Idiopathic Ocular Neuromyotonia: a Neurovascular Compression Syndrome?

Total Page:16

File Type:pdf, Size:1020Kb

Idiopathic Ocular Neuromyotonia: a Neurovascular Compression Syndrome? 642 J Neurol Neurosurg Psychiatry 2000;69:642–644 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.642 on 1 November 2000. Downloaded from SHORT REPORT Idiopathic ocular neuromyotonia: a neurovascular compression syndrome? C Tilikete, C Vial, M Niederlaender, P L Bonnier, A Vighetto Abstract specifically there was no history of radiation Ocular neuromyotonia in the muscles treatment. innervated by the right oculomotor nerve Neuro-ophthalmological examination was diagnosed in a patient without a showed normal visual acuity, visual field, and history of radiation therapy. Electromyog- fundi. Between episodes, the patient had mild raphy of the levator palpebrae showed ptosis, poorly reactive miosis, and slight limita- continuous motor unit activity. Brain MRI tion of infraduction and supraduction of the disclosed a close contact between the right right eye. Orthoptic measurements showed a third cranial nerve and a basilar artery small exophoria worsening on left gaze, a right dolichoectasia. The patient partly ben- hypophoria in primary and far up gaze, and a efited from carbamazepine therapy. This small right hyperphoria in far down gaze. unique finding suggests that neurovascu- There was no evidence of primary misdirection lar compression syndrome could be an phenomena. Her right pupil was 2.4 mm and hitherto unrecognised cause of ocular the left pupil was 2.8 mm diameter. Cocaine neuromyotonia. instillation resulted in a bilateral pupil dilation (J Neurol Neurosurg Psychiatry 2000;69:642–644) (RE=3.5 mm and LE=3.7 mm) ruling out oculosympathetic paresis. Ptosis and limitation copyright. Keywords: ocular neuromyotonia; neurovascular com- pression syndrome; carbamazepine of eye movements suggested a minimal right Neuro-ophthalmology third nerve palsy. Neurological examination Unit, Hôpital was otherwise normal. Neurologique et Diplopic paroxysms could occur spontane- Neurochirurgical P Ocular neuromyotonia is a rare paroxysmal Wertheimer, 59 Bd involuntary contraction of one or more ocular ously but were often triggered by holding the Pinel, 69003 Lyon, motor muscles resulting in paroxysmal diplopia eyes upwards for 20 to 30 seconds. During an France and strabismus. Among 29 patients reported in attack, the right eye showed mild esotropia, C Tilikete the literature, 16 had received radiation marked limitation of supraduction, and infra- A Vighetto therapy to the parasellar and sellar regions a duction with lid retraction, mild limitation of 1–6 Electromyography few months to 18 years before. In seven abduction, and no change in the pupil size. Unit, Hôpital cases, ocular neuromyotonia resulted from Paroxysmal diplopia could not be triggered by http://jnnp.bmj.com/ Neurologique et compressive lesions such as a clivus chordoma, hyperventilation. Neurochirurgical P a supraclinoid carotid aneurysm, a Paget bone Electromyography with a concentric needle Wertheimer, 59 Bd dystrophy, a mycormycosis of the cavernous (diameter=0.3 mm) was normal, showing no Pinel, 69003 Lyon, 3–5 7 spontaneous activity or myotonia in the first France sinus, arachnoiditis, and Graves’ disease. In C Vial the six remaining patients, no lesion was dorsal interosseus, the deltoid, and the orbicu- found.3589We document with EMG and MRI laris oculi. However, EMG was abnormal in Neuro-radiology Unit, a unique case of neurovascular compression the right levator palpebrae, disclosing sponta- Hôpital Neurologique syndrome associated with idiopathic ocular neous continuous muscle unit activity, with on September 30, 2021 by guest. Protected et Neurochirurgical P neuromyotonia. brief bursts of motor unit potentials, firing at Wertheimer, 59 Bd high frequency. Voluntary contraction of this Pinel, 69003 Lyon, France muscle was normal and no afterdischarges M Niederlaender Case report were recorded. A 67 year old man presented with a 1 month Brain CT (GE, slice thickness=5 mm) with Radiology Unit, history of episodic diplopia. He reported spells and without contrast enhancement did not Clinique de la of horizontal or oblique double vision that show any brain stem lesion but disclosed a Sauvegarde, Av des sources, 69 009 Lyon, occurred spontaneously five or six times a day, dolichoectasia of the basilar artery displaying a France lasting less than 10 seconds. While complain- large right sided loop anterior to the mesen- P L Bonnier ing of diplopia, his wife noticed a transient