| Identifier |
163-155 |
| Title |
Bilateral Internuclear Ophthalmoplegia (INO) |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
Stephen Hauser, MD; Anne Osborn, MD; David Zee, MD; Steve Smith, Videographer |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital; (SH) University of California, San Francisco, California; (AO) Professor of Radiology, University of Utah, Salt Lake City, Utah; (DZ) Johns Hopkins University, Baltimore, Maryland |
| Subject |
Bilateral Internuclear Ophthalmoplegia; Abducting Nystagmus; Upbeat Nystagmus on Upgaze; Oscillopsia; Multiple Sclerosis |
| History |
The patient is a 48 year old woman who presented with horizontal double vision looking to the right and to the left. She also noted blurring of vision with the image jerking horizontally back and forth. Neuro-ophthalmological examination: Visual acuity 20/20 OU corrected Visual fields, pupils, and fundus examination normal. Ocular Motility showed: Paresis of adduction of the right eye on gaze left Abducting nystagmus of the left eye on gaze left Paresis of adduction of the left eye on gaze right Abducting nystagmus of the right eye on gaze right Normal convergence Upbeat nystagmus on upgaze Diagnosis: Bilateral internuclear ophthalmoplegia (INO) Lumbar puncture: Cerebrospinal fluid normal. Brain MRI studies were unavailable in 1974 when the patient presented. Diagnosis: Bilateral internuclear ophthalmoplegia Prognosis: On follow-up two months later, the eye movements were normal. Diagnosis: Multiple Sclerosis (MS) MS is the commonest cause of a bilateral internuclear ophthalmoplegia in a young adult. Brainstem infarction is the commonest cause of a unilateral internuclear ophthalmoplegia in middle aged and elderly adults. A bilateral internuclear ophthalmoplegia in a child raises the possibility of a 4th ventricle tumor. (ID163-6). The patient is a little boy who presented with a bilateral INO and was found to have a medulloblastoma. |
| Anatomy |
The medial longitudinal fasciculus (MLF) is a major pathway in the brainstem extending from the pons up to the midbrain. The MLF carries signals for the control of horizontal eye movements. For horizontal gaze: 1. The MLF contains axons from the abducens internuclear neurons and carries signals for horizontal saccades, the vestibulo-ocular reflex (VOR), and smooth pursuit. 2. These axons project to the medial rectus motor neurons in the contralateral oculomotor (third nerve) nucleus. For vertical gaze: 1. The MLF contains axons from the rostral interstitial nucleus of the MLF (riMLF) which carry vertical saccadic signals. 2. The MLF also contains ascending axons from the vesitibular nuclei which carry signals for the vertical VOR, smooth pursuit, gaze holding and otolith-ocular reflex. 3. Axons project to the oculomotor and trochlear (fourth nerve) nuclei, as well as to the Interstitial Nucleus of Cajal. |
| Pathology |
Review (7) |
| Disease/Diagnosis |
Multiple Sclerosis |
| Clinical |
This patient with MS had a bilateral INO with • Paresis of adduction of the right eye on gaze left • Abducting nystagmus of the left eye on gaze left • Paresis of adduction of the left eye on gaze right • Abducting nystagmus of the right eye on gaze right • Normal convergence • Upbeat nystagmus on upgaze Diagnosis: Bilateral Internuclear Ophthalmoplegia. Multiple Sclerosis (MS) is the commonest cause of a bilateral internuclear ophthalmoplegia in a young adult. The Clinical Features of an INO are: 1. Medial rectus muscle weakness ipsilateral to the side of the lesion with paresis of adduction or adduction lag. 2. Abducting nystagmus of the eye contralateral to the lesion - Dissociated nystagmus 3. Normal convergence 4. Skew deviation - hypertropia on the side of the lesion 5. Dissociated vertical nystagmus - downbeat with greater torsional component in the contralateral eye Bilateral INO with bilateral lesions of the MLF may also have Gaze evoked vertical nystagmus Impaired vertical pursuit Decreased vertical vestibular response Small amplitude saccadic intrusions suggesting involvement of the brainstem adjacent to the MLF Weakness of adduction is due to impaired conduction in axons from the abducens internuclear neurons which project to the medial rectus motor neurons in the contralateral oculomotor (third nerve) nucleus. Adduction weakness is most evident during saccades and adduction lag is brought out clinically by asking the patient to look all the way to the right and all the