Journal of Neuro-Ophthalmology, September 1983, Volume 3, Issue 3

Periodic nonalternating ocular skew deviation accompanied by head tilt and pathologic lid retraction.

A 60-year-old black male with a 13-year history of adult onset diabetes mellitus and hypertension with a previous lacunar stroke suddenly developed a periodic head and eye movement disorder characterized by nonalternating skew deviation, rotatory nystagmus, head tilt, and lid retraction. On CT scan, the patient had a lacunar infarct in the right midbrain in the region of the interstitial nucleus of Cajal, an anatomical area involved with head tilt, torsional eye movement, and skew deviation.

}. Clin. Neuro-ophthalmol. 3: 181-184,1983. Periodic Nonalternating Ocular Skew Deviation Accompanied by Head Tilt and Pathologic Lid Retraction HARRY S. GREENBERG, MD. L. DANA DeWITT MD.* Abstract A 60-year-old black male with a 13-year history of adult onset diabetes mellitus and hypertension with a previous lacunar stroke suddenly developed a periodic head and eye movement disorder characterized by nonalternaling skew deviation, rotatory nystagmus, head tilt, and lid retraction. On CT scan, the patient had a lacunar infarct in the right midbrain in the region of the interstitial nucleus of Cajal, an anatomi­cal area involved with head tiIt, torsional eye move­ment, and skew deviation. Introduction Paroxysmal skew deviation is a rare disorder reported with high brain stem pathology in patients with brain stem glioma, J abscess2 and infarct/ 4 multiple sclerosis," herpes simplex encephalitis," and diabetes mellitus." In these cases, there were neurologic signs referable to neuroanatomic path­ways outside the oculomotor system. We describe a hypertensive diabetic male with the sole symp­tom of sudden onset of diplopia and neurologic abnormalities referable only to the oculomotor system with nonalternating periodic skew devia­tion. There was comitant ocular tilt reaction, path­ologic lid retraction, and counterclockwise rotatory nystagmus. CT scan revealed a lacunar infarct in the midbrain in the region of the interstitial nucleus of Cajal. Case History A 60-year-old black male with a 13-year history of insulin-dependent diabetes mellitus and hyper- • Present address: Department of Neurology. Clinical Stroke Unit. Massachusetts General Hospital. Boston. Massachusetts. Supported in part by Department of Neurology. University of Michigan Medical School. Ann Arbor. Michigan. September 1983 tension, noted the sudden onset of diplopia present in primary position and all fields of gaze. The images were described as vertical to one another, changing to horizontal and, at times, oblique. Spells lasted 3-5 minutes and were not accompa­nied by vertigo, facial numbness, extremity numb­ness or weakness, dysarthria, dysphagia, or ataxia. At onset he had a dull, constant vertex headache and complained of slight pressure behind his right eye. Past medical history included a right hemi­spheric CVA in 1977, with a mild residual left hemiparesis. In July 1979, the patient had four to five episodes of right-sided weakness and "slurred speech," each lasting 10-15 minutes. In November 1979, anterior circulation cerebral angiography re­vealed no surgically correctable lesions. He had two myocardial infarctions, the last in March 1981. Cardiac catheterization in June 1981, showed 100% occlusion of the right coronary and 60% occlusion of the proximal circumflex and obtuse marginal. On neurologic examination, mental status was normal. Corrected visual acuity was I-I bilaterally and visual fields were full. Funduscopic exam re­vealed mild a-v nicking, sharp disc margins, and sponataneous venous pulsations. Pupils were 4 mm and briskly reactive to light and near vision. Cover/ uncover test showed no ocular misalignment. Sac­cadic and pursuit extraocular movements were full without nystagmus or diplopia. There was no pto­sis or lid droop. The patient's eye movements were observed over a period of 30-45 minutes; during that time, he experienced periodic intermittent diplopia. Each spell lasted 2-3 minutes followed by a 1-2-minute quiet period in a fixed, nonchang­ing pattern. The patient went from having full extraocular movements with no diplopia to a hor­izontally convergent ocular skew where his right eye moved inward and downward and the left eye moved upward and inward (Figs. Ia -Ie). During this skew, both eyes had intermittent torsional counterclockwise nystagmus. The counterclock­wise nystagmus was of greater amplitude in the 181 Periodic Ocular Skew Deviation ( I ((OJ Figure la-Ie. (a) Baseline quiet periud ""tween episodes-no skew