Paroxysmal Skew Deviation

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Identifier 169-37
Title Paroxysmal Skew Deviation
Ocular Movements Skew Deviation; Ocular Tilt Reaction
Creator Shirley H. Wray, M.D., Ph.D., FRCP, Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Contributor Primary Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Subject Paroxysmal Skew Deviation; Ocular Tilt Reaction; Otolith-Ocular Pathway; Skew Deviation
Presenting Symptom Vertical diplopia
History In 1970 I saw this unique case with Dr. Cogan. The patient is a 60 year old hypertensive woman with a ten year history of intermittent vertical double vision and oscillopsia. Neurological examination between attacks of diplopia showed: Visual acuity 20/25 OU Visual fields, pupils and fundus examination normal Ocular Motility: Full eye movements. No phoria on cover/uncover test No head tilt No nystagmus Normal convergence Normal vertical and horizontal oculocephalic reflex Normal Bell's (deviation of the eyes up under forced closed eyelids) Ocular Motility during diplopia: A right skew deviation Right eye hypotropic and counterclockwise cyclorotation, left eye hypertropic Ipsilateral head tilt to the right Right ocular tilt reaction (OTR) The skew deviation persisted for 4 to 5 minutes and then abruptly stopped. During this time there was no vertical deviation of the eyes and no head tilt. We witnessed a number of repetitive episodes of unilateral skew deviation at irregular intervals of time followed by a prolonged period of normal eye movements. Cogan made the diagnosis of paroxysmal skew deviation. We suspected that this might be epileptic. An Electroencephalogram during the spells of skew deviation showed a normal record. In paroxysmal OTR a distressing rotary oblique oscillopsia of the visual scene is reported secondary to the involuntary eye movements as in this case. Neurological examination: Normal Skull X-Ray: Normal Diagnosis: Paroxysmal skew deviation Ocular tilt reaction Etiology: Idiopathic brainstem lesion Follow-Up: The etiology of her skew deviation and ocular tilt reaction remained obscure. She was lost to follow-up.
Clinical This patient has paroxysmal skew deviation and a right ocular tilt reaction characterized by: Spontaneously elevation of the left eye (hypertropia) A right head tilt Right eye becomes hypotropic and cyclorotated counterclockwise with No associated brainstem or cerebellar signs Repeated attacks occurred at irregular periods of time The ocular tilt reaction (OTR) is a central vestibular disorder involving the vertical vestibulo-ocular reflex in the roll plane. OTR also represents a fundamental pattern of coordinated eye-head roll motion. It is based upon both utricular and vertical canal input, and is mediated by the graviceptive pathways from the labyrinths via pontomedullary vestibular nuclei to the rostral midbain tegmentum. The OTR consists of: Lateral head tilt Skew deviation of the eyes (hypotropia of the under most eye) and cyclorotation (clockwise with head tilt to the left; counterclockwise with head tilt right). In the clinical setting in patients, fundus photography will complete the triad and shows cyclodeviation of the eyes towards the head tilt (excyclotropia of the hypotropic eye; incyclotropia of the hypertropic eye). It is very surprising that most patients do not complain about perceptual tilt of the visual scene or crossed double images. Clinical cases of OTR have been reported as a paroxysmal disorder with concurrent oscillopsia secondary to brainstem lesions. The patient of Rabinovitch et al suffered from MS and the paroxysmal OTR was associated with paroxysms of vertical and horizontal pendular nystagmus. The patient of Hedges and Hoyt had a focal brainstem abscess which involved the rostral midbrain and the area of the subthalamus and zona inserta ipsilateral to the direction of head tilt. A similar case to Cogan's but with intermittent alternating skew deviation was reported by Mitchell et al in 1981. In Mitchell's case, the patient had a comitant right hypertropia over 25 prism diopters which, after a period of 4 to 5 minutes abruptly cleared and the eyes rapidly went through a transition phase during which screening by cover test elicited no vertical deviation at all. A left hypertropia of 8 prism diopters then abruptly appeared and persisted for 40 to 50 seconds before disappearing and then promptly reverting to a right hypertropia. This motility disturbance was associated with downbeat nystagmus. The lesion was localized to the rostral midbrain using thin resolution cuts of the brainstem on CT scan which showed a focal lesion at the level of the interstitial nucleus of Cajal (INC). The authors clarified terminology. Alternating skew refers to varying direction of gaze Intermittent skew refers to variation in the time period between attacks Periodic skew refers to intermittent skew when the time interval adheres to a reproducible pattern. Corbett et al reported a number of patients with intermittent alternating skew deviation similar to Mitchell's case except that Corbett's patients had an unusually slow alternating skew deviation and each patient had midbrain signs of the Sylvian aqueduct syndrome such as pathological lid retraction and supranuclear upgaze palsy. An autopsy case in one of Corbett's patients revealed a lesion in the caudal diencephalon extending down towards the INC.