cephalon. T2 MRI (Philips Gyroscan T5-NT; esodeviation of the right eye. He was symptom 0.5 tesla with head coil; TR=5000 ms, TE=450 Correspondence to: free between attacks. ms, TF=69; slice thickness=1 mm) of the brain Dr Caroline Tilikete [email protected] History disclosed abdominal aortic aneu- stem using fast spin echo high resolution rysm, lumbar sympathectomy for arteriopathy, (FSE-HR) in the plane of the third nerve was Received 6 December 1999 and asymptomatic 50% stenosis of the left performed. It showed a contact between the and in revised form 24 February 2000 carotid artery. There was no history of ocular loop of the dolichoectasic basilar artery and the Accepted 6 March 2000 motor, anisocoria, or neurological disorder and right oculomotor nerve in the premesen- www.jnnp.com Idiopathic ocular neuromyotonia: a neurovascular compression syndrome? 643 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.642 on 1 November 2000. Downloaded from FSE-HR T2 MRI of the brain stem in the plane of the third nerves showing a contact between the loop of the dolichoectasic basilar artery and the right oculomotor nerve (arrows) in the premesencephalic fossa, close to the root exit zone. cephalic fossa, close to the root exit zone paretic pupil dilation, rarely as transient pupil (figure). contraction occurring during the diplopic The patient was treated with 100 mg spells, and in one recent case as permanent carbamazepine daily. He subjectively reported tonic pupil.37Right miosis of our patient could a 50% decrease of spell frequency within a few either be related to unknown physiological ani- days. However, on examination, the same par- socoria or permanent tonic pupil related to copyright. oxysms of ocular spasms could still be triggered ocular neuromyotonia. Other permanent exci- by holding the eyes upward. Finally, car- tatory manifestations in the third nerve de- bamazepine was poorly tolerated even at the pendent muscles have been reported, such as low dose and was stopped. Spell frequency lid retraction and pupil synkinesis. then returned to its pretest level. Gabapentine Neuromyotonia (not limited to ocular neu- (300 mg three times a day) and acetazolamide romyotonia) is a syndrome of spontaneous (250 mg twice a day) trials yielded no benefit. muscle hyperactivity that can be triggered by Aftera1yearfollow up, the patient remained voluntary muscle contraction, due to abnormal medication free and still had the same eye excitability of peripheral nerve motor axons.10 movement disorder. Neuromyotonia belongs to the syndromes of continuous motor unit activity which encom- Discussion pass diVerent diseases that vary in their clinical http://jnnp.bmj.com/ The paroxysmal limitation of right eye move- presentation and associated electrophysiologi- ments that occurred in this patient was typical cal abnormalities, but have in common sus- of ocular neuromyotonia aVecting muscles tained involuntary motor unit activity.10 In innervated by the oculomotor nerve. Indeed, continuous motor unit activity, EMG shows the clinical hallmark of ocular neuromyotonia continuous hyperactivity such as neuromyot- is transient diplopia and strabismus that onic and myokimic discharges and fascicula- develop spontaneously and after holding ec- tions. Continuous motor unit activity is associ- centric gaze, due to a tonic contraction or ated with peripheral motor nerve diseases, on September 30, 2021 by guest. Protected spasm of ocular muscles.8 In this patient, either in a diVuse or a focal distribution.10 sustained upgaze induced spasms of the levator Ocular neuromyotonia is likely to be related to palpebrae and superior rectus of the right eye focal continuous motor unit activity. Indeed, in with resulting lid retraction and limitation of the first reported cases of ocular neuromyoto- infraduction. Concomitant spasms of inferior nia extraocular muscle EMG showed neuro- oblique, inferior rectus, and medial rectus best genic abnormalities.12 Since then, the disease explained the limitation of supraduction and has been clinically recognised without EMG abduction of the same right eye. This coactiva- investigation. In our patient, we were able to tion of muscles related to the same ocular record EMG activity in the levator palpebrae, motor nerve is a frequent symptom of ocular and we showed unambiguous signs of continu- neuromyotonia.3 Our case associated ocular ous motor unit activity. This result indicates neuromyotonia with a chronic third nerve defi- that ocular neuromyotonia, at least in our case, cit. Half of the reported cases of ocular neuro- meets diagnosis criteria for focal continuous myotonia have also a chronic ocular motor motor unit activity. nerve involvement. Involvement of pupil motil- Radiation induced plexus lesion is a well ity has been rarely reported in ocular neuro- established cause of focal limb continuous myotonia of the third nerve, as permanent motor unit activity. Many cases of ocular neu- www.jnnp.com 644 Tilikete, Vial, Niederlaender, et al J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.642 on 1 November 2000. Downloaded from romyotonia
