Pendular Vertical Oscillations

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Identifier 923-1
Title Pendular Vertical Oscillations
Ocular Movements Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Horizontal Gaze Palsy; Full Vertical Gaze
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 Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Horizontal Gaze Palsy; Palatal Tremor (Myoclonus); Pontine Hemorrhage; Degenerative Hypertrophy of the Inferior Olivary Nucleus; Lesion in the Guillain-Mollaret Triangle; Facial Palsy; Bilateral Lid Nystagmus; Bilateral Horizontal Gaze Palsy Hemorrhage
Supplementary Materials PowerPoint presentations: Palatal Tremor: Shirley H. Wray, M.D., Ph.D., FRCP Harvard Medical School Pendular Verical Oscillations: Shirley H. Wray, M.D., Ph.D., FRCP, Harvard Medical School
Presenting Symptom Right hemiparesis
History In October 1988 this 43 year old Spanish speaking man with a history of hypertension, suddenly became weak and collapsed on the floor. He was taken to an outside hospital where his blood pressure was 220/130. He was lethargic and diaphoretic and had slurred speech and a left facial palsy. A CT scan revealed a pontine hemorrhage and he was transferred to the Massachusetts General Hospital for a neurosurgical opinion. Past medical history: Hypertension Obesity Heavy alcohol abuse currently drinking 1 to 2 beers per night. No history of smoking or drug abuse. Neurological examination: Blood pressure 195/110 Oriented x 3 and followed commands well Speech markedly dysarthric, answered in one word phrases. Pupils pinpoint 2 mm OU reactive to light Bilateral horizontal gaze palsy Vertical gaze and convergence normal Continuous vertical oscillations (thought initially to be ocular bobbing) Left lower motor neuron facial palsy (Bell's palsy) Weak gag bilaterally Palate moved normally in the midline Motor system: Spastic dystonic posturing with increased tone and pronator drift in the right upper extremity and 4/5 weakness in the lower extremity, 3 beats of ankle clonus. Reflexes 3+ throughout, right extensor plantar response, left flexor Cerebellar testing: Right upper and lower extremity ataxia Neuro-ophthalmological examination: Visual acuity 20/200 OU Pupils pinpoint 2 mm OU reactive to light The eye movements showed: • Constant pendular vertical oscillations (PVOs) in primary gaze with eyes open and closed • Lid nystagmus • Bilateral horizontal gaze palsy • Normal vertical gaze • Normal convergence • Horizontal oculocephalic reflex absent • Ice water calorics absent • Optokinetic nystagmus absent horizontally and vertically The constellation of eye signs localized to the pons at the level of the abducen's nucleus. Investigations: Electrocardiogram: Normal sinus rhythm, no evidence of ST-T wave changes. CT Scan: A 1.2 x 2.5 cm. hemorrhage in the dorsal pons, left of the midline, extending to the pontomesencephalic junction and toward the floor of the fourth ventricle. No evidence of obstructive hydrocephalus. Brain MRI: A subacute hemorrhage within the dorsal pons extending rostrally into the left inferior tentorial region. Treatment: The patient was significantly hypertensive on admission and had apparently discontinued his medications shortly before his admission. He was able to be weaned rapidly off nitropaste and nifedipine and blood pressure was well controlled on atenolol 100 mg p.o., q. day. The patient was soon able to ambulate in a wheelchair with a residual right hemiparesis and persistent ocular PVOs. He was discharged to rehab. Follow-up in Jan 1989 (three months later) showed a similar constellation of eye signs. • Visual acuity 20/100 OU • Constant ocular PVOs • Bilateral horizontal gaze palsy • Normal convergence, suppressed the PVOs by fixation at near. • Full vertical gaze • Rotary eye movements on downgaze • Mild left Bell's palsy • No palatal tremor The patient was lost to follow-up. In the summer of 1992, approximately 4 years after the acute pontine hemorrhage he turned up unexpectedly. At this time • VA improved J3 OU • Ocular PVOs were intermittent • Bilateral horizontal gaze palsy unchanged • A palatal tremor (myoclonus) synchronous with the ocular PVOs was present. Brain MRI: In the left pontine tegmentum there was a 10 x 10 x 15 mm rounded abnormality demonstrating central high signal and surrounding dark rim on T2 weighted sequence consistent with an area of previous hemorrhage and associated tissue loss. In addition, an abnormal signal in the left inferior olivary nucleus. PET scan: Forty-five minutes after the injection of 329 MBq of FDG, tomographic images of the brain were obtained. There was no clear asymmetry in the glucose uptake of the brainstem with attention to the medulla and inferior olivary nuclear complex bilaterally.
