| Identifier |
Supranuclear_Paralysis_of_Vertical_Gaze |
| Title |
Supranuclear Paralysis of Vertical Gaze |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Somnolence; Global Supranuclear Paralysis of Vertical Gaze; Absent Vertical Saccades and Pursuit Movements; Intact Convergence; Convergence Retraction Nystagmus; Ocular Dysmetria; Ocular Tilt Reaction; Lateropulsion; Unilateral Midbrain and Thalamic Infarct; Supranuclear Paralysis of Up and Downgaze; Supranuclear Paralysis of Up and Downgaze Infarct; Vertical Saccadic Dysmetria; Horizontal Saccadic Dysmetria; Thalamus Infarct |
| History |
This case was presented to the Clinical Eye Movement Society at the American Neurological Association Meeting in October 2007. The patient is a healthy, 36 year old Lieutenant Commander in the Coast Guard who was last seen perfectly well at 2 a.m. on the day of admission. He awoke in the morning around 9 a.m. having over slept - a rare event for him and noticed immediately that his vision was "all askew" due to vertical diplopia. On his way to work he felt tilted to the left and a little off balance tending to veer to the left. His left face was slightly drooped and the left arm and leg were clumsy. When he arrived at the office he was unable to type. The fingers of his left hand were clumsy and failed to hit the keys accurately. He complained of fatigue and attributed this to working almost non-stop for two weeks at his computer to design a software program. Symptomatic Inquiry: Negative for headache, vertigo, speech disturbance, chest pain, shortness of breath or palpitations. Family History: Negative for vascular and neurological disease. On examination: BP 110/60, heart rate 53, normal rhythm. General Neurological Examination: Mild left facial droop No pronator drift and normal motor strength throughout Intact coordination with no dysmetria Deep tendon reflexes 1+ symmetric Plantar responses flexor Sensory system intact Ocular motility showed: A global supranuclear paralysis of vertical up and downgaze (only 15 degrees up, 5 degrees down from midposition) Vertical saccadic pursuit movements were limited over a similar range of eye movement. Convergence normal Horizontal gaze full Gaze evoked nystagmus to the right Horizontal gaze to the left, mild abduction weakness Ocular dysmetria: Right gaze to center overshoot (hypermetric) Left gaze to center undershoot (hypometric) Vertical oculocephalic movements intact Bell's reflex intact (eyes deviated up under tightly closed lids). Additional eye signs: A left ocular tilt reaction (OTR) is a central vestibular disorder involving the vertical vestibulo-ocular reflex in the roll plane. The key to the diagnosis of an OTR is the combination of a 1. Lateral head tilt to the left in this case. 2. Hypertropia of the undermost eye, left eye, 3. Hypertropia of the contralateral right eye. 4. Fundus photography completes the triad and shows cyclodeviation of the eyes towards the head tilt (excyclotropia of the hypotropic eye; incyclotropia of the hypertropic eye). This patient did not complain of any perceptual tilt of the visual scene or crossed double images. Optokinetic nystagmus (OKN) with rotation of the stripes down, showed convergence retraction nystagmus as he attempted to look up to refixate the stripes. The pupils were abnormal - 5 mm OU with light/near dissociation. Investigations: Initially, there was concern for a diagnosis of endocarditis given the history of recent dental work and night sweats. Blood Studies: Complete blood count and erythrocyte sedimentation rate normal. Blood cultures x 3 negative. Hemocoagulable panel normal. Echocardiogram: A transthoracic and transesophageal echo revealed a patent foramen ovale with left to right shunting by Doppler. Chest x-ray and CT: Showed a right pulmonary embolus and lower lobe infarct. Abdominal/pelvic CT: Normal. Ultrasound and MRV of the lower extremities and pelvis revealed no deep vein thrombosis (DVT). CTA of the head showed: 1. Hyperdensity in the right anterior medial thalamus, consistent with infarction. 2. No hemodynamically significant lesion in the cervical or intracranial arterial circulation 3. Three mm. hypodense nodule in the posterior aspect of the left lobe of the thyroid gland Brain MRI perfusion imaging (7/16/07): 1. A DWI hyperintensity and corresponding ADC hypointensity in the right anterior medial thalamus extending into the right parasagittal midbrain adjacent to the red nucleus. (Figure 1) 2. A very faint FLAIR hyperintensity corresponding to the restricted diffusion image in the right thalamus. Diagnosis: Unilateral embolic infarction in the right anterior medial thalamus extending into the right mesencephalon adjacent to the red nucleus. Treatment: Warfarin sodium (Coumadin) 5 MG. p.o. q. p.m. Enoxaparin (Lovenox) 70 MG SC b.i.d. Discharge Diagnosis: 1. Discreet unilateral embolic infarct of the right paramedian thalamus and upper midbrain in the distribution of the posterior thalamo-subthalamic paramedian artery - the artery of Percheron (1). 