Usefulness of Single Fiber EMG for Distinguishing Neuromuscular from Other Causes of Ocular Muscle Weakness

Consecutive patients (n = 114), who had single fiber electromyography of the frontalis muscles for symptoms suggestive of ocular myasthenia gravis, were followed up for a mean of 14 months (3-64 mos). At follow up, based on strict criteria, 23 patients were classified as having ocular myasthenia gravis, 8 patients were diagnosed as having mitochondrial myopathy or oculopharyngeal dystrophy, 18 patients were found to have other diseases and 65 patients remained without a definite diagnosis. The single fiber electromyography data of these patients were then reviewed. The patients with ocular myasthenia gravis had, on average, more than 7/20 single fiber pairs with jitter > 45 microseconds and mean jitter of 56 microseconds. The 8 patients with mitochondrial myopathy or oculopharyngeal dystrophy had an average of 5/20 single fiber pairs with jitter > 45 microseconds and a mean jitter of 52 microseconds and could not be separated from the group with ocular myasthenia gravis on the basis of the single fiber electromyography results. The 18 patients with definite other diagnosis had an average of less than 1/20 single fiber pair with jitter > 45 microseconds and a mean jitter of 25 microseconds. This group could be clearly separated from the group with ocular myasthenia gravis. We conclude that single fiber electromyelography is useful in the separation of ocular myasthenia gravis from other causes of oculomotor weakness except mitochondrial myopathy and oculopharyngeal dystrophy.