Myasthenia gravis natural history, complications and prognosis

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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].

In 15 percent of MG patients the symptoms remain in the area of palpebrae superioris, orbicularis oculi and oculomotor muscles and other symptoms of generalized disease doesn’t occur in them. This kind of myastehnia is OMG (ocular myasthenia gravis).(1-2-3-4-5-6 uptodate ocular)

Complications

Complications that can develop as a result of myasthenia gravis are:

  • Complications from the treatment of myasthenia gravis: the side effects of drugs we use in the treatment of myasthenia gravis can become very serious and reduce the quality of life in these patients:

glucocorticoids: Skin thinning and purpura[20], Cushingoid appearance and weight gain[21], cataracts and glaucoma[22], ischemic heart disease and heart failure[23], gastritis, ulcer formation, and gastrointestinal bleeding[24], menstrual irregularities in women and low fertility in both men and women[25][26] and psychiatric and cognitive symptoms.[27]

Azathioprine: macrocytosis (increased MCV) and malignancies such as non-hodgkin lymphoma.[28][29]

Cyclosporine: nephrotoxicity[30], tremor, nausea, myalgias, gingival hyperplasia, hypertrichosis and malignancies such as squamous cell skin cancer and lymphoma.[31]

Tacrolimus: the side effects of this drug include hyperglycemia, hypomagnesemia, paresthesias and tremor.[32]

Cyclophosphamide: anorexia, nausea and vomiting, leukopenia, alopecia and hemorrhagic cystitis and it can also increase the risk of malignancies.[33][34]

plasmapheresis: infection and thrombosis.[35]

intravenous immune globulin: headache, dizziness, chills, fluid retention, aseptic meningitis, acute renal failure, anaphylaxis and thrombosis.[36][37]

  • Myasthenia crises (Respiratory failur): Because of respiratory muscle weakness, MG patients may experience crises in which the respiratory weakness is so severe that may result in intubation.(1 uptodate crises)
  • since the myasthenia is a chronic disease,most of the Complications that can develop as a result of this disease, may be equal to disease symptoms:

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.[38]
  • Disease severity[38]
  • The age of onset: Younger patients seems to have better chance for remission than older ones.[38] Patients more than 50 years old are more prone to myasthenia crises like respiratory failure.[39]

