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Ocular myasthenia gravis: treatment successes and failures in patients with long-term follow-up

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Abstract

We previously reported that prednisone reduced the frequency of generalized myasthenia (GMG) and controlled diplopia without major adverse effects at 2 years in patients with ocular myasthenia gravis (OMG). Questions remain as to whether study subjects had long-standing disease, biasing results towards a steroid benefit, and if prednisone merely delayed GMG onset. Here, we performed a record review of a referral neuro-ophthalmology service OMG database for patients who were followed-up for ≥4 years or until GMG developed. We studied the effect of prednisone on GMG incidence and control of ocular symptoms. Generally, patients with diplopia were recommended for prednisone therapy. Most remained on daily 2.5–10 mg for diplopia control. We compared the results for prednisone-treated and “untreated” (pyridostigmine only) patients. Of 87 patients, 55 were in the prednisone-treated and 32 were in the untreated groups. GMG developed in 7 (13%) of the prednisone-treated (OR 0.41; 95% CI 0.22–0.76) and in 16 (50%) of the untreated (OR 2.78; 95% CI 1.68–4.60) patients. After OMG onset, GMG developed at a mean 5.8 and 0.22 years in prednisone and untreated groups. Diplopia was present at the last exam in 27% of the prednisone-treated (mean 7.2 years) and in 57% of the untreated (mean 4.6 years) OMG patients. For 48 prednisone-treated patients who did not develop GMG, OMG treatment failure occurred in 13. Thus, prednisone delays the onset of GMG and has sustained benefit in reducing the incidence of GMG and controlling diplopia. Without prednisone, GMG develops in 50% of OMG patients, typically within 1 year.

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Acknowledgment

This work was financially supported by Alice and Richard Netter, the David B. Kriser Foundation, and the Michael Fisher Foundation

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Authors and Affiliations

  1. Neuro-ophthalmology Service, Institute of Neurology and Neurosurgery, Roosevelt Hospital, 10th Flr, 1000, 10th Ave, New York, NY, 10019, USA

    Mark J. Kupersmith

  2. Neuro-ophthalmology Service, New York Eye and Ear Infirmary, New York, NY, USA

    Mark J. Kupersmith

  3. Neuro-ophthalmology Service, Albert Einstein School of Medicine, New York, NY, USA

    Mark J. Kupersmith

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Correspondence to Mark J. Kupersmith.

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Kupersmith, M.J. Ocular myasthenia gravis: treatment successes and failures in patients with long-term follow-up. J Neurol 256, 1314–1320 (2009). https://doi.org/10.1007/s00415-009-5120-8

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  • DOI: https://doi.org/10.1007/s00415-009-5120-8

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