Dystonia with Eye Movement Abnormalities

Clinical Signs: eyes are orthophoric; upgaze barely above primary; orbital position nystagmus; tracks down well, up to about 15 degrees; (fast phases ?) with rotation of head. History: male patient; some improvement in posturing. Clinical: EXAM: 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. Excell.VIDEO. No Kayser. Fleider ring. 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. Presenting Symptom: 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. [same patient as 26-1 and 26-2]. Disease/ Diagnosis: Dystonia