Lambert-Eaton myasthenic syndrome with ophthalmoparesis and pseudoblepharospasm

We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together wi th positive acetylcholine receptor antibodies. Repeated evaluation with hig h-frequency repetitive stimulation revealed an incremental response and ele vated titers of antibodies against presynaptic calcium channels, confirming Lambert-Eaton myasthenic syndrome. Systemic evaluation revealed no maligna nt neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative th erapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridi ne) reduced the ocular abnormalities. We conclude that the ocular manifesta tions in this patient were probably caused by Lambert-Eaton myasthenic synd rome. (C) 1999 John Wiley & Sons, Inc.