deviation. (b. c) Increasing ocular skew deviation with left eye hypertropic. (d. e) After I minute, increasing head tilt to the right and lessening of skew. left eye than in the right eye. When the right eye was most depressed there was asymmetric lid re­traction greater in the right eye (Figs. Ib -Ic). Dur­ing each episode of ocular skew, the patient's pupils remained 4 mm bilaterally, and briskly re­active to light. With the skew deviation, the pa­tient's head gradually tilted to the right. Fixation did not suppress the eye movements. The remain­der of the cranial nerve and neurologic examination was normal except for a mild left pronator drift, left hyperreflexia, left Babinski sign, and Hoffman. The latter neurologic findings were constant, and unchanged since his 1977 CVA. CT scan revealed: 1) a calcified density in the right suprasellar cistern at the level of the right cerebral peduncle felt to be an ectatic basilar artery; 2) multiple low-density areas in the left cerebral hemisphere; 3) mild atrophy; and 4) a small, lucent area in the right brain stem at the level of midbrain, thought to represent a lacunar infarct (Fig. 2). The only major change between this scan and the pre­vious scan was the small abnormality in the brain stem. An angiogram confirmed that the calcified structure was a large, tortuous basilar artery. lum­bar puncture, somatosensory, and brain stem au­ditory evoked responses were normal. On the night of admission the patient was hep­arinized. Episodes of intermittent skew deviation continued despite heparinization. The following day heparin was discontinued. He continued to have intermittent ocular symptoms for 2 more days; however, on day 4, the skew became fixed with the right eye deviated inward and downward and the left eye deviated upward and inward. Counterclockwise rotatory nystagmus continued to occur on attempted upgaze. The pupils were 3.5 mm bilaterally and briskly reactive to light. The remainder of his neurologic exam was unchanged. Two and a half months later, he returned to the neurology clinic with transitory diplopia present only on position change from lying to standing. His visual acuity was J-1 bilaterally with correction. Pupils were 4 mm equal and reactive to light bilaterally. In the primary position of gaze, there was a vertical skew deviation with a left eye hy­pertropic. On right lateral gaze, there was coarse symmetrical horizontal nystagmus in the direction of gaze. On left lateral gaze, there were a few beats of unsustained fine horizontal nystagmus. On up­gaze the right eye had upbeat nystagmus with a coarse, fast counterclockwise movement. In the left eye there was fine upbeat nystagmus with fast counterclockwise movement. The nystagmus dis­appeared after six to seven beats. Convergence was normal. Pursuit and saccadic eye movements were normal. Four months later, he complained of mild double vision lying only on his right side while watching television. Pupils were 3 mm and briskly reactive to light and near vision. Pursuit and saccadic extra- Journal of Clinical Neuro-ophthalmology Greenberg, Dewitt Figure 2. CT scan with contrast shows tortuous basilar artery anterior to the brain stem and a small lacunar infarct in the right midbrain in the region of the interstitial nucleus of Cajal. ocular movements were full and normal. No diplo­pia was elicitable with the patient in any position. On Barany maneuver, the patient was asympto­matic; however, with the head tilted backward and turned to the right or left, there was brief counter­clockwise rotatory nystagmus on left and upgaze. This occurred equally in both eyes and was very brief, lasting for 5-10 seconds. It fatigued on the third Barany attempt. There was a mild palpebral fissure assymetry with the right palpebral fissure 7 mm and the left 10 mm. The rest of the neurologic exam was unchanged. Discussion The onset of skew deviation frequently coincides with the onset of acute brain stem damage, and has been described with midbrain, pontine, medullary, and cerebellar patho!ogy.6. 7 The ocular tilt reaction consists of vertical divergence and torsion of the eyes with tilting of the head.H . 9 It has been pro­duced in animals"" ~ and humans10 by electrical stimulation of the rostral tegmentum of the brain stem. It was described by Rabinovitch in a patient with multiple scierosis. ll Paroxysmal skew deviation is a rare disorder first described in a patient with brain stem glioma. l Recently, Hedges et al.