Clinical Signs: short neck; esotropia since childhood; horizontal nystagmus; cogwheel pursuit; ill-sustained vertical nystagmus; poor vestibular suppression; poor optokinetic response to drum; difficulty swallowing. Presenting Symptom: headache and neck pain w/ sneeze or cough. 1-9-80: 63 yr old man who may have platybasia, now referred by Dr. Pt had spastic quadriplegia at one time. Has see Joel Glaser. Now shows esotropia with pundular nyst. On gz to either side; also upbeat nyst and + dn beat nyst. But eyes are quiet in prim. Pos. Is this congenital or due to br stem involve. Will send letter. 2-12-80: 62 yr. old man with esotropia all his life. Has also been clumsy since childhood: cd never wal a plank, dific. Buttoning shirt. Sneezing; sometimes diffic in swallowing when emotionally upset.No relation to posture but 8 months ago stood up after working in garden and fell over backwards; had quadriparesis for 15- 20 mins. Study at Prince Georege Hosp ( Dr. Joel Falik showed platybasia. Had myelography. Exam now esoptropia with V:20/20 OU Nystagmus on gz to either side. Upbeat nyst. On 1k. up and abortive down beat nyst. On 1k dn. No change with posture. Suggestive dysmetria on gz from left. Poor opkin response Cogwheel following and poor vestb. Suppression. Disease/ Diagnosis: Arnold Chiari Malformation.
David G. Cogan, MD (1908-1993), Former Director of Ophthalmology, National Eye Institute
U.S. Department of Health and Human Services. National Institutes of Health. National Eye Institute
Spencer S. Eccles Health Sciences Library, University of Utah