2. A clinically silent right lower lobe pulmonary embolus. 3. Patent foramen ovale (PFO) with left to right shunting. Prognosis: The patient was advised that the prognosis for full recovery of his eye movements was excellent. On day 5, the vertical diplopia had resolved with complete resolution of the OTR and skew deviation. Two months later further recovery was noted with: 1. Full vertical gaze with slow vertical saccades most marked on up gaze 2. Looking down from a full upgaze position the eyes lateropulsed to the left or converged. 3. Beats of convergence nystagmus were provoked by a fast upward saccade and OKN with the stripes down. 4. He complained of persistent somnolence and had adopted the habit of setting 3 alarm clocks to wake him each morning. The patient returned 3 months after his acute stroke for closure of the PFO. On examination three weeks later, there was no change in his eye movements. He complained of excessive day time sleepiness. Brain MRI showed a rounded focus of T2 hyperintensity along the medial aspect of the right thalamus at the same location as a lesion with restricted diffusion on the prior brain MRI. (Figures 2A, B and C) |
| Anatomy |
Three neural structures in the midbrain reticular formation are involved in the generation of vertical eye movements: • Posterior commisure (PC) • Interstitial nucleus of Cajal (INC) and • Rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) In this case all three structures were damaged by the unilateral right paramedian thalamic midbrain infarct. Damage to the riMLF, and posterior commissure explains the global supranuclear paralysis of vertical gaze. Infarction of the right INC accounts for the left OTR. The infarct interrupted the pathways involved in vertical gaze just before they decussate producing an anatomically unilateral but functionally bilateral lesion. Involvement of the pretectal nuclei in the pupillary pathway, in the dorsum midbrain accounts for light-near dissociation of the pupils, and ischemia of the periaqueductal grey accounts for convergence retraction nystagmus. Literature review: Previous cases of bidirectional vertical gaze palsy from a unilateral right upper midbrain infarct have been reported. Alemdar M et al (2) reported a 47 year old woman who developed sudden complete loss of vertical saccades, smooth pursuit, and vestibular eye movements bilaterally. MRI revealed a unilateral midbrain infarct involving the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) and the interstitial nucleus of Cajal (INC) and spared the posterior commissure (PC). The lesion is presumed to have interrupted the pathways involved in vertical gaze just before they decussate, inducing an anatomically unilateral but functionally bilateral lesion. Previous reports of bidirectional vertical gaze palsy have shown lesions involving the PC or both riMLFs. Alemdar's case is the first to show that a unilateral lesion of the riMLF and the INC that spares the PC may cause complete bidirectional vertical gaze palsy. Ranalli PJ et al (12) quantified the vertical gaze defect in their patient by magnetic search coil oculography and documented the marked restriction of up and down saccades with preservation of only a 15 degree range of downward movement from midposition and a similar restriction of vertical pursuit movements. This patient had, in addition to a global vertical gaze palsy: 1. An inverted Bell's with the eyes deviating down on forced eye closure. 2. Seesaw nystagmus 3. Limited convergence 4. Limited oculocephalic movements 5. The pupil reflexes are not reported At autopsy a wedge-shaped area of infarction in the right midbrain tegmentum was found, situated dorsomedial to the rostral border of the red nucleus. Its caudal extent reached 1 mm below the level of the habenulopeduncular tract (tractus retroflexus of Meynert); it extended 10.5 mm rostrally, extending in two discreet bands in the medial thalamus, adjacent to the wall of the third ventricle. (Figure 1) The infarct destroyed the prerubral region containing the riMLF, the rostral one mm of the interstitial nucleus of Cajal (INC), the nucleus of Darkschewitsth and its invested fiber tracks, the ventral portion of the nucleus of the posterior commissure (NPCE) and parts of the dorsomedial and parafascicular thalamic nuclei. The posterior commissure, the left midbrain tegmentum, and ocular motor nuclei were spared. The rest of the brainstem and cerebellum were also normal. |
| Pathology |