~ reported paroxysmal skew deviation associated with the ocular head tilt reac­tion and counterclockwise rotatory nystagmoid eye jerks in a patient recovering from a zona incerta brain abscess. The episodes lasted 3-5 minutes, punctuated by quiet periods of 30 seconds-2 min­utes. The fixating eye did not move vertically. September 1983 These persisted for 6 months decreasing in fre­quency, possibly in response to baclofen therapy. The authors felt that the elliptical movements looked similar to superior oblique myokymia, but were bilateral. ll In our case, fixation did not sup­press the movement of either eye. Mitchell et al.:l reported periodic alternating skew and downbeat nystagmus in a patient with a small infarct at the level of the interstitial nucleus of Cajal. Corbett described three cases of slowly alternating skew deviation." A hypertensive dia­betic patient had associated pathologic lid retrac­tion and convergence retraction nystagmus. An infarct in the pretectum and periaqueductal grey was the presumed etiology. The other two patients had pupillary abnormalities and vertical ocular motility disorders," in addition to their alternating skew. Our case differs from Corbett's and Mitch­ell's patients in the constancy of the hypertropic eye (nonalternating skew) and the associated ocular tilt reaction. The periodicity was similar to the cases of Hedges~ and Mitchell.:l The present case differs from previous cases in the absence of new neurologic signs outside the oculomotor system. The coincidence of ocular head tilt, pathologic lid retraction, and periodic skew deviation with rotatory nystagmus is not unex­pected and would localize to the pretectal region. Unilateral stimulation studies in the rostral mes­encephalon of monkeys produced a vertical diver­gence- cycloversion pattern which may be coupled with a small horizontal convergence movement.H . 9 This was seen in our patient and would result presumably from unilateral firing of the intact left 183 Periodic Ocular Skew Deviation rostral mesencephalon. The dolichoectatic basilar artery seen on the CT scan and confirmed by angiogram with a lacunar infarct in the midbrain in the region of the interstitial nucleus of Cajal makes an infarct the likely causf'. This may be secondary to basilar branch occlusion from an atheroma in a tortuous basilar artery or a hyper­tensive lacunar infarct. The basilar artery lesion was felt to be surgically unapproachable. Over the next 6 months, the eyes gradually returned to normal position without skew, pupillary, or eye movement abnormalities. This recovery is consist­ent with the clinical course of a cerebrovascular accident. References 1. Alterand, CD.: Paroxysmal skew deviation in asso­ciation with brainstem glioma. Neurology 12: 520­523,1962. 2. Hedges, T.R., and Hoyt, W.F.: Ocular tilt reaction due to an upper brainstem lesion: Paroxysmal skew deviation, torsion, and oscillation of the eyes with head tilt. Ann. Neurol. 11: 537-540, 1982. 3. Mitchell, J.M., Smith, J.L., and Quencer, R.M.: Pe­riodic alternating skew deviation. ]. Clin. Neuro­ophthalmol. 1: 5-8, 1981. 4. Fisher, CM.: Some neuro-ophthalmological obser­vations. ]. Neurol. Neurosurg. Psychiatry 30: 383­392, 1967. 5. Corbett, J.J., Schatz, N.J., Shults, W.T., et al.: Slowly alternating skew deviation: Description of a pretectal syndrome in three patients. Ann. Neurol. 10: 540­546,1981. 6. Keane, J.R.: Ocular skew deviation. Analysis of 100 cases. Arch. Neurol. 32: 185-190, 1975. 7. Smith, J.L., Davis, N.J., and Klintworth, G: Skew deviation. Neurology 14: 96-105, 1964. 8. Westheimer, G, and Blair, S.M.: The ocular tilt reaction-A brainstem oculomotor routine. Invest. Ophthalmol. 14: 833-839, 1975. 9. Westheimer, G, and Blair, S.M.: Synkinesis of head and eye movements evoked by brain stimulation in the alert monkey. Exp. Brain. Res. 24: 89-95, 1975. 10. Sano, K., Sekino, H., Tsukamoto, Y, Yoshimasu, N., and Ishijima, B.: Stimulation and destruction of the region of the interstitial nucleus in cases of torticollis and see-saw nystagmus. Confin. Neurol. 34: 331­338, 1972. 11. Rabinovitch, H.E., Sharpe, J.A., and Sylvester, T.O.: The ocular tilt reaction. A paroxysmal dyskinesia associated with elliptical nystagmus. Arch. Ophthal­mol. 95: 1395-1398, 1977. 12. Hoyt, W.F., and Keane, J.R.: Superior oblique my­okymia. Arch. Ophthalmol. 84: 461-467, 1970. Acknowledgments The authors thank James W. Albers, MD., PhD., for help in preparation of the videotape; and James J. Cor­bett, M.D., for review of the case and videotape; and Thelma Jordan and Connie Stinar for typing the manu­script. Write for reprints to: Harry S. Greenberg, MD., Department of Neurology, B4913 CFOB, University of Michigan Medical School, Ann Arbor, Michigan 48109. Journal of Clinical Neuro-ophthalmology