Lambert–Eaton myasthenic syndrome with ophthalmoparesis and pseudoblepharospasm

✍ Scribed by Naomi Kanzato; Masakatsu Motomura; Masahito Suehara; Kimiyosi Arimura

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 98 KB
• Volume: 22
• Category: Article
• ISSN: 0148-639X
• DOI: 10.1002/(sici)1097-4598(199912)22:12<1727::aid-mus19>3.0.co;2-k

✦ Synopsis

We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together with positive acetylcholine receptor antibodies. Repeated evaluation with high-frequency repetitive stimulation revealed an incremental response and elevated titers of antibodies against presynaptic calcium channels, confirming Lambert-Eaton myasthenic syndrome. Systemic evaluation revealed no malignant neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative therapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridine) reduced the ocular abnormalities. We conclude that the ocular manifestations in this patient were probably caused by Lambert-Eaton myasthenic syndrome.