Neuroimaging No imaging studies were available in this case.
Anatomy The anatomical region from which Westheimer and Blair induced the OTR by brainstem stimulation in the alert monkey was in the midbrain tegmentum, dorsolateral to the third and fourth nuclei. It extended rostrally to include the INC and the nucleus of Darkschewitsch, as well as the walls of the third ventricle near the rostral cerebral aqueduct and caudally to the rostral path of the third and fourth ventricle. The midbrain tegmentum around the INC is thought to be the critical structure in the elicitation of OTR. The INC coordinates eye and head tilt in roll by virtue of its afferent monosynaptic projections, not only to the ocular but also to spinal motor neurons. Although its function is not yet fully understood, it is thought to be involved in the vertical vestibulo-ocular reflex since downward post rotary nystagmus was abolished after unilateral lesions. The review is taken from Brandt T. Vertigo, its multisensory symptoms. Springer-Verlag, London Limited 1991.
Pathology Probable ischemia
Etiology Idiopathic
Disease/Diagnosis Paroxysmal skew deviation; Ocular tilt reaction
Treatment Paroxysmal OTR in MS was treated effectively with carbamazepine. In brainstem abscess baclofen provided some therapeutic benefit. Paroxysmal OTR due to otolithic Tullio phenomenon was successfully treated by surgical fixation of the luxated stapes foot plate.
References 1. Brandt T. Vertigo: Its multisensory symptoms. Springer-Verlag, London Limited 1991. 2. Brandt T, Dieterich M. Skew deviation with ocular torsion: a vestibular sign of topographic diagnostic value. Ann Neurol 1993;33:528-534. http://www.ncbi.nlm.nih.gov/pubmed/8498829 3. Brandt T, Dieterich M. Different types of skew deviation. J Neurol Neurosurg Psychiatr 1991;54:549-550. http://www.ncbi.nlm.nih.gov/pubmed/1880519 4. Brandt T, Dieterich M. Pathological eye-head coordination in roll: tonic ocular tilt reaction in mesencephalic and medullary lesions. Brain 1987;110-649-666. http://www.ncbi.nlm.nih.gov/pubmed/3495315 5. Corbett JJ, Schatz NJ, Shults WT, Behrens M, Berry RG. Slowly alternating skew deviation: description of a pretectal syndrome in three patients. Ann Neurol 1981;10:540-546. http://www.ncbi.nlm.nih.gov/pubmed/7325603 6. Dieterich M, Brandt T. Ocular torsion and tilt of subjective visual vertical sensitive brainstem signs. Ann Neurol 1992;33:2892-299. http://www.ncbi.nlm.nih.gov/pubmed/8498813 7. Donahue SP, Lavin PJ, Hamed LM. Tonic ocular tilt reaction simulating a superior oblique palsy: diagnostic confusion with the 3-step test. Arch Ophthalmol 1999;117:347-352. http://www.ncbi.nlm.nih.gov/pubmed/10088812 8. Donahue SP, Lavin PJ, Mohney B, Hamet L. Skew deviation and inferior oblique palsy. Am J Ophthalmol 2001;132:751-756. http://www.ncbi.nlm.nih.gov/pubmed/11704037 9. Frohman LP, Kupersmith MJ. Reversible vertical ocular deviations