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NEUROLOGY 1992;42:917
© 1992 American Academy of Neurology

Midbrain myasthenia

Fatigable ptosis, 'lid twitch' sign, and ophthalmoparesis from a dorsal midbrain glioma

Nicola K. Ragge, MA, MRCP, FCOphth and William F. Hoyt, MD

Neuro-Ophthalmoiogy Unit, Department of Neurological Surgery, School of Medicine, University of California, San Francisco, CA.

A collection of symptoms and signs indistinguishable from ocular myasthenia can be caused by an intracranial mass. We illustrate this condition with the case of an adolescent girl with neurofibromatosis type 1 and a dorsal midbrain astrocytoma. At presentation, she had fatigable ptosis, upgaze paresis, and a positive "lid twitch" sign. Radiation therapy resulted in marked reduction of her signs, confirming that the muscle fatigue was central in origin. We discuss the possible mechanisms of this central fatigability.

Address correspondence and reprint requests to Dr. William F. Hoyt, Department of Neurological Surgery [Box 0926], c/o The Editorial Office, 1360 Ninth Avenue, Suite 210, San Francisco, CA 94122.

Dr. Ragge is a Keeler Scholar, supported by Keeler Ltd., Clewer Hill Road, Windsor, Berkshire, UK, and also by grants from the Wellcome Trust, London, and the Royal Society of Medicine, London, UK.

Received July 17, 1991. Accepted for publication in final form September 6, 1991.




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