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Ocular Myasthenia

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Book cover Myasthenia Gravis and Related Disorders

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Notes

  1. 1.

    This sign is commonly present in MG patients with unilateral ptosis. The patient is asked to look down, thereby inhibiting the levator muscles. After about 15 seconds, the patient looks up to the examiner’s nose or an object at eye level, and if the sign is present, the previously ptotic lid overshoots and is transiently higher than the other lid. The retracted lid then slowly drops to its previous ptotic position.

References

  1. Perlo VP, Poskanzer DC, Schwab RS, Viets HR, Osserman KE, Genkins G. Myasthenia gravis: evaluation of treatment in 1,355 patients. Neurology 1966;16:431–439.

    PubMed  CAS  Google Scholar 

  2. Ferguson FR, Hutchinson EC, Liversedge LA. Myasthenia gravis; results of medical management. Lancet 1955;269:636–639.

    Article  PubMed  CAS  Google Scholar 

  3. Bever CT, Aquino AV, Penn AS, Lovelace RE, Rowland LP. Prognosis of ocular myasthenia. Ann Neurol 1983;14:516–519.

    Article  PubMed  Google Scholar 

  4. Evoli A, Tonali P, Bartoccioni E, Lo Monaco M. Ocular myasthenia: diagnostic and therapeutic problems. Acta Neurol Scand 1988;77:31–35.

    Article  PubMed  CAS  Google Scholar 

  5. Simpson JF, Westerberg MR, Magee KR. Myasthenia gravis. An analysis of 295 cases. Acta Neurol Scand 1966;42:Suppl 23 21–27.

    Google Scholar 

  6. Grob D, Arsura EL, Brunner NG, Namba T. The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci 1987;505:472–499.

    Article  PubMed  CAS  Google Scholar 

  7. Christensen PB, Jensen TS, Tsiropoulos I, et al. Incidence and prevalence of myasthenia gravis in western Denmark: 1975 to 1989. Neurology 1993;43:1779–1783.

    PubMed  CAS  Google Scholar 

  8. Casetta I, Fallica E, Govoni V, Azzini C, Tola M, Granieri E. Incidence of myasthenia gravis in the province of Ferrara: a community-based study. Neuroepidemiology 2004;23:281–284.

    Article  PubMed  CAS  Google Scholar 

  9. Lavrnic D, Jarebinski M, Rakocevic-Stojanovic V, et al. Epidemiological and clinical characteristics of myasthenia gravis in Belgrade, Yugoslavia (1983–1992). Acta Neurol Scand 1999;100:168–174.

    Article  PubMed  CAS  Google Scholar 

  10. Robertson N, Deans J, Compston D. Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry 1998;65:492–496.

    Article  PubMed  CAS  Google Scholar 

  11. Phillips LH, Torner JC, Anderson MS, Cox GM. The epidemiology of myasthenia gravis in central and western Virginia. Neurology 1992;42:1888–1893.

    PubMed  Google Scholar 

  12. Anonymous. Incidence of myasthenia gravis in the Emilia-Romagna region: a prospective multicenter study. Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology. Neurology 1998;51:255–258.

    Google Scholar 

  13. Aiello I, Pastorino M, Sotgiu S, et al. Epidemiology of myasthenia gravis in northwestern Sardinia. Neuroepidemiology 1997;16:199–206.

    Article  PubMed  CAS  Google Scholar 

  14. Fenichel G. In: Clinical Pediatric Neurology, A Signs and Symptoms Approach. Philadelphia: Elsevier Saunders; 2005;305–307.

    Google Scholar 

  15. Yu Wai Man CY, Chinnery PF, Griffiths PG. Extraocular muscles have fundamentally distinct properties that make them selectively vulnerable to certain disorders. Neuromuscul Disord 2005;15:17–23.

    Article  PubMed  CAS  Google Scholar 

  16. Spencer RF, Porter JD. Structural organization of the extraocular muscles. Rev Oculomot Res 1988;2:33–79.

    PubMed  CAS  Google Scholar 

  17. Porter JD, Khanna S, Kaminski HJ, et al. Extraocular muscle is defined by a fundamentally distinct gene expression profile. Proc Natl Acad Sci U S A 2001;98:12062–12067.

