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 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.
Kanzato
N
N
Department of Neurology, National Okinawa Hospital, Ganeko 3-20-14, Ginowan-shi, Okinawa 901-2214, Japan.
Motomura
M
M
Suehara
M
M
Arimura
K
K
D001323:Autoantibodies / Q000097:blood; D001764:Blepharospasm / Q000209:etiology*; D003937:Diagnosis, Differential; D004568:Electrodiagnosis; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000150:complications; D009157:Myasthenia Gravis / Q000175:diagnosis; D009886:Ophthalmoplegia / Q000209:etiology*; D010951:Plasma Exchange; D011950:Receptors, Cholinergic / Q000276:immunology / Q000378:metabolism; D013967:Thyroiditis, Autoimmune / Q000150:complications; D014459:Ulnar Nerve / Q000503:physiopathology
D001323:Autoantibodies; D011950:Receptors, Cholinergic