DOCSLIB.ORG

Pupil Sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: a Case Report and Literature Review, SPR, 2021, Volume 1, Issue, 4, Page No.: 216 – 219

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Pupil Sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: a Case Report and Literature Review, SPR, 2021, Volume 1, Issue, 4, Page No.: 216 – 219 Sunuwar N, Twayana AR, Panthi S, Koirala A, Gautam S, Pupil sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: A Case Report and literature review, SPR, 2021, Volume 1, issue, 4, Page No.: 216 – 219. DOI: https://doi.org/10.52152/spr/2021.136 ISSN: 2635-0955 Case Report DOI: https://doi.org/10.52152/spr/2021.136 Pupil sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: A Case Report and literature review Neela Sunuwar1,*, Anu Radha Twayana2, Sagar Panthi1, Aakash Koirala1, Swotantra Gautam1 1BP Koirala Institute of Health Sciences, Sunsari, Dharan, Nepal 2Kathmandu University School of Medical Sciences, Dhulikhel, Nepal *Corresponding Author: Dr. Neela Sunuwar, BP Koirala Institute of Health Sciences, Sunsari, Dharan, Nepal, Email: [email protected] Submission Date: 4/07/2021 Acceptance Date: 1/08/2021 Published Date: 6/08/2021 ABSTRACT Midbrain stroke especially Weber syndrome is a rare case seldom reported in the literature. It involves oculomotor nerve palsy and contralateral hemiparesis. A 46-year-old female presented with sudden onset of blurring of vision along with right-sided hemiparesis, right upper motor neuron type cranial nerve (CN) VII palsy, left-sided CN III palsy, and left-sided ptosis. Magnetic resonance imaging (MRI) revealed T2 flair/hyperintensity in the left side of the midbrain, bilateral gangliocapsular regions, and centrum semiovale indicating acute infarct and chronic ischemic changes respectively as well as a high signal area on the T2/FLAIR sequence, indicating sinusitis. Ocular manifestations of a midbrain stroke are highlighted in this case study, particularly Weber syndrome, which also entails contralateral hemiparesis. A better prognosis can be achieved with early diagnosis and treatment. Keywords: Hemiparesis, Midbrain infarct, Oculomotor palsy, Weber syndrome. INTRODUCTION Weber syndrome is a type of superior erior cerebral artery. Hemorrhage, aneurysms, alternating hemiplegia that affects the tumors, and demyelinating disorders are some oculomotor fascicles in the interpeduncular of the less prevalent causes. Weber syndrome, cisterns and cerebral peduncle, resulting in like other strokes, is frequently associated with ipsilateral third nerve palsy and contralateral a history of hypertension, diabetes, and hyper- hemiparesis. 1 It is characterized by a charact- cholesterolemia. 3 eristic appearance of the left-sided third nerve There are some noteworthy exceptions in palsy with right-sided hemiplegia produced by weber syndrome to the clinical dictum that an an abrupt bleed in the left cerebral peduncle, as ischemic lesion causes pupil sparing in described by Hermann Weber.2 oculomotor nerve palsy while pupil The most common etiology of Weber involvement predicts compressive lesion. 4 Syndrome is the obstruction of a branch of the However, in Weber’s Syndrome pupils may or peduncular perforating branches of the post- may not be spared depending upon the area of This article has been published under the terms of CC BY-NC 4.0, which permits noncommercial unrestricted use, distribution, and reproduction in any medium, provided that the following statement is provided. “This article has been published in Science Progress and 216 Research at DOI https://doi.org/10.52152/spr/2021.136 and can also be viewed on the Journal’s website at https://sprjonline.com/”. inciting insult; dilated unresponsive pupils present a case of pupil sparing oculomotor may occur if both upper and middle midbrain palsy with contralateral hemiparesis also are affected and pupils may be spared if only known as Weber syndrome which is a rare the lower midbrain is affected.1 Herein we phenomenon. Figure 1. Cranial magnetic resonance imaging axial T2-weighted image shows hyperintensity in the midbrain on the left side. Case report history included an eight-year history of type 2 A 46-year-old woman presented to the Diabetes Mellitus and hypertension both under emergency department with sudden onset of control with medications. The patient was blurring of vision and dizziness with spontaneous conscious and oriented. Her best-corrected visual resolution of both symptoms after an hour. She also acuity was 6/6 in both eyes. Ocular examination of reported headache, tingling sensation in the right the right eye was normal except for the limitation upper and lower limb, diplopia, and drooping of the of elevation in upgaze. The left eye showed left eyelid for the last two days. Her medical complete ptosis with down and out pupil with Citation: Sunuwar N, Twayana AR, Panthi S, Koirala A, Gautam S, Pupil sparing Cranial Nerve III Palsy and Hemiparesis, Weber Syndrome: A Case Report and literature review, SPR, 2021, Volume 1, issue, 4, Page No.: 217 216 – 219. DOI: https://doi.org/10.52152/spr/2021.136 limitation of adduction, elevation, and depression. muscles, except the lateral rectus and the superior Convergence was impaired. However, the pupillary oblique. The oculomotor nucleus' filaments cross size and accommodation were unaffected. Slit- through the red nucleus to emerge on the medial lamp biomicroscopy of the anterior segment and side of the crus cerebri. The third nerve fascicle is fundus was normal. Color vision was normal in relatively separate. As a result, a lesion affecting both eyes. However, the patient could not complete the lower midbrain affects the extraocular muscles the visual field testing and diplopia charting. only and spares the