Single fiber electromyography in chronic progressive external ophthalmoplegia

We have reviewed the electromyographic (EMG) studies of 17 patients with chronic progressive external ophthalmoplegia (CPEO). In 13 of 17 patients, conventional concentric needle EMG demonstrated a "myopathic" pattern, usually predominating in the shoulder muscles. Single-fiber EMG showed increased jitter and/or blocking in at least one muscle in 13 of 16 patients. Jitter was increased in the frontalis muscle in 10 of 13 patients and in an arm muscle in 5 of 12. When both muscles were tested, jitter was greater in the frontalis muscle in 5 patients and in the arm muscle in 2. These observations demonstrate that it may be difficult to distinguish myasthenia gravis from CPEO by EMG. The frequency with which abnormal jitter is found in CPEO suggests that, in addition to a mild generalized myopathy, a primary defect in neuromuscular transmission may be present.