Supranuclear Paralysis of Downgaze

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Identifier 944-7
Title Supranuclear Paralysis of Downgaze
Ocular Movements Supranuclear Paralysis of Downgaze; Vertical Oculocephalic Reflex Normal; Impaired Convergence; Exophoria; 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 Supranuclear Paralysis of Downgaze; Vertical Oculocephalic Reflex Normal; Impaired Convergence; Exophoria; Ocular Tilt Reaction; Artery of Percheron; Midbrain Infarct; Supranuclear Paralysis of Downgaze Infarct
Presenting Symptom Unsteadiness and double vision
History The patient is a 62 year old man who, on getting out of bed one morning, found his eyes did not open fully. The right eye hardly opened at all and the left just a little. He staggered to the bathroom and started to floss his teeth and dropped a piece of floss on the floor. He felt unsteady bending down to pick it up and fell on the floor. His wife came to help him and noted his speech was slurred. In the ambulance he had vertical double vision looking up at the overhead light. He was admitted to the Massachusetts General Hospital. In the ER he was found to be in atrial fibrillation and he had: • Global paralysis of downgaze (saccades and pursuit) • Impaired convergence • Exophoria on cover/uncover test • Deviation of the eyes up under forced eye closure (Bell phenomenon) • Optokinetic nystagmus absent with vertical rotation of the drum • Normal vertical oculocephalic reflex (Doll's head maneuver) and • Skew deviation, left hypertropia with a • Slight right head tilt - a right ocular tilt reaction (OTR) Neurological examination: Normal except for mild dysarthria which cleared within a few hours. Brain MRI: Axial FLAIR scan through the midbrain shows hyperintensity in the periaqueductal and ventral midbrain regions and through the occipital lobe patchy hyperintensity in the distribution of the right posterior cerebral artery (PCA). Axial DWI though the midbrain showed acute restriction in the ventral midbrain consistent with acute infarction and through the occipital lobe restriction in the right PCA territory. Diagnosis: Cardio-embolic midbrain infarct This case should be reviewed alongside a similar case, ID907-3. ID907-3 is of particular interest because an autopsy localized the midbrain lesion to bilateral infarction of the rostral interstitial nucleus of the MLF.
Clinical The video of this patient with an acute midbrain infarct shows: • Global paralysis of downgaze (saccades and pursuit) • Impaired convergence • Exophoria on cover/uncover test • Deviation of the eyes up under forced eye closure (Bell phenomenon) • Optokinetic nystagmus absent with vertical rotation of the drum • Normal vertical oculocephalic reflex (Doll's head maneuver) and • Skew deviation, left hypertropia with a • Slight right head tilt - a right ocular tilt reaction (OTR) The global supranuclear downgaze palsy is attributed to bilateral infarction of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) in the prerubral area of the ventral midbrain due to embolic occlusion of the artery of Percheron. Impaired convergence is sometimes associated with lesions in the mesodiencephalic region and was present in two of the four previously reported cases of downgaze paralysis. Skew deviation has been attributed to unilateral or incomplete bilateral destruction of the medial longitudinal fasciculus (MLF) and the distruption of the otolith-ocular pathway from the utricle in the inner ear to the nucleus of Cajal in the midbrain. Ocular tilt reaction: Lesions in the midbrain and interstitial nucleus of Cajal may present with a sustained contralesional OTR. Eye movement abnormalities in OTR are: 1. Skew deviation (e.g. hypotropia of the right eye in right OTR) 2. Ocular torsion with upper poles of the eyes rotated towards the lower ear (e.g. in right OTR, excyclotorsion of the right eye and incyclotorsion of the left eye) and 3. Head tilt (e.g. right head tilt in right OTR). Acutely, there may be associated torsional nystagmus. Deviation of the subjective visual vertical (e.g. tilting of the subjective visual vertical to the right in right OTR). Skew deviation was present in two previous cases of supranuclear downgaze palsy including one reported by Cogan. (For a full discussion of the OTR review ref (10)).
Neuroimaging Neuroimaging studies are not available on this patient
Anatomy 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) The rostral interstitial nucleus of the MLF (riMLF) is a cluster of neurons which lie adjacent to, but are distinct from the INC. The riMLF is situated rostral to the latter nucleus among the fibers of MLF and ventral to the nucleus of Darkshevich within a few millimeters of the midline. It also lies near the fasciculus retroflexus and the rostral pole of the red nucleus. Büttner-Ennerver and Büttner gave this region the name riMLF in the monkey, whereas Graybiel referred to it as the nucleus of the prerubral field in the cat. In the riMLF approximately equal numbers of neurons are activated by upward and downward gaze, but none are activated by horizontal movements. With respect to the supranuclear structure mediating downgaze in humans, only four autopsy cases were available when we published our findings and destruction of fiber tracts and neurons in the region bordering the dorsomedial portion of the red nucleus, i.e. the riMLF, was a feature common to all the case reported. Nevertheless, the present case differed from the previous cases in three significant ways: First, our patient lived longer than any of the others and afforded an opportunity for follow-up ocular motility examinations for 3 years. All the previous patients died from 1 week to 1 year after the onset of symptoms, and in three cases death occurred within 4 months. Second, although improvement was noted in some patients, our patient was the only one who fully recovered vertical downgaze. Full or more complete recovery might have eventuated in the other patients had they lived longer. Finally, the lesion in our patient was smaller than three of the lesions in the previously reported cases. The lesion in the fourth case is difficult to evaluate, since it was illustrated with only one section through the midbrain, although it was said to extend rostrally into the thalamus. Furthermore, the exact duration of the downward gaze paralysis is not stated, although the patient did survive 1 year after the onset of his deficit.