pupils, whereas lesions Neurological examination revealed right-sided affecting both the upper and lower regions of the hemiparesis with right upper motor neuron type midbrain cause pupillary dilation as well. This cranial nerve (CN) VII palsy and left-sided CN III explains the patient's pupillary sparing in Weber's palsy. Reflexes were brisk on the right side with condition which is apparent in our case. 6 extensor plantar response. Coordination was As evidenced in the case, the patient presented normal, and no other cranial nerve abnormalities with a long history of diabetes indicating were detected. Examination of pulmonary and demyelination of the third cranial nerve with cardiovascular systems was within normal limits. A ipsilateral ptosis. The patient also reported provisional diagnosis of pupil sparing left eye CN contralateral upper and lower limb tingling III nerve palsy secondary to long-term diabetes was sensation and decreased strength observed on made and a magnetic resonance imaging (MRI) clinical evaluation. Similar to these findings, scan was ordered to rule out brain stem infarction Silverman et al found the oculomotor palsies at the and meningoencephalitis. level of the midbrain are either nuclear or MRI of the brain revealed T2 /fluid-attenuated fascicular: nuclear involves bilateral ptosis and inversion recovery in the left side of the midbrain contralateral superior rectus involvement while showing restriction on diffusion suggestive of the fascicular involves ipsilateral signs and symptoms.2 acute infarct. T2 hyperintensity in bilateral Therefore, we could conclude that the presence of gangliocapsular region and centrum semi-ovale ipsilateral ptosis in our patient is suggestive of without restriction on diffusion indicating chronic fascicular involvement at the level of the midbrain. ischemic changes and high signal area on T2/ Oculomotor palsy occurs either due to infarction, FLAIR sequence at left maxillary sinus suggestive demyelination, and tumors due to the occlusion of of sinusitis were found (Figure 1). Hence, a branches of the posterior cerebral artery. diagnosis of Weber syndrome was made. In comparison to CT scan, MRI is a more Clinical examination showed a pupil involving sensitive technique for detecting intracranial left third nerve palsy with upgaze palsy together disease. 7 In our case report, a CT scan was found with right hemiparesis which favored the final to be normal and the diagnosis of Weber syndrome diagnosis of Weber syndrome. was made only after MRI confirming its sensitivity to detect intracranial lesions. Tamhankar et al in DISCUSSION their study recommended early neuro-imaging as a Weber syndrome is a relatively uncommon general guideline in all patients presenting with midbrain stroke syndrome. The patient often acute solitary ocular motor palsies, particularly presents with partial oculomotor nurve palsy with when the patient presents to a non-specialist who ipsilateral ptosis and contralateral hemiparesis may fail to obtain a comprehensive history and lack often linked with a long-term history of diabetes the competence of a neuro-ophthalmologist. and hypertension. Subsequently, in the case, MRI observations of Ruchalski et al concluded that understanding hyperintensity in the left side of the midbrain, the functions and interconnections between the suggestive of acute infarction on the chronically several midbrain areas helps for a more accurate ischemic area, corroborated a final diagnosis of linkage of disease involvement and patient Weber syndrome, which traditionally appears as a symptoms. 3 Similarly, we found a connection combination of oculomotor palsy and contralateral between the symptom of the patient and the areas hemiparesis. involved in the midbrain. The patient was Besides pupil sparing, another unique finding of exhibiting normal pupillary activity despite ptosis this case report lies in the recovery of the patient of the left eye and weakness on the contralateral even though most brainstem lesions have a bad right side of the body. It could be comprehended by prognosis. Prompt initiation
Load more

Recommended publications
  • Approach to a Case of Congenital Heart Disease
    BAI JERBAI WADIA HOSPITAL FOR CHILDREN PEDIATRIC CLINICS FOR POST GRADUATES PREFACE This book is a compilation of the discussions carried out at the course for post-graduates on ” Clinical Practical Pediatrics” at the Bai Jerbai Wadia Hospital for Children, Mumbai. It has been prepared by the teaching faculty of the course and will be a ready-reckoner for the exam-going participants. This manual covers the most commonly asked cases in Pediatric Practical examinations in our country and we hope that it will help the students in their practical examinations. An appropriately taken history, properly elicited clinical signs, logical diagnosis with the differential diagnosis and sound management principles definitely give the examiner the feeling that the candidate is fit to be a consultant of tomorrow. Wishing you all the very best for your forthcoming examinations. Dr.N.C.Joshi Dr.S.S.Prabhu Program Directors. FOREWARD I am very happy to say that the hospital has taken an initiative to organize this CME for the postgraduate students. The hospital is completing 75 years of its existence and has 2 done marvelous work in providing excellent sevices to the children belonging to the poor society of Mumbai and the country. The hospital gets cases referred from all over the country and I am proud to say that the referrals has stood the confidence imposed on the hospital and its faculty. We do get even the rarest of the rare cases which get diagnosed and treated. I am sure all of you will be immensely benefited by this programme. Wish you all the best in your examination and career.