    Article  PubMed  CAS  Google Scholar 

  18. Marino M, Barbesino G, Pinchera A, et al. Increased frequency of euthyroid ophthalmopathy in patients with Graves' disease associated with myasthenia gravis. Thyroid 2000;10:799–802.

    Article  PubMed  CAS  Google Scholar 

  19. Kaminski HJ, Li Z, Richmonds C, Ruff RL, Kusner L. Susceptibility of ocular tissues to autoimmune diseases. Ann N Y Acad Sci 2003;998:362–374.

    Article  PubMed  CAS  Google Scholar 

  20. Leigh R, Zee D. The Neurology of Eye Movements. In. Fourth ed. New York: Oxford University Press; 2006;439–444.

    Google Scholar 

  21. Loewenfeld I. The Pupil: Anatomy, Physiology and Clinical Applications. Boston: Butterworth-Heinemann; 1999.

    Google Scholar 

  22. Calvert P. Disorder of Neuromuscular Transmission. In: NR M, N N, Biousse V, Kerrison J, eds. Walsh & Hoyt's Clinical Neuro-Ophthalmology. Sixth ed. Philadelphia: Lippincott Williams & Wilkins; 2005;1041–1084.

    Google Scholar 

  23. Digre K. Principles and Techniques of Examination of the Pupils, Accommodation, and Lacrimation. In: Miller N, Newman N, Biousse V, Kerrison J, eds. Walsh & Hoyt's Clinical Neuro-Opthalmology. Philadelphia: Lippincott Williams & Wilkins; 2005;715–805.

    Google Scholar 

  24. Glaser J, Siatkowski. Infranuclear disorders of eye movement. In: Glaser J, ed. Neuroophthalmology. Third ed. Philadelphia: Lippincott Williams & Wilkins; 1999;405–409.

    Google Scholar 

  25. Verhagen WI, Venderink D. Ptosis due to metastasis in the levator palpebrae muscle as first symptom of an adenocarcinoma of the lung. Neuroophthalmology 2001;26:197–199.

    Article  Google Scholar 

  26. Liesegang TJ. Amyloid infiltration of the levator palpebrae superioris muscle: case report. Ann Ophthalmol 1983;15:610–613.

    PubMed  CAS  Google Scholar 

  27. Ertas FS, Ertas NM, Gulec S, et al. Unrecognized side effect of statin treatment: unilateral blepharoptosis. Ophthal Plast Reconstr Surg 2006;22:222–224.

    Article  PubMed  Google Scholar 

  28. Daroff R. Ocular myasthenia: diagnosis and therapy. In: Glaser J, ed. Neuro-Ophthalmology. St Louis: Mosby; 1980;62–71.

    Google Scholar 

  29. Ragge NK, Hoyt WF. Midbrain myasthenia: fatigable ptosis, 'lid twitch' sign, and ophthalmoparesis from a dorsal midbrain glioma. Neurology 1992;42:917–919.

    PubMed  CAS  Google Scholar 

  30. Kao YF, Lan MY, Chou MS, Chen WH. Intracranial fatigable ptosis. J Neuroophthalmol 1999;19:257–259.

    Article  PubMed  CAS  Google Scholar 

  31. Bhatt A, Haviland J, Black E, Stavern G. Sensitivity and specificity of the Cogan's lid twitch for myasthenia gravis in a prospective series of patients with symptomatic ptosis. Neurology 2005;64:A36.

    Google Scholar 

  32. Averbuch-Heller L, Poonyathalang A, von Maydell RD, Remler BF. Hering's law for eyelids: still valid. Neurology 1995;45:1781–1783.

    PubMed  CAS  Google Scholar 

  33. Benatar M. A systematic review of diagnostic studies in myasthenia gravis. Neuromuscul Disord 2006;16:459–467.

    Article  PubMed  Google Scholar 

  34. Kubis KC, Danesh-Meyer HV, Savino PJ, Sergott RC. The ice test versus the rest test in myasthenia gravis. Ophthalmology 2000;107:1995–1998.

