Supranuclear Paralysis of Upgaze

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Identifier 162-8
Title Supranuclear Paralysis of Upgaze
Ocular Movements Supranuclear Paralysis of Upgaze Convergence Retraction Nystagmus Eyelid Retraction - Collier's Sign Normal Downgaze Esotropia Sixth Nerve Palsy
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 Supranuclear Paralysis of Upgaze; Convergence Retraction Nystagmus; Eyelid Retraction - Collier's Sign; Normal Downgaze; Esotropia; Sixth Nerve Palsy; Dorsal Midbrain Syndrome; Obstructive Hydrocephalus; Aqueduct Stenosis; Medulloblastoma; Increased Intracranial Pressure; Unilateral Sixth Nerve Palsy
Presenting Symptom Headache and unsteadiness
History This young child presented with headache and unsteadiness. He was found to have obstructive hydrocephalus, aqueduct stenosis and a medulloblastoma. The constellation of clinical eye signs localized to the Dorsal Midbrain and included: • Supranuclear paralysis of upgaze (saccadic and pursuit movements) • Convergence retraction nystagmus on attempted upgaze • Lid retraction - Collier's sign • Pupillary abnormalities (light/near dissociated) In addition he had: • Esotropia of the right eye due to • A right sixth nerve palsy Neurological examination: Slight enlargement of head circumference Trunkal ataxia Diminished motor strength throughout Hyperreflexia and extensor plantar responses Brain CT showed: Obstructive hydrocephalus Aqueduct stenosis An infiltrating brainstem neoplasm consistent with a medulloblastoma Treatment: Ventriculoperitoneal Shunt Diagnosis: Medulloblastoma Obstructive hydrocephalus Aqueduct stenosis
Clinical This infant was filmed in the Intensive Care Unit shortly after placement of a ventriculoperitoneal shunt for treatment of obstructive hydrocephalus. Dorsal midbrain eye signs illustrated are: • Supranuclear paralysis of upgaze (saccadic and pursuit movements) • Convergence retraction nystagmus on attempted upgaze • Lid retraction - Collier's sign • Normal downgaze • Pupillary abnormalities (light/near dissociated) In addition he had: • Esotropia of the right eye due to • A right sixth nerve palsy Dorsal Midbrain (Pretectal) Syndrome in childhood: Other causes include: Congenital aqueduct stenosis leading to hydrocephalus Niemann-Pick disease (Type C) Gaucher's disease Tay-Sach's disease Kernicterus Pretectal pseudobobbing: Another disorder of saccades called pretectal pseudobobbing is reported in patients with acute obstructive hydrocephalus. The disorder consists of non-rhythmic, rapid movements which carry the eyes down and medially, (i.e. adducting movements) followed by a slow return toward the midline. Each movement may be preceded by a blink. The horizontal component may be a variant of convergence nystagmus. Convergence Retraction Nystagmus: Convergence retraction nystagmus has been regarded as a saccadic disorder consisting of asynchronous, opposed saccades whenever upward quick phases are stimulated. However, other studies indicate that, at least in some patients, convergence-retraction nystagmus is a primary disorder of the vergence system. Sixth Nerve Palsy in Children: Impaired abduction of one eye, sometimes transient, maybe the first sign of a tumor in the posterior fossa. Children however seldom complain of double vision and a head turn to the involved side is the most prominent finding. Co-existence of a sixth nerve palsy with cerebellar signs is usually indicative of an astrocytoma or ependymoma, or a medulloblastoma as in this child. Less commonly a supratentorial mass lesion may present with a sixth nerve palsy as a false localizing sign, in a child with raised intrarcranial pressure and papilledema. Pseudo-Abducens Palsy: The dorsal midbrain syndrome also results in a disturbance of vergence eye movements. In some patients convergence is paralyzed whilst in others it is excessive and causes convergent spasm. During horizontal saccades, the abducting eye may move more slowly than its adducting fellow. This finding has been called pseudo-abducens palsy and may reflect excess of convergence tone. Alternatively, abduction weakness with midbrain lesions that cause sustained esotropia may be due to effects on ocular motor internuclear neurons that project to the pons, since experimental inactivation of these cells causes paresis of abduction.
Neuroimaging The CT study is unavailable in this case.
