RSS-Feed abonnieren
![](/products/assets/desktop/img/oa-logo.png)
DOI: 10.1055/s-0043-122245
Pure Ocular Myasthenia gravis
Publikationsverlauf
Publikationsdatum:
21. Februar 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00033101/201801/lookinside/thumbnails/174_10-1055-s-0043-122245-1.jpg)
Abstract
Pure ocular myasthenia was always described as a separate entity in historical publications. More than 50% of patients develop generalized symptoms in the course of the disease. Therefore pure ocular symptoms can be the beginning of generalized myasthenia, nevertheless there is a small group of patients who suffer lifelong purely ocular symptoms. The basis, therefore, seems to be special immunological, biochemical and pathophysiological mechanisms leading to specific local changes of the neuromuscular junction. The diagnostic procedure is hampered as antibodies against acethylcholine receptors and MuSK can be demonstrated in less than 50% of these patients. Therapy of pure ocular myasthenia does not differ fundamentally from other subgroups of myasthenia gravis and is implemented with cholinesterase inhibitors, steroids and common immunosuppressants. Thymomectomy is not recommended in general, but randomized controlled studies are lacking.
-
References
- 1 Oosterhuis HJH. Myasthenia gravis. Groningen: Groningen Neurological Press; 1997
- 2 Langmann A, Lindner S, Koch M. et al. Okuläre Myasthenie: eine diagnostische und therapeutische Herausforderung – eine Übersicht. Klin Monatsbl Augenheilkd 2004; 22: 77-86
- 3 Van den Berg B, Wohlgemuth M, Tijssen CC. Oculaire myasthenia gravis: Diagnose vaak vertraagd. Ned Tijdschr Geneeskd 2011; 155: A3241
- 4 Gasperi C, Melms A, Schoser B. et al. Anti-agrin autoantibodies in myasthenia gravis. Neurology 2014; 82: 1976-1983
- 5 Pevzner A, Schoser B, Peters K. et al. Anti-LRP4 autoantibodies in AChR- and MuSK-antibody-negative myasthenia gravis. J Neurol 2012; 259: 427-435
- 6 Schumm F, Wiethölter H, Fateh-Moghadam A. et al. Thymectomy in myasthenia with pure ocular symptoms. J Neurol Neurosurg Psychiatry 1985; 48: 332-337
- 7 Kearsey C, Fernando P, D'Costa D. et al. The use of the ice pack test in myasthenia gravis. JRSM Short Rep 2010; 1: 14
- 8 Calvert CP. Disorders of neuromuscular transmission. In: Miller NR, Newman NJ, Biousse V, Kerrison JB, eds. Walsh & Hoyt’s clinical Neuro-Ophthalmology. 6th edition. Phliadelphia, Lippincott Williams & Wilkins; 2005: 1041-1084
- 9 Schumm F, Dichgans J. Klinisches Bewertungssystem (Score) der oculären Symptomatik bei Myasthenia gravis. Nervenarzt 1985; 56: 186-187
- 10 Barton JJ. Quantitative ocular tests for myasthenia gravis: a comparative review with detection theory analysis. J Neurol Sci 1998; 155: 104-114
- 11 Besinger UA, Toyka KV, Hömberg M. et al. A. Myasthenia gravis: Long-term correlation of binding and bungarotoxin blocking antibodies against acetylcholine receptors with changes in disease severity. Neurology 1983; 33: 1316-1321
- 12 Gajdos P, Sharshar T, Chevret S. Standards of measurements in myasthenia gravis. Ann N Y Acad Sc 2003; 998: 445-452
- 13 Barnett C, Katzberg H, Nabav M. et al. The quantitative myasthenia gravis score: comparison with clinical, electrophysiological, and laboratory markers. J Clin Neuromuscul Dis 2012; 13: 201-205
- 14 Campbell MJ, Simpson E, Crombie AL. et al. Ocular myasthenia: evaluation of tensilon tonography and electronystagmography as diagnostic tests. J Neurol Neurosurg Psychiatry 1970; 33: 639-646
- 15 Oda K, Ito Y. Myasthenia gravis: Antibodies to acetylcholine receptor in ocular myasthenia gravis. J. Neurol. 1981; 225: 251-258
- 16 Singman EL, Matta NS, Silbert DI. Use of the Cogan lid twitch to identify myasthenia gravis. J Neuroophthalmol 2011; 31: 239-240
- 17 Cogan DG. Myasthenia gravis. A review of the disease and a description of lid twitch as a characteristic sign. Arch. Opthalmol 1965; 74: 217-221
- 18 Ragge ML, Hoyt WF. Midbrain myasthenia, fatigable ptosis, `lid twitch´sign, and ophthalmoparesis from a dorsal midbrain glioma. Neurology 1992; 42: 917-919
- 19 Gorelick PB, Rosenberg M, Pagano RJ. Enhanced ptosis in myasthenia gravis. Arch Neurol 1981; 38: 531
- 20 Gorelick PB, Rosenberg M, Pagano RJ. Enhanced ptosis in myasthenia gravis. Arch Neurol 1981; 38: 531
- 21 Dijk JL, Florence L. The tensilon test: A safe office procedure. Ophthalmology 1980; 87: 210
- 22 Fox JM. et al. Neurologische Pharmakotherapie. Stuttgart: Thieme Verlag; 2004: 217
- 23 AAEM Quality Assurance Committee. American Association of Electrodiagnostic Medicine. Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome: summary statement. Muscle Nerve 2001; 24: 1236-1238
- 24 Katirji B. Electrodiagnosis of neuromuscular junction disorders. In: Kaminski HJ. ed. Myasthenia gravis and related disorders. Totowa, NJ, US: Humana Press; 2003: 149-175
- 25 Serra A, Ruff RL, Leigh RJ. Neuromuscular transmission failure in myasthenia gravis: Decrement of safety factor and susceptibility of extraocular muscles. Ann N Y Acad Sci 2012; 1275: 129-135
- 26 Liu R, Xu H, Wang G. et al. Extraocular muscle characteristics related to myasthenia gravis susceptibility. PLoS One 2013; 8: e 55611
- 27 Wekerle H, Ketelsen UP, Zurn AD. et al. Intrathymic pathogenesis of myasthenia gravis: Transient expression of acetylcholine receptors on thymus-derived myogenic cells. Eur J Immunol 1978; 8: 579-582
- 28 Wu X, Tuzun E, Li J. et al Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunisation. Muscle Nerve 2012; 45: 209-216
- 29 Benatar M, Kaminski H. Medical and surgical treatment for ocular myasthenia. Cochrane Database Syst Rev 2012; 12: CD005081
- 30 Kupersmith MJ. Ocular myasthenia gravis: Treatment successes and failures in patients with long-term follow-up. J Neurol 2009; 256: 1314-1320
- 31 Kupersmith MJ, Ying G. Ocular motor dysfunction and ptosis in ocular myasthenia gravis: Effects of treatment. Br J Ophthalmol 2005; 89: 1330-1334
- 32 Hoffmann S, Kohler S, Ziegler A. et al. Glucocorticoids in myasthenia gravis – if, when, how, and how much?. Acta Neurol Scand 2014; 130: 211-221
- 33 Zach H, Cetin H, Hilger E. et al. The effect of early prednisolone treatment on generalization rate in ocular myasthenia gravis. Eur J Neurol 2013; 20: 708-713
- 34 Wong SH, Huda S, Vincent A. et al. Ocular myasthenia gravis: Controversies and updates. Curr Neurol Neurosci Rep 2014; 14: 421