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Show Journal of Neuro- Ophthalmology 17( 1): 76, 1997. © 1997 Lippincott- Raven Publishers, Philadelphia Letter to the Editor To the Editor: The patient with rippling muscle disease and intermittent esotropia described by Kosmorsky et al ( 1) showed up in spring of 1995 with myasthenia gravis ( 2). I had seen the patient about a month after the onset of his rippling muscles ( about 16 months before Dr. Kosmorsky et al.), and he had mild weakness and occasional diplopia at that time that was overshadowed by the striking rippling phenomenon. The onset of his myasthenic symptoms ( diplopia and weakness) was almost simultaneous with the rippling phenomenon. Until our report, myasthenia gravis with rippling muscles had not been previously described. The lesson to be learned from this patient is that anyone with intermittent diplopia and weakness should probably be considered to have myasthenia gravis until proved otherwise. Carl F. Ansevin, M. D. Youngstown, Ohio REFERENCES 1. Kosmorsky GS, Mehta N, Mitsumoto H, Pray son R. Intermittent esotropia associated with rippling muscle disease. J Neuro- ophthalmol 1995; 15( 3): 147- 151. 2. Ansevin CF, Agamanolis DP. Rippling muscles and myasthenia gravis with rippling muscles. Arch Neurol I996; 53: 197- 9. Author's Reply To the Editor: We have had the opportunity to evaluate the records of our patient described with rippling muscle disease and conclude, as did Dr. Ansevin, that the patient has concomitant myasthenia. The patient had a decremental phenomenon present on his electromyelogram ( EMG) recently, indicative of a defect in neuromuscular transmission as well as positive acetylcholine receptor antibodies. When first evaluated here at The Cleveland Clinic, however, he had a complete EMG, including decrimental responses, which were normal. At that time, needle examination of the muscle revealed contraction that was electrically silent, which is not a feature of myasthenia gravis. Additionally, rippling has never been described in myasthenia. We believe that if rippling were even an unusual feature of myasthenia, given the number of myasthenia patients seen and evaluated to date, surely it would have been reported previously. With regard to his intermittent esotropia, these episodes were either spontaneous or more easily brought on by near viewing. The patient noticed a crampy sensation in his eyes during the episodes of esotropia associated with diplopia. We have cared for many patients with myasthenia with ocular involvement. We have never encountered anyone who described a crampy sensation during their episodes of diplopia. Likewise, we have never seen nor heard of the distinct intermittent nature of the diplopia, which clearly could be brought on with a near effort being associated with myasthenia. Finally, we conclude that this patient had both rippling muscle disease and myasthenia gravis. The role that myasthenia may play in the patient's presentation of rippling muscle disease is not fully understood. Gregory S. Kosmorsky, D. O. Hiroshi Mitsumoto, M. D. Richard Pray son, M. D. The Cleveland Clinic Foundation Cleveland, Ohio 76 |