Pendular Vertical Oscillations

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Identifier 923-2
Title Pendular Vertical Oscillations
Ocular Movements Pendular Vertical Oscillations; Rotary Nystagmus; Saccadic Dysmetria
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; Rotary Nystagmus; Saccadic Dysmetria; Oscillopsia; Multiple Sclerosis; Torsional Nystagmus
Supplementary Materials PowerPoint Presentation: Lessons from the Bench and Bedside: http://library.med.utah.edu/NOVEL/Wray/PPT/Lessons_From_Bench_Bedside.ppt Stephen L. Hauser, M.D., University of California, San Francisco
Presenting Symptom Difficulty Reading; Oscillopsia
History This 36 year old woman had a nine year history of multiple sclerosis (MS) with onset in 1980. In 1989 she was seen in the neurovisual clinic for evaluation of oscillopsia, (an illusion of movement of the visual world). This symptom was accompanied by difficulty in reading because the print was "jumping". Neuro-ophthalmological examination: Visual acuity 20/25 OU, J2 Visual fields, pupils and fundus examination normal Ocular Motility Full eye movements Pendular vertical oscillations (PVO) in primary gaze, OD > OS. Clockwise rotary nystagmus OU gaze left Anti-clockwise rotary nystagmus OU gaze right No nystagmus on up or downgaze Horizontal saccadic dysmetria Right gaze to center hypermetric (overshoot) Left gaze to center hypermetric Upgaze to center no dysmetria Downgaze to center no dysmetria Alternate cover test no change in amplitude of the PVOs. PVOs visible under closed lids, OD > OS Brain MRI: T2 WI showed diffuse rounded hyperintense signals in the centrum semiovale just at and above the lateral ventricles and the corpus callosum. Larger hyperintense signals were present in the occipital horns bilaterally and to a lesser degree in the frontal horns. Smaller hyperintense signals were present in the white matter lateral to the lateral ventricles and involving the temporal and occipital horns bilaterally. Large bilateral hyperintense signals were also seen in the pons, midbrain and the cerebellar peduncles, greater on the right side. Impression: Multiple Sclerosis Oscillopsia: Oscillopsia is usually caused by excessive motion of images of stationary objects upon the retina. Excessive retinal slip not only causes oscillopsia but also impairs vision. The relationship between retinal image velocity in visual acuity is a direct one: for higher spacial frequencies, image motion in excess of about 5 degrees per second impairs vision. Leigh and Zee (6) discuss the etiology of oscillopsia under three headings: 1. Oscillopsia with head movements: abnormal vestibular ocular reflex (which includes central and peripheral vestibular hypofunction) 2. Oscillopsia due to nystagmus and 3. Central oscillopsia (with cerebral disorders such as seizures). Classification of oscillopsia due to nystagmus: • Acquired nystagmus (especially pendular nystagmus, upbeat, downbeat, seesaw, dissociated nystagmus) • Saccadic oscillations (psychogenic flutter/voluntary nystagmus, ocular flutter, micro-saccadic flutter and opsoclonus) • Superior oblique myokymia (monocular oscillopsia) • Congenital nystagmus (rarely a complaint). Oscillopsia due to nystagmus is a prominent symptom in MS patients with eye movement disorders, which include internuclear ophthalmoplegia, or pendulcar oscillations, as in this case. In such cases, oscillopsia occurs even when the head is still. The magnitude of oscillopsia is usually less than the magnitude of nystagmus. For example, in patients with downbeat nystagmus, oscillopsia is equivalent to about one-third of what would be predicted from the amplitude of the nystagmus. This finding implies that the brain compensates for the excessive retinal image motion by using an extraretinal signal, such as efference copy, to maintain visual constancy. Treatment: This patient was seen prior to the double-blind controlled study of gabapentin and baclofen as treatment for acquired nystagmus published in 1997 (1). Botulinum Toxin Therapy : Monocular botulinum toxin injections into the extraocular muscles to suppress nystagmus or oscillations had also not yet been introduced as a therapeutic option. The patient was referred to the Low Vision Aid Clinic to see if optical devices could stabilize the retinal image. a) High plus lenses combined with a minus contact lens (Rushton and Cox, 1987, JNNP Vol 50, 411-415) b) Base out prisms to induce convergence with minus lenses to correct accommodation (as used in congenital nystagmus (Dickenson Physiological Optics 1986). Prism therapy failed to stabilize her vision.