Clinical The first video of this patient with an acute pontine hemorrhage was made in the Intensive Care Unit. The eye movements show: • Constant pendular vertical oscillations (PVOs) in primary gaze with eyes open and closed • Lid nystagmus • Bilateral horizontal gaze palsy • Normal vertical gaze • Normal convergence • Horizontal oculocephalic reflex absent • Ice water calorics absent • Optokinetic nystagmus absent horizontally and vertically The second video(18 months after the acute hemorrhage) shows: • Very small amplitude PVO's • Bilateral horizontal gaze palsy for saccadic and pursuit movements to the left greater than to the right • Normal convergence • Full vertical gaze • Clockwise torsional nystagmus on upgaze • Larger amplitude torsional nystagmus on downgaze • Absent horizontal oculocephalic reflexes • Mild left Bell's palsy • No palatal tremor After 4 years, this patient with PVOs post pontine hemorrhage was found to have a palatal tremor (myoclonus). PVOs are characterized by: • Smooth, pendular movements occurring at a frequency of 1 to 3 Hz (typically 2 Hz). • PVOs are accentuated under closed lids • PVOs are synchronized with movements of the palate, facial muscles, pharynx, tongue, larynx and diaphragm. Review ID927-1 and ID936-4 alongside this case. Box10-10 Clinical Features of Acquired Pendular Nystagmus Pg 506 (12).
Neuroimaging MRI in two cases of palatal tremor are illustrated: Case 1: Figure 1. Axial NECT scan shows a large pontine hemorrhage extending to the midbrain in patient (ID936-4), who survived this massive hypertensive intracranial hemorrhage and 2 years later developed palatal tremor. Case 2: Figure 2. Axial T2WI in a patient who developed palatal tremor 6 months after a midbrain bleed from a cavernous malformation shows a small mixed signal intensity lesion in the dorsal midbrain tegmentum. Figure 3. Axial T2WI (same case as Fig. 2) shows enlarged olives with striking hyperintensity characteristic for classic hypertrophic olivary degeneration. Courtesy Anne Osborn, M.D.
Anatomy According to Guillain and Mollaret the crucial location for the lesion(s) producing palatal tremor is one that involves the dentato-olivary pathway through the superior cerebellar peduncle. This pathway is an interconnecting circuit connecting three brainstem nuclei - the dentate, the red nucleus and the inferior olivary nucleus. The lesion can be located in one of four places: 1. The dentate nucleus 2. The dentate outflow through the superior cerebellar peduncle 3. At the level of the red nucleus where the pathway passes dorsally and inferior to the contralateral red nucleus or 4. In the descending central tegmental tract to the contralateral inferior olivary nucleus. More recent studies have implicated interruption of a pathway from the deep cerebellar nuclei through the superior cerebellar peduncle, which then loops caudally through the central tegmental tract to the inferior olive. When the syndrome is due to unilateral infarction of the dentate nucleus and superior cerebellar peduncle, hypertrophic changes in the inferior olivary nucleus appear on the contralateral side, as in this patient with a cavitary infarct in the left brainstem and contralateral hypertrophy of the right inferior olivary nucleus.
Pathology Histologically, the olivary nucleus is enlarged, due to hypertrophy of neurons that contain increased acetylcholinesterase reaction product. Such changes begin within a month of the stroke and maximize in about six months, and are accompanied by astrocytosis, and synaptic and axonal remodeling. At the same time, the number of olivary neurons progressively declines, so that after six years, they are less than 10% of control brains. Also, both the myelin and the axons of efferent fibers from olivary neurons are severely degenerated in patients with persistent palatal tremor who survive several years. Despite the anatomic demonstration of atrophy, functional imaging studies suggest increased metabolism of the inferior olive.
Etiology Pontine hemorrhage
Disease/Diagnosis Pontine hemorrhage; Palatal tremor (myoclonus); Contralateral hypertrophy of the inferior olivary nucleus
Treatment Only rarely does palatal tremor resolve spontaneously. Gabapentin, ceruletide, memantine, and anticholinergic agents may help some patients. Drugs that block connexin channels and there by reduce synchronized discharge of electronically coupled olivary neurons might provide a new therapeutic approach.
