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From the Department of Neurology (M.J.B., M.B.), and Neurostatistics Section (J.W.), Department of Neurology, Emory University, Atlanta, GA.
Address correspondence and reprint requests to Dr. Michael Benatar, Emory University, Department of Neurology, 101 Woodruff Circle, Atlanta, GA 30322 michael.benatar{at}emory.edu
Background: The choice between acetyl-cholinesterase inhibitors (AChE-Is) and steroids as symptomatic therapy for ocular symptoms in myasthenia is controversial.
Methods: Thirty-five patients with myasthenia and ocular symptoms were evaluated by a single investigator. The ocular-quantitative myasthenia gravis (QMG) score was determined at each visit. The longitudinal construct validity of the ocular-QMG was assessed. Treatment epochs on AChE-I therapy alone or on steroids were defined for each patient. Changes in ocular-QMG scores between the start and end of each treatment epoch as well as the proportion of subjects achieving remission of symptoms were documented. The frequency of steroid-induced side effects was documented.
Results: The longitudinal construct validity was favorable and comparable to that for the total QMG score. Eight patients were treated with AChE-I therapy alone, 6 were initially treated with AChE-I followed by steroids, and 21 received steroids ab initio. There were 14 epochs of AChE-I treatment and 27 epochs of steroid treatment. The mean improvement in ocular-QMG score was greater during the steroid epoch (3.6 ± 2.4) than during the AChE-I epoch (1.1 ± 1.9) (p = 0.0021). Complete resolution of ocular symptoms occurred in 29% of AChE-I treatment epochs and in 70% of steroid treatment epochs. The most common steroid-induced side effects observed were impaired glucose tolerance (67%) and reduced bone mineral density (20%).
Conclusion: The ocular-quantitative myasthenia gravis score may be a useful tool for monitoring ocular symptom severity in myasthenia. Steroids appear to be more effective than acetyl-cholinesterase inhibitors. These findings warrant a more formal evaluation in a randomized controlled trial.
Abbreviations: AChE-I = acetyl-cholinesterase inhibitor; GMG = generalized myasthenia gravis; MG = myasthenia gravis; MGFA = Myasthenia Gravis Foundation of America; nAChR Ab = nicotinic acetylcholine receptor antibody; OM = ocular myasthenia; O-QMG = ocular-quantitative myasthenia gravis; QMG = quantitative myasthenia gravis; RNS = repetitive nerve stimulation; SFEMG = single-fiber EMG.
Disclosure: The authors report no disclosures.
Received April 24, 2008. Accepted in final form July 15, 2008.
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