Pseudo-Internuclear Ophthalmoplegia

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Identifier 163-11
Title Pseudo-Internuclear Ophthalmoplegia
Ocular Movements Pseudo-internuclear Ophthalmoplegia; Bilateral Weakness of Adduction
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 Pseudo-internuclear Ophthalmoplegia; Bilateral Weakness of Adduction; Tensilon Test; Ocular Myasthenia Gravis; Bilateral Myasthenia Gravis
Supplementary Materials PowerPoint Presentation: Ocular Myasthenia Gravis: Past, Present and Future: Victoria S. Pelak, M.D. University of Colorado Health Sciences Center
Presenting Symptom Difficulty focusing, double vision
History In 1969 this 54 year old man presented with a one month history of blurry vision which started suddenly one afternoon. He said he was "just not focusing" and he saw two images when he "relaxed his sight". He could see clearly covering one eye. Initially, the difficulty in focusing occurred late in the afternoon but as it progressed it appeared earlier and earlier each day. Finally he awoke in the morning with double vision. Past History: Three years prior to admission he had two or three similar transient episodes of blurred vision. On one occasion this occurred at the end of a bowling session when the pins appeared out of focus. Negative for strabismus as a child In 1967 had an episode of chest pain A cardiogram ruled out myocardial infarct Family History: Negative for neurologic disease Symptomatic Inquiry: Negative for generalized fatigue, droopy eyelids, difficulty chewing, swallowing or respiratory difficulty. Neuro-ophthalmic Examination: Visual acuity J1 OU with glasses Visual fields, pupils and fundus examination normal. Ocular Motility: No ptosis Adduction weakness of the left eye which could not cross the midline on gaze right. Adduction weakness of the right eye which could not cross the midline on gaze left. No abduction nystagmus Full vertical gaze Normal convergence Deviation of the eyes up under forced eye closure (Bell's phenomenon) Horizontal oculocephalic reflex impaired adduction. Vertical oculocephalic reflex normal. Intravenous Tensilon Test (edrophonium chloride): Patient received a test dose of 0.1 ml no side effects Next dose of 0.3 ml resulted in immediate recovery of full adduction of both eyes. The full dose of 1 ml (10 mg) of tensilon was not given as the test was positive. Antibody Studies: This patient was seen in the 1970's and predated the detection of antiacetylcholine receptor antibodies. Chest X-Ray: Cardiac silhouette normal No mediastinal mass. Diagnosis: Pseudo-Internuclear Ophthalmoplegia Ocular Myasthenia Gravis An Oculoelectromyogram was recorded with an indwelling needle electrode in the right medial rectus, inserted by Dr. Cogan. Following intravenous tensilon there was a recordable increase in the muscle action potential indicating a positive test confirming myasthenia gravis. This is the only patient that Dr. Cogan and I studied using electromyography of an extraocular muscles. Hospital Course: Within two weeks, he developed bilateral ptosis. Medication: Mestinon 60 mg 1 tab t.i.d. with good recovery . Follow-Up: He was followed for ten years and never developed signs of generalized myasthenia gravis. New Presentation: In 1980 he presented with a one month history of polyarthralgias and swelling and redness of the hands, knees and periarticular joints. Chest X-Ray showed: Pulomonary infiltrates in a bilateral perihilar distribution. General Examination: Low grade fever, hacking cough, hemoptysis and palpable purpuric lesions of the skin. Lung and skin biopsy performed. Pathology: Idiopathic leukoplastic primary vasculitis Hospital Course: His respiratory reserve became severely diminished and he went into respiratory failure and required intubation. He died two weeks after admission. Final Diagnosis: Idiopathic leukoplastic primary vasculitis Pulmonary hemorrhage. Associated diagnosis: Ocular Myasthenia Gravis
Clinical This patient with pseudo-internuclear ophthalmoplegia illustrates how closely bilateral weakness of adduction due to ocular myasthenia gravis can mimic a brainstem bilateral internuclear ophthalmoplegia due to a lesion of the medial longitudinal fasciculus in the brainstem. The significant signs are: • No nystagmus of the abducting eye • No ptosis • Full vertical gaze • A positive intravenous tensilon test with • Full recovery of adduction after 0.4 ml of tensilon. This patient was initially admitted on the stroke service for a vascular workup and possible vertebral angiogram.
Neuroimaging No neuro-imaging studies are available in this patient.