References

  1. ↑ 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.
  2. ↑ Osher RH, Glaser JS (March 1980). "Myasthenic sustained gaze fatigue". Am. J. Ophthalmol. 89 (3): 443–5. PMID 7369304.
  3. ↑ Silvestri NJ, Wolfe GI (July 2012). "Myasthenia gravis". Semin Neurol. 32 (3): 215–26. doi:10.1055/s-0032-1329200. PMID 23117946.
  4. ↑ Keesey JC (April 2004). "Clinical evaluation and management of myasthenia gravis". Muscle Nerve. 29 (4): 484–505. doi:10.1002/mus.20030. PMID 15052614.
  5. ↑ 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.
  6. ↑ Spooner JW, Baloh RW (January 1979). "Eye movement fatigue in myasthenia gravis". Neurology. 29 (1): 29–33. PMID 570673.
  7. ↑ 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.
  8. ↑ Patten BM (1978). "Myasthenia gravis: review of diagnosis and management". Muscle Nerve. 1 (3): 190–205. doi:10.1002/mus.880010304. PMID 86952.
  9. ↑ Roberts ME, Steiger MJ, Hart IK (January 2002). "Presentation of myasthenia gravis mimicking blepharospasm". Neurology. 58 (1): 150–1. PMID 11781428.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ Keesey JC (November 1999). "Does myasthenia gravis affect the brain?". J. Neurol. Sci. 170 (2): 77–89. PMID 10561522.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ Oh SJ, Kuruoglu R (June 1992). "Chronic limb-girdle myasthenia gravis". Neurology. 42 (6): 1153–6. PMID 1603341.
  16. ↑ Nations SP, Wolfe GI, Amato AA, Jackson CE, Bryan WW, Barohn RJ (February 1999). "Distal myasthenia gravis". Neurology. 52 (3): 632–4. PMID 10025802.
  17. ↑ 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.
  18. ↑ Greene LF, Ghosh MK, Howard FM (August 1974). "Transurethral prostatic resection in patients with myasthenia gravis". J. Urol. 112 (2): 226–7. PMID 4843338.
  19. ↑ 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. ↑ Fardet L, Flahault A, Kettaneh A, Tiev KP, Généreau T, Tolédano C, Lebbé C, Cabane J (July 2007). "Corticosteroid-induced clinical adverse events: frequency, risk factors and patient's opinion". Br. J. Dermatol. 157 (1): 142–8. doi:10.1111/j.1365-2133.2007.07950.x. PMID 17501951.
  21. ↑ Huscher D, Thiele K, Gromnica-Ihle E, Hein G, Demary W, Dreher R, Zink A, Buttgereit F (July 2009). "Dose-related patterns of glucocorticoid-induced side effects". Ann. Rheum. Dis. 68 (7): 1119–24. doi:10.1136/ard.2008.092163. PMID 18684744.
  22. ↑ Da Silva JA, Jacobs JW, Kirwan JR, Boers M, Saag KG, Inês LB, de Koning EJ, Buttgereit F, Cutolo M, Capell H, Rau R, Bijlsma JW (March 2006). "Safety of low dose glucocorticoid treatment in rheumatoid arthritis: published evidence and prospective trial data". Ann. Rheum. Dis. 65 (3): 285–93. doi:10.1136/ard.2005.038638. PMC 1798053. PMID 16107513.
  23. ↑ Wei L, MacDonald TM, Walker BR (November 2004). "Taking glucocorticoids by prescription is associated with subsequent cardiovascular disease". Ann. Intern. Med. 141 (10): 764–70. PMID 15545676.
  24. ↑ Messer J, Reitman D, Sacks HS, Smith H, Chalmers TC (July 1983). "Association of adrenocorticosteroid therapy and peptic-ulcer disease". N. Engl. J. Med. 309 (1): 21–4. doi:10.1056/NEJM198307073090105. PMID 6343871.
  25. ↑ MacAdams MR, White RH, Chipps BE (May 1986). "Reduction of serum testosterone levels during chronic glucocorticoid therapy". Ann. Intern. Med. 104 (5): 648–51. PMID 3083749.
  26. ↑ Crilly R, Cawood M, Marshall DH, Nordin BE (October 1978). "Hormonal status in normal, osteoporotic and corticosteroid-treated postmenopausal women". J R Soc Med. 71 (10): 733–6. PMC 1436218. PMID 712726.
  27. ↑ Wolkowitz OM, Burke H, Epel ES, Reus VI (October 2009). "Glucocorticoids. Mood, memory, and mechanisms". Ann. N. Y. Acad. Sci. 1179: 19–40. doi:10.1111/j.1749-6632.2009.04980.x. PMID 19906230.
  28. ↑ Witte AS, Cornblath DR, Schatz NJ, Lisak RP (November 1986). "Monitoring azathioprine therapy in myasthenia gravis". Neurology. 36 (11): 1533–4. PMID 3762975.
  29. ↑ Herrlinger U, Weller M, Dichgans J, Melms A (May 2000). "Association of primary central nervous system lymphoma with long-term azathioprine therapy for myasthenia gravis?". Ann. Neurol. 47 (5): 682–3. PMID 10805346.
  30. ↑ de Mattos AM, Olyaei AJ, Bennett WM (February 2000). "Nephrotoxicity of immunosuppressive drugs: long-term consequences and challenges for the future". Am. J. Kidney Dis. 35 (2): 333–46. PMID 10676738.
  31. ↑ Ciafaloni E, Nikhar NK, Massey JM, Sanders DB (August 2000). "Retrospective analysis of the use of cyclosporine in myasthenia gravis". Neurology. 55 (3): 448–50. PMID 10932288.
  32. ↑ Ponseti JM, Azem J, Fort JM, López-Cano M, Vilallonga R, Buera M, Cervera C, Armengol M (May 2005). "Long-term results of tacrolimus in cyclosporine- and prednisone-dependent myasthenia gravis". Neurology. 64 (9): 1641–3. doi:10.1212/01.WNL.0000160392.32894.6D. PMID 15883336.
  33. ↑ McCune WJ, Golbus J, Zeldes W, Bohlke P, Dunne R, Fox DA (June 1988). "Clinical and immunologic effects of monthly administration of intravenous cyclophosphamide in severe systemic lupus erythematosus". N. Engl. J. Med. 318 (22): 1423–31. doi:10.1056/NEJM198806023182203. PMID 3259286.
  34. ↑ De Feo LG, Schottlender J, Martelli NA, Molfino NA (July 2002). "Use of intravenous pulsed cyclophosphamide in severe, generalized myasthenia gravis". Muscle Nerve. 26 (1): 31–6. doi:10.1002/mus.10133. PMID 12115946.
  35. ↑ Rodnitzky RL, Goeken JA (June 1982). "Complications of plasma exchange in neurological patients". Arch. Neurol. 39 (6): 350–4. PMID 7092611.
  36. ↑ Dalakas MC (November 1999). "Intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: present status and practical therapeutic guidelines". Muscle Nerve. 22 (11): 1479–97. PMID 10514226.
  37. ↑ Dalakas MC (June 2004). "The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile". Pharmacol. Ther. 102 (3): 177–93. doi:10.1016/j.pharmthera.2004.04.002. PMID 15246245.
  38. ↑ 38.0 38.1 38.2 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.
  39. ↑ 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.


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