associated with raised intracranial pressure. J Clin Neuroophthalmol 1985;5:158-163. http://www.ncbi.nlm.nih.gov/pubmed/2934416 10. Galimberti CA, Versino M, Sartori I, Manni R, Martelli A, Tartara A. Epileptic skew deviation. Neurology 1998;50:1469-1472. http://www.ncbi.nlm.nih.gov/pubmed/9596010 11. Greenberg HS, DeWitt LD. Periodic nonalternating ocular skew deviation accompanied by head tilt and pathologic lid retraction. J Clin Neuropophthalmol 1983;3:181-184. http://www.ncbi.nlm.nih.gov/pubmed/6226716 12. Hedges TR III, Hoyt WF. Ocular tilt reaction due to an upper brainstem lesion: Paroxysmal skew deviation torsion, and oscillation of the eyes with head tilt. Ann Neurol 1982;11:537-540. http://www.ncbi.nlm.nih.gov/pubmed/7103431 13. Keane JR. Ocular skew deviation. Analysis of 100 cases. Arch Neurol 1975;32:185-190. http://www.ncbi.nlm.nih.gov/pubmed/1119961 14. Leigh JR, Zee DS. Diagnosis and Management of Vestibular Disorders. Chp 11;559-597. In: The Neurology of Eye Movements, 4th Edition. Oxford University Press, 2006. 15. Mitchell JM, Smith JL. Quencer RM. Periodic alternating skew deviation. J Clin Neuroophthalmol 1981;1:5-8. http://www.ncbi.nlm.nih.gov/pubmed/6213644 16. Moster ML, Schatz NJ, Savino PJ, Benes S, Bosley TM, Sergott C. Alternating skew on lateral gaze (bilateral abduction hypertropia). Annals of Neurology 1988;23:190-192. http://www.ncbi.nlm.nih.gov/pubmed/3377440 17. Rabinovitch HE, Sharpe JA, Sylvester TO. The ocular tilt reaction. A paroxysmal dyskinesia associated with elliptical nystagmus. Arch Ophthalmol 1997;95:1395-1398. http://www.ncbi.nlm.nih.gov/pubmed/889516 18. Radtke A, Bronstein ASM, Gresty MA, Feldon M, Taylor W, Stevens JM, Rudge P. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula. J Neurol Neurosurg Psychiatry 2001;70:790-793. http://www.ncbi.nlm.nih.gov/pubmed/11385016 19. Straube A, Brandt T. Recurrent attacks with skew deviation torsional nystagmus and contractions of the left frontalis muscle. Neurology 1994;44:17-18. http://www.ncbi.nlm.nih.gov/pubmed/8290062 20. Westheimer GM, Blair SM. Synkinesis of head and eye movements evoked by brainstem stimulation in the alert monkey. Exp Brain Res 1975;24:89-95. http://www.ncbi.nlm.nih.gov/pubmed/812717 21. Westheimer GM, Blair SM. The ocular tilt reaction- a brainstem oculomotor routine. Invest Ophthalmol 1975;14:833-839. http://www.ncbi.nlm.nih.gov/pubmed/810454
Relation is Part of 167-6, 207-1, 907-3, 944-7
Contributor Secondary Ray Balhorn, Video Compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1970
Type Image/MovingImage
Format video/mp4
Source 16 mm Film
Rights Management Copyright 2002. 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/s6fb80hr
Setname ehsl_novel_shw
Date Created 2008-09-23
Date Modified 2017-02-22
ID 188635
Reference URL https://collections.lib.utah.edu/ark:/87278/s6fb80hr
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