Infarction destroying the prerubral region of the right midbrain and the dorsomedial and parafascicular region of the right thalamus. |
| Disease/Diagnosis |
Unilateral embolic infarction right medial thalamus; Patent foramen ovale - paradoxical embolus |
| Clinical |
The video clips of this case were taken with a hand held digital camera 4 days after the acute stroke. The first strip shows: Complete supranuclear paralysis of saccadic and pursuit upgaze with esodeviation of the left eye as he attempts to look up. Impaired conjugate downgaze and, on attempting to look down, the eyes converge. Convergence intact Vertical oculocephalic movements intact Bell's (upward deviation of the eyes under closed eyelids) intact A second clip taken 2 months later showed: Full up and downgaze with slow saccades on upgaze only and remarkable contraversive lateropulsion of the eyes to the left on full downgaze Vertical pursuit saccadic up and down Convergence intact Normal pupils |
| Presenting Symptom |
Double vision; Gait imbalance |
| Ocular Movements |
Global Supranuclear Paralysis of Vertical Gaze; Absent Vertical Saccades and Pursuit Movements; Intact Convergence; Convergence Retraction Nystagmus; Ocular Dysmetria; Ocular Tilt Reaction; Lateropulsion |
| Neuroimaging |
See above |
| Treatment |
Anticoagulation followed by closure of a patent foramen ovale. |
| Etiology |
Unilateral Embolic Infarction in the distribution of the right posterior thalamus-subthalamic paramedian artery (Type 1) associated with a PFO. |
| References |
1. Auerbach SH, DePiero TJ, Romanul F. Sylvian aqueduct syndrome caused by unilateral midbrain lesion. Ann Neurol 1982;11:91-94. http://www.ncbi.nlm.nih.gov/pubmed/7059132 2. Alemdar M, Kamaci MD, Budak F. Unilateral midbrain infarction causing upward and downward gaze palsy. J Neuro-Ophthalmol 2006;26:173-176. http://www.ncbi.nlm.nih.gov/pubmed/16966933 3. Brandt T, Dieterich M. Pathological eye-head coordination in roll: tonic ocular tilt reaction in mesencephalic and medullary lesions. Brain 1987;110:694-666. http://www.ncbi.nlm.nih.gov/pubmed/3495315 4. Bhidayasiri R, Plant GT, Leigh RJ. A hypothetical scheme for the brainstem control of vertical gaze. Neurology 2000;54:1985-1993. http://www.ncbi.nlm.nih.gov/pubmed/10822441 5. Bogousslavsky J,Miklossy J, Regli F, Janzer R. Vertical gaze palsy and selective unilateral infarction of rostral interstitial nucleus of the medial longitudinal fasiculus (riMLF). J Neurol Neurosurg Psychiatry 1990;53:67-71. http://www.ncbi.nlm.nih.gov/pubmed/2303833 6. Buttner-Ennever JA, Buttner U, Cohen B. Baumgartner G. Vertical gaze paralysis and the rostral interstitial nucleus of the medial longitudinal fasciculus. Brain 1982;105:125-149. http://www.ncbi.nlm.nih.gov/pubmed/7066670 7. Castaigne P, Lhermitte F, Buge A, Escourolle R, Hauw JJ, Lyon-Caen O. Paramedian thalamic and midbrain infarcts; clinical and neuropathological study. Ann Neurol 1981;10:127-148. http://www.ncbi.nlm.nih.gov/pubmed/7283400 8. Halmagyi GM, Brandt T, Dieterich M, Curthoys IS, Stark RJ, Hoyt WF. Tonic contraversive ocular tilt reaction due to unilateral meso-diencephalic lesion. Neurology 1990;40:1503-1509. http://www.ncbi.nlm.nih.gov/pubmed/2215939 9. Hommel M., Bogousslavsky J. The spectrum of vertical gaze palsy following unilateral brainstem stroke. Neurology 1991;41:1229-1234. http://www.ncbi.nlm.nih.gov/pubmed/1866011 10. Percheron G. Les artères du thalamus humain. II Artères et territores thalamiques paramédians de l'artère basilaire communicante. Rev Neurol 1976;132:309-324. 11. Pierrot-Deselligny C. Chain F, Gray F, Serdaru M, Escourolle R, Lhermitte F. Parinaud's syndrome: electrooculographic and anatomical analyses of six vascular cases with deductions about vertical gaze organization in the premotor structures. Brain 1982;105:667-696. http://www.ncbi.nlm.nih.gov/pubmed/7139250 12. Ranalli PJ, Sharpe JA, Fletcher WA. Palsy of upward and downward saccadic, pursuit, and vestibular movements with a unilateral midbrain lesion: pathophysiologic correlations. Neurol 1988; 38(1):114-122. http://www.ncbi.nlm.nih.gov/pubmed/3336442 13. Seifert T, Enzinger C, Ropele S, Storch MK, Fazekas F. Midbrain ischemia presenting as vertical gaze palsy; value of diffusion-weighted magnetic resonance imaging. Cerebrovasc Dis 2004;18:3-7. http://www.ncbi.nlm.nih.gov/pubmed/15159614 14. Trojanowski JQ, Wray SH. Vertical gaze ophthalmoplegia: selective paralysis of downgaze. Neurology 1980;30:605-610. http://www.ncbi.nlm.nih.gov/pubmed/7189837 |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Relation is Part of |
207-1 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6mqdyfj |
| Setname |
ehsl_novel_shw |
| ID |
2174237 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6mqdyfj |