Recommended publications
  • Cranial Nerve Palsy
    Cranial Nerve Palsy What is a cranial nerve? Cranial nerves are nerves that lead directly from the brain to parts of our head, face, and trunk. There are 12 pairs of cranial nerves and some are involved in special senses (sight, smell, hearing, taste, feeling) while others control muscles and glands. Which cranial nerves pertain to the eyes? The second cranial nerve is called the optic nerve. It sends visual information from the eye to the brain. The third cranial nerve is called the oculomotor nerve. It is involved with eye movement, eyelid movement, and the function of the pupil and lens inside the eye. The fourth cranial nerve is called the trochlear nerve and the sixth cranial nerve is called the abducens nerve. They each innervate an eye muscle involved in eye movement. The fifth cranial nerve is called the trigeminal nerve. It provides facial touch sensation (including sensation on the eye). What is a cranial nerve palsy? A palsy is a lack of function of a nerve. A cranial nerve palsy may cause a complete or partial weakness or paralysis of the areas served by the affected nerve. In the case of a cranial nerve that has multiple functions (such as the oculomotor nerve), it is possible for a palsy to affect all of the various functions or only some of the functions of that nerve. What are some causes of a cranial nerve palsy? A cranial nerve palsy can occur due to a variety of causes. It can be congenital (present at birth), traumatic, or due to blood vessel disease (hypertension, diabetes, strokes, aneurysms, etc).
    [Show full text]
  • Strabismus: a Decision Making Approach
    Strabismus A Decision Making Approach Gunter K. von Noorden, M.D. Eugene M. Helveston, M.D. Strabismus: A Decision Making Approach Gunter K. von Noorden, M.D. Emeritus Professor of Ophthalmology and Pediatrics Baylor College of Medicine Houston, Texas Eugene M. Helveston, M.D. Emeritus Professor of Ophthalmology Indiana University School of Medicine Indianapolis, Indiana Published originally in English under the title: Strabismus: A Decision Making Approach. By Gunter K. von Noorden and Eugene M. Helveston Published in 1994 by Mosby-Year Book, Inc., St. Louis, MO Copyright held by Gunter K. von Noorden and Eugene M. Helveston All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior written permission from the authors. Copyright © 2010 Table of Contents Foreword Preface 1.01 Equipment for Examination of the Patient with Strabismus 1.02 History 1.03 Inspection of Patient 1.04 Sequence of Motility Examination 1.05 Does This Baby See? 1.06 Visual Acuity – Methods of Examination 1.07 Visual Acuity Testing in Infants 1.08 Primary versus Secondary Deviation 1.09 Evaluation of Monocular Movements – Ductions 1.10 Evaluation of Binocular Movements – Versions 1.11 Unilaterally Reduced Vision Associated with Orthotropia 1.12 Unilateral Decrease of Visual Acuity Associated with Heterotropia 1.13 Decentered Corneal Light Reflex 1.14 Strabismus – Generic Classification 1.15 Is Latent Strabismus
    [Show full text]
  • Internuclear Ophthalmoplegia As a Presenting Feature in a COVID-19-­Positive Patient Varshitha Hemanth Vasanthpuram,1 Akshay Badakere ‍ ‍ 2
    Case report BMJ Case Rep: first published as 10.1136/bcr-2021-241873 on 13 April 2021. Downloaded from Internuclear ophthalmoplegia as a presenting feature in a COVID-19- positive patient Varshitha Hemanth Vasanthpuram,1 Akshay Badakere 2 1Ophthalmic Plastic Surgery SUMMARY was unremarkable. His vitals at the time of screening Services, LV Prasad Eye Institute, A 58-year -old man presented with vertical diplopia were 94% saturation of peripheral oxygen (SpO2), Hyderabad, India temperature of 35.8°C and pulse rate of 91 per 2 for 10 days which was sudden in onset. Extraocular Child Sight Institute, Jasti V movement examination revealed findings suggestive minute. His overall systemic status was stable, with Ramanamma Children’s Eye of internuclear ophthalmoplegia. Investigations were no respiratory symptoms