    Article  PubMed  CAS  Google Scholar 

  35. Odel JG, Winterkorn JMS, Behrens MM. The sleep test for myasthenia gravis. A safe alternative to tensilon. J Clin Neuro Ophthalmol 1991;11:288–292.

    CAS  Google Scholar 

  36. Saavedra J, Femminini R, Kochen S, deZarate JC. A cold test for myasthenia gravis. Neurology 1979;29:1075.

    PubMed  CAS  Google Scholar 

  37. Borenstein S, Desmedt JE. Local cooling in myasthenia. Improvement of neuromuscular failure. Arch Neurol 1975;32:152–157.

    Article  PubMed  CAS  Google Scholar 

  38. Ellis FD, Hoyt CS, Ellis FJ, Jeffery AR, Sondhi N. Extraocular muscle responses to orbital cooling (ice test) for ocular myasthenia gravis diagnosis. J AAPOS 2000;4:271–281.

    Article  PubMed  CAS  Google Scholar 

  39. Ertas M, Arac N, Kumral K, Tuncbay T. Ice test as a simple diagnostic aid for myasthenia gravis. Acta Neurol Scand 1994;89:227–229.

    Article  PubMed  CAS  Google Scholar 

  40. Lertchavanakul A, Gamnerdsiri P, Hirunwiwatkul P. Ice test for ocular myasthenia gravis. J Med Assoc Thai 2001;84 Suppl 1:S131–136.

    PubMed  Google Scholar 

  41. Daroff RB. The office Tensilon test for ocular myasthenia gravis. Arch Neurol 1986;43:843–844.

    Article  PubMed  CAS  Google Scholar 

  42. Seybold ME. The office Tensilon test for ocular myasthenia gravis. Arch Neurol 1986;43:842–843.

    Article  PubMed  CAS  Google Scholar 

  43. Kupersmith MJ, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol 2003;60:243–248.

    Article  PubMed  Google Scholar 

  44. Barton JJ, Fouladvand M. Ocular aspects of myasthenia gravis. Semin Neurol 2000;20:7–20.

    Article  PubMed  CAS  Google Scholar 

  45. Tian J, Yang Q, Wei M, Terdiman J, Sun F. Neostigmine-induced alterations in fast phase of optokinetic responses in myasthenic ocular palsies. J Neurol 2002;249:867–874.

    Article  PubMed  CAS  Google Scholar 

  46. Toth L, Toth A, Dioszeghy P, Repassy G. Electronystagmographic analysis of optokinetic nystagmus for the evaluation of ocular symptoms in myasthenia gravis. Acta Otolaryngol 1999;119:629–632.

    Article  PubMed  CAS  Google Scholar 

  47. Ing EB, Ing SY, Ing T, Ramocki JA. The complication rate of edrophonium testing for suspected myasthenia gravis. Can J Ophthalmol 2000;35:141–144; discussion 145.

    PubMed  CAS  Google Scholar 

  48. Nicholson GA, McLeod JG, Griffiths LR. Comparison of diagnostic tests in myasthenia gravis. Clin Exp Neurol 1983;19:45–49.

    PubMed  CAS  Google Scholar 

  49. Howard FM, Lennon VA, Finley J, Matsumoto J, Elveback LR. Clinical correlations of antibodies that bind, block or modulate human acetylcholine receptors in myasthenia gravis. Ann N Y Acad Sci 1987;505:526–538.

    Article  PubMed  Google Scholar 

  50. Lefvert A, Bergstrom K, Matell G, Osterman P, Pirskanen R. Determination of acetylcholine receptor antibody in myasthenia gravis: clinical usefulness and pathogenetic implications. J Neurol, Neurosurgery and Psychiatry 1978;41:394–403.

    Article  CAS  Google Scholar 

  51. Limburg P, The T, Hummel-Tappel E, Oosterhuis H. Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1: relation to clinical parameters in 250 patients. J Neurol Sci 1983;58:357–370.