References 1. Averbuch-Heller L, Tusa RJ, Fubry L, Rottach KG, Ganser GL, Heide W, Büttner U, Leigh RJ. A double-blind controlled study of gabapentin and baclofen as treatment for acquired nystagmus. Ann Neurol 1997;41:818-825. 2. Barton JJ, Cox TA. Acquired pendular nystagmus in multiple sclerosis: clinical observations and the role of optic neuropathy. J Neurol Neurosurg Psychiatry. 1993 Mar;56(3):262-267. 3. Dehaene I., Van Zandycke M, Appel B. Acquired pendular nystagmus. Neuro-ophthalmol 1987;7(5);297-300. 4. Deuschl G, Toro C, Valls-Solé J, Zeffiro T, Zee DS, Hallett M. Symptomatic and essential palatal tremor. 1. Clinical, physiological and MRI analysis. Brain. 1994 Aug;117 ( Pt 4):775-788. 5. Dubinsky RM, Hallett M, Di Chiro G, Fulham M, Schwankhaus J. Increased glucose metabolism in the medulla of patients with palatal myoclonus. Neurology. 1991 Apr;41(4):557-562. 6. Gautier JC, Blackwood W. Enlargement of the inferior olivary nucleus in association with lesions of the central tegmental tract or dentate nucleus. Brain 1961;84(3):342-361. 7. Goyal M, Versnick E, Tuite P, Saint Cyr J, Kucharczyk W, Montanera W, Willinsky R, Mikulis D. Hypertrophic olivary degeneration: meta-analysis of the temporal evolution of MR findings. Am J Neuroradiol 2000; 21:1073-1077. 8. Guillain G, Mollaret P. Deux cas myoclonies synchrones et rhythmées vélo-pharyngo-laryngo-oculodiaphragmatiques: Le problèm anatomique et physiolopathologique de ce syndrome. rev. Neurol (Paris) 1931;2:545-566. 9. Katz B, Hoyt W, Townsend J. Ocular Bobbing and Unilateral Pontine Hemorrhage. Report of a Case. J Clin Neuro-ophthalmol 1982;2:193-195. 10. Keane JR. Acute vertical ocular myoclonus. Neurology 1986;36:86-89. 11. Koeppen AH. Olivary hypertrophy; histochemical demonstration of hydrolytic enzymes. Neurology 1980;30:471-480. 12. Leigh RJ, Hong S, Zee DS, Optican LM. Oculopalatal tremor: clinical and computational study of a disorder of the inferior olive. Soc Neurosci Abstr 2005; 933.8. 13. Leigh RJ, Zee DS. Diagnosis of Nystagmus and Saccadic Intrusions. Chp 10:475-558. In: The Neurology of Eye Movements, 4th Edition. Oxford University Press, New York 2006. 14. Lopez LI, Bronstein AM, Gresty MA, Du Boulay EP, Rudge P. Clinical and MRI correlates in 27 patients with acquired pendular nystagmus. Brain. 1996 Apr;119 ( Pt 2):465-472. 15. Nishie M, Yoshida Y, Hirata Y, Matsunaga M. Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy. Brain. 2002 Jun;125(Pt 6):1348-1357. 16. Ruigrok TJ, deZeeuw CI, Vogel J. Hypertrophy of inferior olivary neurons : a degenerative regenerative or plasticity phenomenon. Eur J Morphol 1990 ;28 :224-239. 17. Samuel M, Torun N, Tuite PJ, Sharpe JA, Lang AE. Progressive ataxia and palatal tremor (PAPT): clinical and MRI assessment with review of palatal tremors. Brain. 2004 Jun;127(Pt 6):1252-1268. Epub 2004 Apr 16. 18. Yokota T, Hirashima F, Furukawa T, Tsukagoshi H, Yoshikawa H. MRI findings of inferior olives in palatal myoclonus J Neurol 1989;236:115-116.
Relation is Part of 3-2, 167-6, 927-1, 936-4
Contributor Secondary Steve Smith, Videographer; Ray Balhorn, Digital Video Compressionist
Reviewer David Zee, M.D., The Johns Hopkins Hospital, Baltimore, MD
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1988
Type Image/MovingImage
Format video/mp4
Source 3/4" Umatic master videotape
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/s6gt8jrn
Setname ehsl_novel_shw
Date Created 2005-08-22
Date Modified 2017-11-22
ID 188534
Reference URL
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