73 yo man French-Canadian presented to our institution for another opinion for a long standing diagnosis of Oculopharyngeal Dystrophy given at an outside institution. 17 years ago, the patient developed unfluctuating double vision that progressed to include severe, unfluctuating ptosis and mild dysphagia without involvement of the extremities or trunk. EMG at that time showed myopathy with mild involvement of the limbs and more significant involvement of the muscles of the eye and face. Prior testing was negative for acetylcholine receptor and MUSK antibodies. No other family member is affected. He denied any significant variability in his symptoms and thought it might be secondary to Agent Orange exposure. Examination showed normal vision and color vision, normally reactive pupils without an APD, and normal looking optic nerves and retina. He had severe ptosis requiring taping his eyelids up and severe limitation of eye movement in all fields of gaze. With the limited eye movements and use of prism, he was noted to be orthophoric at distance and and a 6PD exotropia at near.
Relation is Part of
NANOS Annual Meeting 2021: Walsh Session II
Raghu C. Mudumbai, MD
Spencer S. Eccles Health Sciences Library, University of Utah