noted. Care Centre, LV Prasad Eye Institute, Hyderabad, India suggestive of diabetes mellitus, and reverse transcription- PCR for SARS-CoV -2 was positive. At 3 weeks of INVESTIGATIONS follow-up , his diplopia had resolved. Neuro-ophthalmic Correspondence to Extraocular motility examination, the abducting manifestations in COVID-19 are increasingly being Dr Akshay Badakere; nystagmus in the left eye and the saccades were akshaybadakere@ gmail.com recognised around the world. Ophthalmoplegia due indicative of INO. Fatigue and ice pack test were to cranial nerve palsy and cerebrovascular accident negative. Initial blood investigations of complete Accepted 26 March 2021 in COVID-19 has been reported. We report a case blood count, lipid profile and 24- hour urine protein of internuclear ophthalmoplegia in a patient with were within normal range. Fasting and postprandial COVID-19. blood sugar levels were 121 mg/dL and 205 mg/dL, respectively, and haemoglobin A1c (HbA1c) was 7.5%, suggestive of diabetes mellitus.
    [Show full text]
  • Diplopia Evaluation
    Diplopia [ ] Monocular (monocular if diplopia present even when only 1 eye open) vs [ ] Binocular (binocular only when both eyes open) Causes If painful, think of the following: Red Flags Monocular diplopia Binocular diplopia usually d/t something usually d/t disconjugate Compressive lesion/tumor/aneurysm >1 cranial nerve deficit distorting light through alignment of eyes eye to retina Sinusitis/abscess/cavernous sinus thrombosis Pupillary involvement cataract CN palsy (3rd, 4th, 6th) corneal shape problems Myasthenia gravis Orbital myositis Other neurologic s/s alongwith diplopia (keratoconus) uncorrected refractive Orbital infiltration (e.g. Trauma (fracture/hematoma) Pain error (usually thyroid infiltrative astigmatism) ophthalmopathy, orbital pseudotumor) Skull base tumors (pain often unrelated to eye movement) Proptosis other: corneal scarring, Other causes: CVA dislocated lens, affecting pons/midbrain; malingering compressive lesion (aneurysm, tumor); History idiopathic; inflammatory/infectious (sinusitis, cavernous sinus monocular vs binocular? gait difficulties (CN 8) thrombosis, abscess); Wernicke’s ; orbital myositis; trauma intermittent or constant? difficulty with bladder control (MS) (fracture, hematoma); tumors near base of skull/sinuses/orbits; images separated horizontally or vertically or a weakness/sensory abnormalities (intermittent or botulism; GBS/Miller- combination of both? constant) Fisher; MS vision changes? (CN2) N/V/diarrhea (botulism) Key points numbness of forehead/face/cheek (CN5) swallowing or speech difficulties
    [Show full text]
  • Diplopia Following Cataract Surgery: a Review of 150 Patients
    Eye (2008) 22, 1057–1064 & 2008 Nature Publishing Group All rights reserved 0950-222X/08 $30.00 www.nature.com/eye Diplopia following H Nayak, JP Kersey, DT Oystreck, RA Cline and CLINICAL STUDY CJ Lyons cataract surgery: a review of 150 patients Abstract Eye (2008) 22, 1057–1064; doi:10.1038/sj.eye.6702847; published online 27 April 2007 Aim To study the motility pattern, underlying mechanism, and management of Keywords: cataract; diplopia; strabismus; patients who complained of double vision anaesthesia after cataract surgery. Methods A retrospective case note analysis of 150 patients presenting with diplopia after cataract surgery to an orthoptic clinic over a Introduction 70-month period. Information was retrieved from orthoptic, ophthalmological, and The recent rapid evolution of cataract surgical operating room records. technique has made this one of the most Results A total of 3% of patients presenting commonly performed and successful surgical to the orthoptic clinic had diplopia after procedures. However, the substantial benefit of cataract surgery. We grouped these according visual acuity improvement resulting from to the underlying mechanisms which were: cataract extraction can be reduced by the (1) decompensating pre-existing strabismus introduction of post-operative diplopia. Most of (34%), (2) extraocular muscle restriction/ the recent literature regarding the cause of this paresis (25%), (3) refractive (8.5%), complication1–20 has focused on anaesthetic (4) concurrent onset of systemic disease myotoxicity, trauma during infiltrational (5%), (5) central fusion disruption (5%), and anaesthesia, or the use of a rectus bridle suture. (6) monocular diplopia (2.5%). Twenty per cent In this study, we reviewed the motility of the patients could not be categorised with characteristics, likely aetiology, and Department of certainty.
