Dystonia with Eye Movement Abnormalities

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Identifier NOVEL_Cogan_26-1
Title Dystonia with Eye Movement Abnormalities
Clinical Signs vertical saccades are defective; tracking is good vertically and laterally
Subject Eye Movement Systems Pathology; Eye Movement Recordings
Presenting Symptom prominent sardonic grin; retraction of eyelids; eyes conjugate with steady fixation
History Male patient; facial changes were noted first, followed by retraction of the eyelids, stiffness and occasional spontaneous movement of the arms, especially on the right. Pt. of Dr. Norman Foster. 24 yr old man with prog. dystonia of 2 yrs dur.involv. side. Initial Sx was retraction of lids and diplopia. Has to support limb by sitting on hands. Has extreme facial contraction and sardonic grin. Bilateral Internuclear Opthalmoplegia
Clinical V-20/20. Lag of adduction suggesting subclin. INO but no nyst of abduct eye and no vert nyst. Lid retraction on dn gz but closes eyes when asllep (with flickering of lids when done voluntarily) Little or no vert saccades but some vert pursuit. Impression: Atyp.subclin INO(due to hypertonus of lat.recti ie failure of relaxion? Also failure of lids to relax. Review of Record: 2 yr Hx, tightness of voice, 1 yr Hx, (handwritten word) (handwritten word) rt arm (handwritten word). (More Hand Written Notes And Can Not Read) Review of tape: Conspicuous features were the lid retraction (despite spasticity of facial muscles), infrequency of blinking, and vertical gaze palsy; INO also present. Pt. then had a left thalamotomy with marked improvement in lid functions (no longer lid retraction & presence of blinking) but vert, gz and INO unaffected.
Disease/Diagnosis Dystonia
Creator David G. Cogan, MD (1908-1993), Former Director of Ophthalmology, National Eye Institute
Contributor Primary Fred C. Chu
Contributor Secondary U.S. Department of Health and Human Services. National Institutes of Health. National Eye Institute
Date 1975; 1985
Type Image/MovingImage
Format video/mp4
Source 3/4"" Umatic master videotape
Relation is Part of David G. Cogan Collection
Rights Management Copyright 2008. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Language eng
ARK ark:/87278/s64t6m2n
Setname ehsl_novel_dgc
Date Created 2006-03-06
Date Modified 2017-02-08
ID 177253
Reference URL https://collections.lib.utah.edu/ark:/87278/s64t6m2n
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