    Article  PubMed  CAS  Google Scholar 

  52. Padua L, Stalberg E, LoMonaco M, Evoli A, Batocchi A, Tonali P. SFEMG in ocular myasthenia gravis diagnosis. Clin Neurophysiol 2000;111:1203–1207.

    Article  PubMed  CAS  Google Scholar 

  53. Costa J, Evangelista T, Conceicao I, de Carvalho M. Repetitive nerve stimulation in myasthenia gravis--relative sensitivity of different muscles. Clin Neurophysiol 2004;115:2776–2782.

    Article  PubMed  Google Scholar 

  54. Bau V, Hanisch F, Hain B, Zierz S. [Ocular involvement in MuSK antibody-positive myasthenia gravis]. Klin Monatsbl Augenheilkd 2006;223:81–83.

    Article  PubMed  CAS  Google Scholar 

  55. Bennett DL, Mills KR, Riordan-Eva P, Barnes PR, Rose MR. Anti-MuSK antibodies in a case of ocular myasthenia gravis. J Neurol Neurosurg Psychiatry 2006;77:564–565.

    Article  PubMed  CAS  Google Scholar 

  56. Kennett RP, Fawcett PRW. Repetitive nerve stimulation of anconeus in the assessment of neuromuscular transmission disorders. Electroencephalogr Clin Neurophysiol: Electromyogr Motor Control 1993;89:170–176.

    CAS  Google Scholar 

  57. Zinman LH, O'Connor PW, Dadson KE, Leung RC, Ngo M, Bril V. Sensitivity of repetitive facial-nerve stimulation in patients with myasthenia gravis. Muscle Nerve 2006;33:694–696.

    Article  PubMed  Google Scholar 

  58. Oey PL, Wieneke GH, Hoogenraad TU, van Huffelen AC. Ocular myasthenia gravis: the diagnostic yield of repetitive nerve stimulation and stimulated single fiber EMG of orbicularis oculi muscle and infrared reflection oculography. Muscle Nerve 1993;16:142–149.

    Article  PubMed  CAS  Google Scholar 

  59. Rouseev R, Ashby P, Basinski A, Sharpe J. Single fiber EMG in the frontalis muscle in ocular myasthenia: specificity and sensitivity. Muscle Nerve 1992;15:399–403.

    Article  PubMed  CAS  Google Scholar 

  60. Ukachoke C, Ashby P, Basinski A, Sharpe J. Usefulness of single fiber EMG for distinguishing neuromuscular weakness from other causes of oculr muscle weakness. Can J Neurol Sci 1994;21:125–128.

    PubMed  CAS  Google Scholar 

  61. Benatar M, Hammad M, Doss-Riney H. Concentric-needle single-fiber electromyography for the diagnosis of myasthenia gravis. Muscle Nerve 2006.

    Google Scholar 

  62. Weizer JS, Lee AG, Coats DK. Myasthenia gravis with ocular involvement in older patients. Can J Ophthalmol 2001;36:26–33.

    PubMed  CAS  Google Scholar 

  63. Mullaney P, Vajsar J, Smith R, Buncic JR. The natural history and ophthalmic involvement in childhood myasthenia gravis at the hospital for sick children. Ophthalmology 2000;107:504–510.

    Article  PubMed  CAS  Google Scholar 

  64. Weinberg DH, Rizzo JF, 3rd, Hayes MT, Kneeland MD, Kelly JJ, Jr. Ocular myasthenia gravis: predictive value of single-fiber electromyography. Muscle Nerve 1999;22:1222–1227.

    Google Scholar 

  65. Kim JH, Hwang JM, Hwang YS, Kim KJ, Chae J. Childhood ocular myasthenia gravis. Ophthalmology 2003;110:1458–1462.

    Article  PubMed  Google Scholar 

  66. Badrising U, Brandenburg H, van Hilten J, Brietl P, Wintzen A. Intranasal neostigmine as add-on therapy in myasthenia gravis. J Neurol 1996;243:S59.