    [Show full text]
  • Diplopia As the Presenting Symptom of Type 1 Diabetes
    Diabetes Care Volume 37, March 2014 e45 Diplopia as the Presenting Charlotte G. Krol, Frederikus A. Klok, and Eelco J.P. de Koning Symptom of Type 1 Diabetes Diabetes Care 2014;37:e45–e46 | DOI: 10.2337/dc13-2244 Neuropathy is a common complication was the diagnosis. Given his normal nerve, sparing the superomedial- of diabetes, in which cranial nerve BMI and rapidly progressive symptoms, concentrated pupillomotor fibers and palsies are rare and associated with type 1 diabetes was considered the thereby pupil function, in contrast to long-standing poorly controlled type 2 most likely diagnosis. Therefore, and palsies due to other diagnoses (4,5). diabetes. We report a case of a young because cranial mononeuropathy Differential diagnoses include patient with oculomotor nerve palsy as requires rapid correction of infectious diseases, aneurysms, the presenting symptom of type 1 hyperglycemia, insulin therapy was inflammatory disorders, tumors, diabetes. initiated immediately. Normoglycemia trauma, and surgery (4). Diabetic A 36-year-old previously healthy was achieved with a basal-bolus reg- oculomotor nerve palsies typically Sudanese man was referred to our imen and a daily dose of 0.67 units/kg recover over weeks to months emergency department because of within a few weeks. Four months after without sequelae. Management is ’ progressive diplopia of his right eye for the initial diagnosis, the patient socu- supportive, including optimal glycemic 7 days. At the emergency department, lomotor nerve palsy had improved controlaswellasminimizationofother he also reported weight loss, fatigue, dramatically, with almost complete risk factors for ischemia, including control of blood pressure and lipid excessive thirst, and polyuria in the resolution of symptoms.
    [Show full text]
  • Visual Symptoms and Findings in MS: Clues and Management
    6/5/2014 Common visual symptoms and findings in MS: Clues and Identification Teresa C Frohman, PA-C, MSCS Neuro-ophthalmology Research Manager, UT Southwestern Medical Center at Dallas Professor Biomedical Engineering, University of Texas Dallas COMMON COMPLAINTS 1 6/5/2014 Blurry Vision Corrected with Refraction? YES NO Refractive Keep Looking Error IN MS : ON, Diplopia, Nystagmus Most Common Visual Issues Encountered in MS patients • Optic Neuritis • Diplopia • Nystagmus result from damage to the optic nerve or from an incoordination in the eye muscles or damage to a part of the oculomotor pathway or apparatus 2 6/5/2014 Optic Neuritis Workup ‘frosted glass’ Part of visual field missing Pain +/- Color desaturation Work up for Yes diplopia or nystagmus Seeing double images YES NO Or ‘jiggling’ No Neuro-ophth exam Humphrey’s OCT MRI Fundoscopy CRANIAL NERVE ANATOMY There are 12 pairs of cranial nerves CN I Smell CN II Vision CN III, IV, VI Oculomotor CN V Trigeminal Sensorimotor muscles of the Jaw CN VII Sensorimotor of the face CN VIII Hearing//vestibular CN IX, X, XII Mouth, esophagus, oropharynx CN XI Cervical Spine and shoulder 6 3 6/5/2014 NEURO-OPHTHALMOLOGY EXAM Visual Acuity Color Vision Afferent pupillary reaction- objective test of CNII function Alternating flashlight test – afferent arc of pupillary light reflex pathway Fundus exam Visual Fields –confrontation at bedside CRANIAL NERVE II: OPTIC once the retinal ganglion cell axons leave the back of the eye they become myelinated behind the lamina cribosa ---and
    [Show full text]
  • Diplopia: Neuromuscular Junction
    DIPLOPIA: NEUROMUSCULAR JUNCTION Janet C. Rucker, MD New York University School of Medicine INTRODUCTION Myasthenia gravis (MG) is the most common disease of the neuromuscular junction. Ocular motor dysfunction in MG can mimic virtually any pupil-sparing abnormal eye movement, from pupil-sparing third nerve palsies to fourth and sixth nerve palsies to brainstem supranuclear gaze palsies to internuclear ophthalmoplegia. The pupil is not involved in MG. Diagnostic confusion often arises when the eye movements of MG mimic another disorder and ptosis is not present to raise suspicion of MG. It is always appropriate to keep MG in the differential diagnosis for any unexplained eye movement abnormality and to have a low threshold for pursuing diagnostic testing. Botulism from Clostridium botulinum neurotoxin blockade also affects neuromuscular junction