way to the left (i.e. make large saccades) back and forth across the midline. The speed of the adducting eye depends on a strong agonist contraction. The adducting saccade may be slow and hypometric. In the abducting eye, abducting saccades are hypometric with centripetal drifts of the eye and slowing. A series of small saccades and drifts have the clinical appearance of abducting nystagmus - dissociated nystagmus. Dissociated nystagmus may be due to: 1. Impaired ability to inhibit the affected medial rectus or 2. Dissociated nystagmus reflects the brain's attempts to compensate for the adduction weakness. For further discussion review Leigh JR, Zee DS. Diagnosis and Central Disorders of Ocular Motility Chp 12 Pg620-627 In The Neurology of Eye Movements 4th Edition Oxford University Press, New York 2006. Skew deviation, commonly seen in unilateral INO, is due to interruption of central projections in the otolithic pathway ascending in the MLF to the midbrain. The higher eye (hypertrophic) is usually on the side of the MLF lesion. Interruption of pathways mediating the vertical vestibulo-ocular reflex (VOR) may cause downbeat nystagmus with a greater torsional component in the eye contralateral to a unilateral INO. Oscillopsia, an illusion of movement of the visual world, is a common presenting symptom of INO. Horizontal oscillopsia usually occurs from either the adduction lag or the abducting nystagmus. Vertical oscillopsia occurs during head movements and is caused by a deficient vertical VOR or, as in this case, by pendular vertical oscillations. |
| Presenting Symptom |
Diplopia |
| Ocular Movements |
Bilateral Internuclear Ophthalmoplegia; Abducting Nystagmus; Normal convergence; Upbeat Nystagmus on Upgaze |
| Neuroimaging |
Neuroimaging studies were not available in this patient. Illustrative images in another MS case are shown here. Figure 1 MRI Axial FLAIR scan showing white matter foci of increased signal intensity surrounding cavitating areas characteristic of long-standing MS. Figure 2 MRI axial FLAIR scan of deep periventricular foci of increased signal intensity surrounding cavitating areas Figure 3 MRI sagittal FLAIR scan with classic calloseptal deep periventricular foci perpendicular to ventricle surface classic for Dawson fingers Courtesy of Anne Osborn, M.D. To view neuroimaging of the brain in MS go to PowerPoint Presentation "Brain MRI in Multiple Sclerosis". See link in Get Media field above. |
| Treatment |
No treatment was prescribed. Review (3) |
| Etiology |
Table 12-3 Etiology of Internuclear Ophthalmoplegia pg 622 (5) |
| Supplementary Materials |
Brain MRI in Multiple Sclerosis: https://collections.lib.utah.edu/details?id=2174181 Lessons from the Bench and Bedside: https://collections.lib.utah.edu/details?id=2174205 |
| Date |
1974 |
| References |
1. Gordon RM, Bender MB. Visual phenomenon in lesions of the medial longitudinal fasciculus. Arch Neurol 1966;15:238-240. http://www.ncbi.nlm.nih.gov/pubmed/5912004 2. Gresty MA, Hess K, Leech J. Disorders of the vestibuloocular reflex producing oscillopsia and mechanisms compensating for loss of labyrinthine function. Brain 1977;100:693-716. http://www.ncbi.nlm.nih.gov/pubmed/305272 3. Hauser SL, Goodin DE. Multiple Sclerosis and other demyelinating diseases in Harrison's Principals of Internal Medicine, 16th Edition, Kasper DL et al eds. McGraw-Hill, New York, 2005. 4. Keane JR. Internuclear ophthalmoplegia: unusual causes in 114 of 410 patients. Arch Neurol. 2005;62(5):714-717. http://www.ncbi.nlm.nih.gov/pubmed/15883257 5. Leigh JR, Zee DS. Diagnosis of Central Disorders of Ocular Motility. Chp 12 pg 598-718. In: The Neurology of Eye Movements, 4th Edition, Oxford University Press, New York 2006 6. Muri RM, Meienberg O. The clinical spectrum of internuclear ophthalmoplegia in multiple sclerosis. Arch Neurol 1985, 42: 851-855. http://www.ncbi.nlm.nih.gov/pubmed/4026628 7. Strominger MB, Mincy EJ, Strominger AI, Strominger NL. Bilateral internuclear ophthalmoplegia with absence of convergence eye movements. Clinico-pathological correlations. J Clin Neuro-ophthalmol 1986,6:57-65. http://www.ncbi.nlm.nih.gov/pubmed/3009554 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
16 mm Film |
| Relation is Part of |
163-6, 168-6, 906-4, 933-1, 937-8, 941-2, 941-3 |
| 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/s6zg9ps1 |
| Setname |
ehsl_novel_shw |
| ID |
188600 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6zg9ps1 |
