JN (June 1982). "A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine-induced ion channel". Ann. Neurol. 11 (6): 553–69. doi:10.1002/ana.410110603. PMID 6287911.
- ↑ Oh SJ, Kuruoglu R (June 1992). "Chronic limb-girdle myasthenia gravis". Neurology. 42 (6): 1153–6. PMID 1603341.
- ↑ Nations SP, Wolfe GI, Amato AA, Jackson CE, Bryan WW, Barohn RJ (February 1999). "Distal myasthenia gravis". Neurology. 52 (3): 632–4. PMID 10025802.
- ↑ Ochs CW, Bradley RJ, Katholi CR, Byl NN, Brown VM, Jones LL, Shohet JS (1998). "Symptoms of patients with myasthenia gravis receiving treatment". J Med. 29 (1–2): 1–12. PMID 9704288.
- ↑ Greene LF, Ghosh MK, Howard FM (August 1974). "Transurethral prostatic resection in patients with myasthenia gravis". J. Urol. 112 (2): 226–7. PMID 4843338.
- ↑ Wise GJ, Gerstenfeld JN, Brunner N, Grob D (August 1982). "Urinary incontinence following prostatectomy in patients with myasthenia gravis". Br J Urol. 54 (4): 369–71. PMID 6180793.
- ↑ 20.0 20.1 20.2 Beghi E, Antozzi C, Batocchi AP, Cornelio F, Cosi V, Evoli A, Lombardi M, Mantegazza R, Monticelli ML, Piccolo G (December 1991). "Prognosis of myasthenia gravis: a multicenter follow-up study of 844 patients". J. Neurol. Sci. 106 (2): 213–20. PMID 1802969.
- ↑ Bever CT, Aquino AV, Penn AS, Lovelace RE, Rowland LP (November 1983). "Prognosis of ocular myasthenia". Ann. Neurol. 14 (5): 516–9. doi:10.1002/ana.410140504. PMID 6651238.