transmission. The eye movements are similar to those seen in MG, with variable patterns of ophthalmoplegia. However, tonic pupillary involvement (with slow tonic reaction and re-dilation to light and light-near pupillary dissociation manifested as better reaction to a near stimulus than to a light stimulus) is typical of botulism, whereas the pupils are not affected in MG. A third disorder of the neuromuscular junction is the Lambert-Eaton syndrome (LEMS), which is due to pre- synaptic neuromuscular junction failure (in contrast to MG, which is a post-synaptic disorder). The primary clinical manifestation is skeletal muscle weakness. Up to half of patients have ptosis.1 However, eye movements are affected minimally, if at all and when affected are rarely the presenting clinical feature. This talk and the remainder of this syllabus will focus on MG.
    [Show full text]
  • Question: Monocular Or Binocular? Standard Office Visit
    Deciphering DIPLOPIA The symptom is diplopia. But what’s the diagnosis? When your patient sees two images, you may need to consider 20 possible causes, and ruling out 19 of those can be a headache. he various etiologies of diplopia because they’re not trained in techniques to measure ocular comprise one of the most sweeping alignment.” But the consequences of a delayed or incorrect differential diagnoses in all of oph- diagnosis are not comfortable, either. “Most double vision thalmology. The patient who com- is not dangerous. But some is super dangerous,” said An- plains of double vision can have drew G. Lee, MD. “You have to do a complete and thorough Tsomething as benign as dry eye or as life- exam to look for the distinctive dangerous sign. That sign threatening as an intracranial tumor. The could suggest a life-threatening diagnosis. It’s a red flag,” he cause may be as rare as Wernicke encephal- said. Dr. Lee is professor of ophthalmology, neurology and opathy or as common as convergence insuf- neurosurgery at Weill Cornell Medical College in New York ficiency. “It’s a huge differential diagnosis,” as well as chairman of ophthalmology at The Methodist said Nurhan Torun, MD, director of the Hospital in Houston, adjunct professor of ophthalmology at neuro-ophthalmology service at Beth Israel the University of Iowa in Iowa City and clinical professor of Deaconess Medical Center and an instruc- ophthalmology at the University of Texas in Galveston. tor of ophthalmology at Harvard Medical School in Boston. The exam of a diplopic patient demands a meticulous “Diplopia tends to be intimidating for many practitioners.” history and the posing of questions pertinent to the dispo- Intimidation may even turn to dread.
    [Show full text]
  • Clinical Outcomes and Aetiology of Fourth Cranial Nerve Palsy with Acute Vertical Diplopia in Adults
    Eye (2020) 34:1842–1847 https://doi.org/10.1038/s41433-019-0749-8 ARTICLE Clinical outcomes and aetiology of fourth cranial nerve palsy with acute vertical diplopia in adults 1 2 Shin Yeop Oh ● Sei Yeul Oh Received: 10 June 2019 / Revised: 2 December 2019 / Accepted: 9 December 2019 / Published online: 13 January 2020 © The Author(s), under exclusive licence to The Royal College of Ophthalmologists 2020 Abstract Background We investigated the clinical outcomes of fourth cranial nerve (CN4) palsy with acute vertical diplopia in adults. Methods A total of 80 patients with acute CN4 palsy who underwent at least 3 months of follow-up were included in this study. We retrospectively investigated the aetiology, rate of recovery, and factors associated with recovery between March 2016 and January 2019. Results The average age of patients with CN4 palsy was about 60 years, and the duration of recovery was 1.5 months: 48 (60.0%) patients had a vascular aetiology and 17 (21.3%) patients had a trauma history. Brain lesions were found in four (5.0%) patients and decompensated cause accounted for four (5.0%) cases. Among the total of 80 patients, 13 (16.3%) failed 1234567890();,: 1234567890();,: to completely recover. Non-isolated CN4 palsy with other cranial nerve palsies were recorded in seven cases. The com- parison between recovery and non-recovery groups showed that initial deviation angle, aetiology, fundus extorsion, and head tilt status were significantly different factors. Conclusion The recovery rate of acute CN4 palsy was about 80% and duration of recovery was 1.5 months.