    Google Scholar 

  67. Mount F. Corticotropin in Treatment of Ocular Myasthenia – A Controlled Clinical Trial. Arch Neurol 1964;11:114–124.

    Article  PubMed  CAS  Google Scholar 

  68. Benatar M, Kaminski H. Medical and surgical treatments for ocular myasthenia. Cochrane Database Syst Rev 2006.

    Google Scholar 

  69. Papatestas AE, Genkins G, Kornfeld P, et al. Effects of thymectomy in myasthenia gravis. Ann Surg 1987;206:79–88.

    Article  PubMed  CAS  Google Scholar 

  70. Kawaguchi N, Kuwabara S, Nemoto Y, et al. Treatment and outcome of myasthenia gravis: Retrospective multi-center analysis of 470 Japanese patients, 1999–2000. J Neurol Sci 2004;224:43–47.

    Article  PubMed  Google Scholar 

  71. Mee J, Paine M, Byrne E, King J, Reardon K, O'Day J. Immunotherapy of ocular myasthenia gravis reduces conversion to generalized myasthenia gravis. J Neuroophthalmol 2003;23:251–255.

    Article  PubMed  Google Scholar 

  72. Monsul NT, Patwa HS, Knorr AM, Lesser RL, Goldstein JM. The effect of prednisone on the progression from ocular to generalized myasthenia gravis. J Neurol Sci 2004;217:131–133.

    Article  PubMed  CAS  Google Scholar 

  73. Papapetropoulos TH, Ellul J, Tsibri E. Development of generalized myasthenia gravis in patients with ocular myasthenia gravis. Arch Neurol 2003;60:1491–1492.

    Article  PubMed  CAS  Google Scholar 

  74. Sommer N, Sigg B, Melms A, et al. Ocular myasthenia gravis: response to long-term immunosuppressant treatment. J Neurol, Neurosurg Psychiatry 1997;62:156–162.

    Article  CAS  Google Scholar 

  75. Kaminski H, Daroff R. Treatment of ocular myasthenia: steroids only when compelled. Arch Neurol 2000;57:752–753.

    Article  PubMed  CAS  Google Scholar 

  76. Lloyd ME, Spector TD, Howard R. Osteoporosis in neurological disorders. J Neurol Neurosurg Psychiatry 2000;68:543–547.

    Article  PubMed  CAS  Google Scholar 

  77. Smith GD, Stevens DL, Fuller GN. Myasthenia gravis, corticosteroids and osteoporosis prophylaxis. J Neurol 2001;248:151.

    Article  PubMed  CAS  Google Scholar 

  78. Beekman R, Kuks JB, Oosterhuis HJ. Myasthenia gravis: diagnosis and follow-up of 100 consecutive patients. J Neurol 1997;244:112–118.

    Article  PubMed  CAS  Google Scholar 

  79. Bentley CR, Dawson E, Lee JP. Active management in patients with ocular manifestations of myasthenia gravis. Eye 2001;15:18–22.

    Article  PubMed  CAS  Google Scholar 

  80. Bradley EA, Bartley GB, Chapman KL, Waller RR. Surgical correction of blepharoptosis in patients with myasthenia gravis. Ophthal Plast Reconstr Surg 2001;17:103–110.

    Article  PubMed  CAS  Google Scholar 

  81. Sergott RC. Ocular myasthenia. In: Lisak R, ed. Handbook of Myasthenia Gravis and Myasthenic Syndromes. New York: Marcel Dekker; 1994;21–31.

    Google Scholar 

  82. Lange DJ, Rubin M, Greene PE, et al. Distant effects of locally injected botulinum toxin: a double-blind study of single fiber EMG changes. Muscle Nerve 1991;14:672–675.

    Article  PubMed  CAS  Google Scholar 

  83. Sanders DB, Massey EW, Buckley EG. Botulinum toxin for blepharospasm: single-fiber EMG studies. Neurology 1986;36:545–547.

    PubMed  CAS  Google Scholar 

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Daroff, R.B., Benatar, M. (2009). Ocular Myasthenia. In: Myasthenia Gravis and Related Disorders. Current Clinical Neurology. Humana Press. https://doi.org/10.1007/978-1-59745-156-7_6

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