Anatomy Ptosis: Ptosis is defined as the lid covering more than 2 mm of the cornea. Ptosis is routinely measured by documenting the width of the palpebral fissure in millimeters with the eye in primary gaze and the eyebrow held down. Approximately 50% of patients with ocular myasthenia gravis present with ptosis. More than 90% eventually develop eye movement abnormalities and typical ocular myasthenia. Of those patients who present only with ocular symptoms, half persist with purely ocular myasthenia and half go on to develop generalized myasthenia gravis. Of those who develop generalized myasthenia gravis, most do so within 2 years of the onset of ocular symptoms.
Pathology Myasthenia gravis (MG) is an autoimmune disease caused by sensitized T-helper cells and an IgG-directed attack on the nicotinic acetylcholine receptor of the neuromuscular junction (NMJ). The mechanism of antibody damage to the receptor and motor endplate probably involves several steps. 1. There is a complement-directed attack with the destruction of acetylcholine receptor and the junctional folds. 2. Binding of the antibody to the receptor can cause receptor blockade. 3. The abnormal and reduced numbers of acetylcholine receptors lead to impaired NMJ transmission. 4. In post synaptic disorders such as MG, the number of quanta of acetylcholine released by each nerve stimulus is normal, but the effect of each quantum on its receptor is reduced. 5. The net result is a lower endplate potential and a reduced safety factor of transmission at the NMJ. Clinically this manifests as pathologic fatigability, that is, progressive muscle weakness with use - the hallmark of MG. Patients typically improve after rest or upon arising in the morning, with worsening as the day passes. In MG, fatigue is limited to muscular fatigue alone and often progresses to frank muscle weakness.
Etiology Autoimmune disease
Disease/Diagnosis Ocular Myasthenia Gravis
Treatment Mestinon 60 mg. 1 tablet t.i.d.
References 1. Cogan DG. Myasthenia gravis: a review of the disease and a description of lid twitch as a characteristic sign. Arch Ophthalmol 1965;74:217-221. 2. Cogan DG, Yee RD, Gittinger J. Rapid eye movements in myasthenia gravis. I Clinical observations. Arch Ophthalmol 1976;94:1083-1085. 3. Daroff RD. The office tensilon test for ocular myasthenia gravis. Arch Neurol 1986:43:843-844. 4. Elrod RD, Weinberg DA. Ocular myasthenia gravis. Ophthalmol Clin North Am 2004;17:275. 5. Glaser JS. Myasthenic psuedo-internuclear ophthalmoplegia. Arch Ophthalmol. 1966 Mar;75(3):363-366. 6. Kaminski HJ, LI Z, Richmonds C, Ruff RL, Kusner L. Susceptibility of Ocular tissues to Autoimmune Diseases. Ann N.Y. Acad Sci 2003;998:362-374. 7. Kupersmith MJ, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol 2003;60:243-248. 8. Leigh JR,Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Ch 9:385-474. In: The Neurology of Eye Movements 4th Edition Oxford University Press, NY 2006. 9. Meriggioli MN, Sanders DB. Myasthenia gravis: diagnosis. Semin Neurol 2004;24:31. 10. Pelak VS, Quan D. Ocular Myasthenia Gravis. In: Rose BD (Ed) UpToDate, Wellesley, MA. 2006 11. Pierrot-Deseilligny C, Michelin T. Myasthenic internuclear pseudo-ophthalmoplegia. Rev Neurol (Paris). 1983;139:527-528. 12. Swick HM. Pseudointernuclear ophthalmoplegia in acute idiopathic polyneuritis (Fisher's syndrome). Am J Ophthalmol. 1974;77(5):725-728. 13. Valls-Canals J, Povedano M, Montero J, Pradas J. Stimulated single-fiber EMG of the frontalis and orbicularis oculi muscles in ocular myasthenia gravis. Muscle Nerve 2003;28:501-503. 14. Vincent A, Newsom-Davis J. Acetylcholine receptor antibody as a diagnostic test for myasthenia gravis: results in 153 validated cases and 2967 diagnostic assays. J Neurol Neurosurg Psychiatry 1985;48:1246-1252. 15. Vincent A, Newsom-Davis J. Anti-acetylcholine receptor antibodies. J Neurol Neurosurg Psychiatry 1980;43:590-600. 16. Wray SH, Pavan-Langston D. A reevaluation of edrophonium chloride (Tensilon) tonography in the diagnosis of myasthenia gravis. Neurology 1971;21:586-593.
Relation is Part of 163-10, 166-25
Contributor Secondary Ray Balhorn, Video Compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1969
Type Image/MovingImage
Format video/mp4
Source 16 mm Flim
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/s6808075
Setname ehsl_novel_shw
Date Created 2007-03-09
Date Modified 2017-11-22
ID 188590
Reference URL