Pathology Trojanowski and Wray reported the fifth autopsy case of a patient with isolated supranuclear paralysis of downgaze (12). Their patient recovered and had full normal eye movements at the time of death. Nevertheless, the autopsy showed the following structures were damaged bilaterally: • riMLF • Periaqueductal gray • Ventral fibers of the PC and a portion of its nuclei • Dorsomedial red nucleus and adjacent midbrain • Ventromedial edge of the medial dorsal nucleus, fasciculus retroflexus, dorsal and medial longitudinal fasciculi and • Nucleus of Darkshevich Following the report of this case, Trojanowski and Lafontaine published another autopsy case (number six) which can be considered the first clinicopathological study of an unambiguously permanent downgaze paralysis. The patient was a 56-year-old woman who had the sudden onset of a selective paralysis of downgaze associated with a partial third nerve paralysis on the right. On numerous examinations over the ensuing 3 and ¾ years, these deficits were observed to persist. At autopsy, bilateral somewhat asymmetrical cavitated lesions were seen in the region of the thalamo-mesencephalic junction. Because this patient's selective downgaze paralysis was permanent, the authors concluded that the supranuclear neural elements mediating downgaze are situated within the confines of these lesions. A consideration of this case together with the five previous reports of selective vertical downgaze paralysis permits the relevant region to be further localized to an area extending from the oculomotor nucleus to the rostral pole of the red nucleus and immediately dorsomedial to the latter. This is likely to be the location of the riMLF nuclei, the human analogue of the monkey "nucleus of the prerubral fields', a supranuclear structure which is thought to mediate vertical gaze and especially downgaze.
Etiology Embolic infarction of the midbrain
Disease/Diagnosis Midbrain infarct
References 1. Büttner U, Büttner-Ennever JA, Henn V. Vertical eye movement related unit activity in the rostral mesencephalic reticular formation of the alert monkey. Brain Res 1977;130:239-252. http://www.ncbi.nlm.nih.gov/pubmed/406969 2. Büttner-Ennever JA, Büttner U. A cell group associated with vertical eye movements in the rostral mesencephalic reticular formation of the monkey. Brain Res 1978, 151:31-47. http://www.ncbi.nlm.nih.gov/pubmed/98208 3. Case records of the Massachusetts General Hospital, N Engl J Med 1979;301:370-377. http://www.ncbi.nlm.nih.gov/pubmed/460326 4. Cogan D. Paralysis of downgaze. Arch Ophthalmol 1974;912:192-199. http://www.ncbi.nlm.nih.gov/pubmed/4814966 5. Graybiel AM. Organization of oculomotor pathways in the cat and rhesus monkey. In Baker R, Berthoz A, Eds. Control of Gaze by Brainstem Neurons: Developments in Neuroscience. Amsterdam, Elsevier 1977:1:79-889. 6. Halmagyi GM, Evans WA, Hallinan JM. Failure of downward gaze: The site and nature of the lesion. Arch Neurol 1978;35:22-26. http://www.ncbi.nlm.nih.gov/pubmed/304343 7. Jacobs L, Anderson PJ, Bender MB. The lesions producing paralysis of downward but not upward gaze. Arch Neurol 1973; 28:319-323. http://www.ncbi.nlm.nih.gov/pubmed/4696014 8. Jenkyn LR, Margolis G, Reeves AG. Reflex vertical gaze and the medial longitudinal fasciculus. J Neurol Neurosurg Psychiatry 1978;41:1084-1091. http://www.ncbi.nlm.nih.gov/pubmed/731253 9. Koempf D, Pasik T, Pasik P. Critical structures for downward gaze in monkeys. Neurosci Abstr 1977;3:156. 10. Leigh RJ, Zee DS. Diagnosis and Management of Vestibular Disorders. Chp 11:559-598. In: The Neurology of Eye Movements, 4th Edition, Oxford University Press, New York, 2006. 11. Thomas A, Schaefer H, Bertrand F. Paralysis de l'abaissement du regard, paralysie des inferogyres, hypertonie des superogyres et des releveurs du regard. rev Neurol (Paris) 1933;40:535-542. 12. Trojanowski JQ, Wray SH. Vertical gaze ophthalmoplegia: Selective paralysis of downgaze. Neurology 1980;30:605-610. http://www.ncbi.nlm.nih.gov/pubmed/7189837 13. Trojanowski JQ, LaFontaine MH. Neuroanatomical correlates of selective downgaze paralysis. J Neuro Sci 1981;52:91-101. http://www.ncbi.nlm.nih.gov/pubmed/7299418
Relation is Part of 907-3
Contributor Secondary Steve Smith, Videographer; Ray Balhorn, Digital Video Compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1997
Type Image/MovingImage
Format video/mp4
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/s6vd9w0k
Setname ehsl_novel_shw
Date Created 2008-10-07
Date Modified 2017-02-23
ID 188655
Reference URL https://collections.lib.utah.edu/ark:/87278/s6vd9w0k
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