    [Show full text]
  • Central Fourth Nerve Palsies Mitchell S.V
    RESIDENT &FELLOW SECTION Pearls and Oy-sters: Section Editor Central fourth nerve palsies Mitchell S.V. Elkind, MD, MS Daniel R. Gold, DO CLINICAL PEARLS Lesions of the fourth (trochlear) Clinical features suggestive of bilateral fourth nerve Robert K. Shin, MD cranial nerve cause vertical or oblique diplopia by impair- palsies include right hypertropia in left gaze, left hyper- Steven Galetta, MD ing the ability of the superior oblique muscle to intort tropia in right gaze, and alternating hypertropia with and depress the eye. This binocular diplopia worsens in head tilt to either side (i.e., right hypertropia with right downgaze and lateral gaze away from the affected eye. tilt and left hypertropia with left head tilt).8 Correspondence & reprint Because intorsion is necessary to maintain fusion in ocu- requests to Dr. Gold: [email protected] lar counter-roll, this diplopia also worsens with head tilt CASE REPORTS Case 1. A20-year-oldmanpresented 1,2 toward the affected eye. to the emergency department complaining of 6 days of Diagnosis of a superior oblique palsy can be made binocular vertical diplopia and a left eyelid droop. He using the Parks-Bielschowsky 3-step test: 1) determine had noted fatigue, bilateral eye pain, and flu-like symp- which eye is hypertropic, 2) determine if the hypertro- toms 2 to 4 weeks prior to presentation. pia worsens in left or right gaze, and 3) determine if the Left-sided ptosis and miosis were present on exam- hypertropia worsens in right or left head tilt. In a supe- ination, along with a left adduction deficit.
    [Show full text]
  • Monocular Vs Binocular Diplopia BRENDA BODEN, CO PARK NICOLLET PEDIATRIC and ADULT STRABISMUS CLINIC Monocular Diplopia
    Monocular vs Binocular Diplopia BRENDA BODEN, CO PARK NICOLLET PEDIATRIC AND ADULT STRABISMUS CLINIC Monocular Diplopia Patient sees double vision with ONE eye open Second image appears as an OVERLAP or GHOST image Monocular Diplopia How to test? Cover test: cover each eye and ask the patient if they see single or double Pinhole: monocular diplopia will likely resolve Monocular Diplopia Causes Refractive Cornea abnormalities High astigmatism Keratoconus Tear Film Insufficiency Lens abnormalities Early tear break up time Lens opacities Dry eye syndrome IOL decentrations where the edge of lens is within the visual axis Abnormalities in blink Change in refractive error (anisometropia) Retinal Pathology s/p ocular surgery Maculopathy due to fluid, hemorrhage, or fibrosis (epiretinal membranes are the most Refractive surgery can cause irregular symptomatic) astigmatism and ocular aberrations Polycoria after iridectomy Monocular Diplopia Additional Testing Refractive Macular Pathology Pinhole, optical aberrations can be caused from Fundus exam irregular astigmatism OCT Refract with retinoscopy or over hard contact Amsler Grid lens Let patient dial in astigmatism axis Cornea abnormalities Slit lamp exam Tear Film Insufficiency Corneal topography instruments Early tear film break up time or Schirmer test Use artificial tear to see if symptoms resolve Binocular Diplopia Patient sees double vision with BOTH eyes open A A Vertical and Horizontal Diplopia Vertical Diplopia Binocular Diplopia How to test? Covering
    [Show full text]