Anatomy Hydrocephalus with aqueduct stenosis leads to dilation of the third ventricle and aqueduct or enlargement of the suprapineal recess with pressure on the posterior commissure. Three neural structures in the midbrain reticular formation are involved in the generation of vertical eye movements: 1. The posterior commissure (PC) 2. The interstitial nucleus of Cajal (INC) 3. The rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) Lesions of the posterior commissure are traditionally equated with a syndrome of loss of upward gaze and associated findings, and is generally known by a variety of names: Dorsal midbrain syndrome Pretectal syndrome Parinaud's syndrome Koeber-Salus-Elschig syndrome Sylvian aqueduct syndrome The clinical features of the dorsal midbrain (pretectal) syndrome are due to lesions of the nucleus of the posterior commissure (nPC), and axons projecting to the INC which may be important for the control of vertical gaze and eyelid movements. Cells in the nPC project through the posterior commissure and may contact the riMLF, INC, and M-group of neurons, which relays to the central caudal subdivision of the ocular motor nucleus and to coordinate vertical eye and lid movements. The supranuclear vertical gaze defect observed with clinical lesions affecting the posterior commissure usually affects all types of eye movements, though the vestibulo-ocular reflex and Bell's phenomenon (upward eye deviation during forceful lid closure) may be spared. Hydrocephalus may produce the pretectal syndrome by enlarging the aqueduct and third ventricle or the suprapineal recess and so stretching or compressing the posterior commissure. Acutely, in infants who have suffered intraventricular hemorrhage, the eyes may be tonically deviated downward "setting sun sign".
Pathology Medulloblastoma Review ID163-6
Etiology Medulloblastoma
Disease/Diagnosis Obstructive hydrocephalus
Treatment Ventriculoperitoneal shunt
References 1. Bernstein R, Bernardini GL. Abnormal vergence with upper brainstem infarcts: pseudoabducens palsy. Neurology 2001;56:424-425. 2. Büttner-Ennever JA, Büttner U, Cohen B, Baumgartner G. Vertical gaze paralysis and the rostral interstitial nucleus of the medial longitudinal fasciculus. Brain 1982;105:125-149. 3. Collier J. Nuclear ophthalmoplegia with especial reference to retraction of the lids and ptosis and to lesions of the posterior commissure. Brain 1927;50:488-498. 4. Clendaniel RA, Mays LE. Characteristics of antidromically identified oculomotor internuclear neurons during vergence and versional eye movements. J Neurophysiol 1994;71:1111-1127. 5. Corbett JJ. Neuro-ophthalmological complications of hydrocephalus and shunting procedures. Semin Neurol 1986;6:111-123. 6. Frohman LP, Kupersmith MJ. Reversible vertical ocular deviations associated with raised intracranial pressure. J Clin Neuro-ophthalmol 1985;5:158-163. 7. Keane JR. Pretectal pseudobobbing. Five patients with ‘V'-pattern convergence nystagmus. Arch Neurol 1985;42: 592-594. 8. Keane JR. The pretectal syndrome. Neurology 1990;40:684-690. 9. Kumar AN, Han Y, Dell'osso LF, Durand DM, Leigh RJ, Directional asymmetry during combined saccade-vergence movements. J Neurophysiol 2004;93:2797-2808. 10. Lee MS, Galetta SL, Volpe NJ, Liu GT. Sixth nerve palsies in children. Pediatr Neurol 1999;20:49-52. 11. Leigh RJ, Zee DS. Vergence Eye Movements. Ch 8;343-382 and Diagnosis and Central disorders of Ocular Motility. Ch 12;598-718. In: The Neurology of Eye Movements 4th Edition. Oxford University Press, New York 2006. 12. Ochs AL, Stark L, Hoyt WF, D'Amico D. Opposed adducting saccades in convergence-retraction nystagmus. A patient with sylvian aqueduct syndrome. Brain 1979;102:479-508. 13. Osher RH, Corbett JJ, Schatz NJ, Savino PJ, Orr LS. Neuro-ophthalmological complications of enlargement of the third ventricle. Br J Ophthalmol 1978;62:536-542. 14. Pierrot-Deseilligny CH, Chain F, Gray F, Serdaru M, Escourolle R, Lhermitte F. Parinaud's syndrome. Electro-oculographic and anatomical analyses of six vascular cases with deductions about vertical gaze organization in the premotor structures. Brain 1982;105:667-696. 15. Pullicino P. Lincoff N, Truax BT. Abnormal vergence with upper brainstem infarcts: pseudoabducens palsy. Neurology 2000, 55:352-358. 16. Rambold H, Kompf D, Helmchen C. Convergence retraction nystagmus: a disorder of vergence? Ann Neurol 2001;50:677-681. 17. Schmidtke K, Buttner-Ennever JA. Nervous control of eyelid function. A review of clinical experimental and pathological data. Brain 1992;115:227-247. 18. Tamura EE, Hoyt CS. Oculomotor consequences of intraventricular hemorrhages in premature infants. Arch Ophthalmol 1987;105:533-535. 19. Tijssen CC, De Letter MAC J. Op de Coul AAW. Convergence-retraction nystagmus. Neuro-ophthalmology 1996;16:215-218. 20. Wiest G. Abnormal vergence with upper brainstem infarcts: pseudoabducens palsy. Neurology 2001;56:424-425.
Relation is Part of 163-6
Contributor Secondary Ray Balhorn, Video compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1974
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:
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
Language eng
ARK ark:/87278/s6rz28kp
Setname ehsl_novel_shw
Date Created 2007-03-08
Date Modified 2017-11-22
ID 188586
Reference URL
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