Clinical This MS patient who presented with difficulty reading has: • Asymmetric oscillopsia in association with • Constant pendular vertical oscillations (PVO) of the eyes She describes asymmetric oscillopsia worse in the right eye With the right eye she saw jumping of the whole visual scene up and down With the left eye only jumping up and down of the object she was looking at. The eye movements showed: • Asymmetric PVOs in primary gaze, OD > OS. • Clockwise rotary nystagmus OU gaze left • Anti-clockwise rotary nystagmus OU gaze right • No nystagmus on up or downgaze • Horizontal saccadic dysmetria Right gaze to center hypermetric (overshoot) Left gaze to center hypermetric Upgaze to center no dysmetria Downgaze to center no dysmetria • Alternate cover test no change in amplitude of the PVOs. • PVOs visible under closed lids, OD > OS Discussion with Dr. David Zee (DZ) I had the opportunity to review this case with Dr. Zee and, the marked asymmetry in the amplitude of the oscillations between the two eyes prompted DZ to call the movements Dissociated Nystagmus with Pendular High Fequency Oscillations. I called the movements "nystagmus" on the tape which was recorded in 1989. In MS the amplitude of oscillations is generally greater in an eye with poor vision. This patient had, however, equal visual acuity in the two eyes. PVOs are: 1. Relatively rare in MS 2. A prominent finding in patients with palatal tremor (review cases ID167-6 and 923-1). 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 DZ also noted that: • On gaze left the eyes show almost seesaw nystagmus with a rotary component • On gaze right anti-clockwise rotary (torsional) gaze evoked nystagmus Torsional nystagmus is seen in a wide range of lesions throughout the brainstem, including lateral medulla infarction, syringobulbia, pontine tegmental venous angioma and midbrain lesions involving the rostral interstitial nucleus of the MLF and interstitial nucleus of Cajal. Box 10-10 Clinical Features of Acquired Pendular Nystagmus Pg.506(6) ID941-2 an MS patient with Pendular Horizontal Oscillations and Bilateral Internuclear Ophthalmoplegia should be reviewed alongside this case.
Neuroimaging The brain MRI was not available in this case. Review (7)
Anatomy Review (6)
Pathology Review (3)
Etiology Genetics: Compelling data indicate that susceptibility to MS is inherited. The MS-prone genotype results from multiple independent or interacting genes, each exerting a small, or at most, a moderate effect on the overall risk. It is also likely that genetic heterogeneity exists, meaning that specific genes influence the susceptibility and pathogenesis in some individuals but not in others. A corollary of this observation is that the contribution of genetics to MS may vary in different affected individuals or groups. Concordance in families for early and late clinical features have been observed as well, indicating that, in addition to susceptibility, genes influence disease severity or other aspects of the clinical phenotype. Hence, some genes may be involved in the individual pathogenic events, whilst other could influence the development and progression of the disease. The strongest and most consistently replicated evidence for an MS susceptibility gene has been localized to the major histo- compatibility complex (MHC) superlocus on chromosome 6P21.3. The HLA class II DR2 haplotype has been linked to MS in multiple studies, primarily in populations of northern European descent. Analysis of a large African-American MS dataset permitted the identification of DRB1, and exclusion of DQA1, as the origin of the MS signal. A subset of MS cases occur in the setting of familial susceptibility to MS and also to multiple other autoimmune diseases - including autoimmune thyroid disease, psoriasis, ulcerative colitis, and lupus erythematosis. In such families, common in "familial" MS populations, the occurrence of autoimmunity is associated with variance of the CTLA4 gene. Thus, in some patients MS may arise in the setting of a generalized predisposition to autoimmunity. Genetic studies, likely combined with biomarkers that help to stratify different subsets of patients with MS, have the potential to identify who is at risk for the disease, assess prognosis, select the most appropriate therapy, and even make possible identification of presymptomatic high-risk individuals. (3)
Disease/Diagnosis Multiple Sclerosis
Treatment Review (1)
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. http://www.ncbi.nlm.nih.gov/pubmed/9189045 2. Barton JJ, Cox TA. Acquired pendular nystagmus in multiple sclerosis: clinical observations and the role of optic neuropathy. J Neurol Neurosurg Psychiatry. 1993;56:262-267. http://www.ncbi.nlm.nih.gov/pubmed/8459242 3. Barton JJ, Cox TA, Digre KB. Acquired convergence-evoked pendular nystagmus in multiple sclerosis. J Neuroophthalmol 1999;19:34-38. http://www.ncbi.nlm.nih.gov/pubmed/10098546 4. Dehaene I., Van Zandycke M, Appel B. Acquired pendular nystagmus. Neuro-ophthalmol 1987;7(5);297-300. 5. Keane JR. Acute vertical ocular myoclonus. Neurology 1986;36:86-89. http://www.ncbi.nlm.nih.gov/pubmed/3941790 6. Leigh JR and 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. 7. Lopez LI, Gresty MA, Bronstein AM, DuBoulay EP, Rudge P. Acquired pendular nystagmus: oculomotor and MRI findings. Acta Oto-Laryngologica 1995;285-287. http://www.ncbi.nlm.nih.gov/pubmed/8749141 8. Washio N, Suzuki Y, Yamaki T, Kase M, Ohtsuka K. Vertical-torsional oscillations and dissociated bilateral horizontal gaze palsy in a patient with a pontine cavernous angioma. J Neurol Neurosurg Psychiatry 2005;76:283-285. http://www.ncbi.nlm.nih.gov/pubmed/15654054
Relation is Part of 3-2, 167-6, 923-1, 927-1, 936-4, 941-3, 943-1
Contributor Secondary Ray Balhorn, Digital Video Compressionist; Steve Smith, Videographer
Reviewer David Zee, M.D., The Johns Hopkins University Hospital, 2007. Frederick Hochberg, M.D, Massachusetts General Hospital, 2007.
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1989
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: 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/s6c27t1n
Setname ehsl_novel_shw
Date Created 2005-08-22
Date Modified 2017-11-22
ID 188535
Reference URL https://collections.lib.utah.edu/ark:/87278/s6c27t1n
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