    [Show full text]
  • Basilar Artery and Its Branches Called Pontine Arteries
    3 Farah Mohammad Ahmad Al-Tarefe Mohammad Al salem تذكر أ َّن : أولئك الذين بداخلهم شيء يفوق كل الظروف ، هم فقط من استطاعوا أ ّن يحققوا انجازاً رائعاً .... كن ذا همة Recommendation: Study this sheet after you finish the whole anatomy material . Dr.Alsalem started talking about the blood supply for brain and spinal cord which are mentioned in sheet#5 so that we didn't write them . 26:00-56:27/ Rec.Lab#3 Let start : Medulla oblengata : we will study the blood supply in two levels . A- Close medulla (central canal) : It is divided into four regions ; medial , anteromedial , posteriolateral and posterior region. Medially : anterior spinal artery. Anteromedial: vertebral artery posterolateral : posterior inferior cerebellar artery ( PICA). Posterior : posterior spinal artery which is a branch from PICA. B-Open medulla ( 4th ventricle ) : It is divided into four regions ; medial , anteromedial , posteriolateral region. Medially : anterior spinal artery. Anteromedial: vertebral artery posterolateral : posterior inferior cerebellar artery ( PICA). 1 | P a g e Lesions: 1- Medial medullary syndrome (Dejerine syndrome): It is caused by a lesion in anterior spinal artery which supplies the area close to the mid line. Symptoms: (keep your eyes on right pic). Contralateral hemiparesis= weakness: the pyramid will be affected . Contralateral loss of proprioception , fine touch and vibration (medial lemniscus). Deviation of the tongue to the ipsilateral side when it is protruded (hypoglossal root or nucleus injury). This syndrome is characterized by Alternating hemiplegia MRI from Open Medulla (notice the 4th ventricle) Note :The Alternating hemiplegia means ; 1- The upper and lower limbs are paralyzed in the contralateral side of lesion = upper motor neuron lesion .
    [Show full text]
  • Alternating Hemiplegia of Childhood Syndrome
    orphananesthesia Anaesthesia recommendations for Alternating Hemiplegia of Childhood syndrome Disease name: Alternating hemiplegia of childhood syndrome (AHC) ICD 10: G98 Synonyms: AHC syndrome (An ATP1A3-related neurologic disorder). AHC was named for its most striking and diagnostic motor symptom; however, the range of manifestations show it to be a CNS disorder affecting function broadly in various brain circuits, heart and the disease evolves with age. Disease summary: AHC is a very rare neurological disorder first described in 1971 which has received increasing interest recently [1]. It is characterized by hemiplegia of either side of the body, paroxysmal tonic or dystonic spells, oculomotor abnormalities and developmental delay.2-4 Onset occurs before 18 months of age. This condition is diagnosed based on the occurrence of the above combination of symptoms, is usually due to de novo pathogenic variant in ATP1A3 and has also been reported in a few families [2-3]. Onset and progression of neurological symptoms have been well characterized. While the course and severity of deficits can vary considerably, there appears to be progression over time, at least in some patients. The differential diagnosis of AHC includes familial hemiplegic migraine (FHM) syndromes (e.g. FHM1-CACNA1A; FHM2-ATP1A2), episodic ataxia type 6, glutamate transporter disorders (SLC1A3), glucose transporter defects, GLUT1 deficiency (SLC2A1), infantile onset epileptic encephalopathies, severe myoclonic epilepsy of infancy (Dravet syn- drome), SCN1A mutations, mitochondrial disorders, and disorders of dopamine biosynthesis/ neurotransmitter disorders. The prevalence has been estimated at 1:1,000,000 with most cases being due to de novo mutations [4-6]. Triggers in AHC and other ATP1A3 related diseases that can induce paroxysmal episodes in AHC are frequent.
    [Show full text]
  • Alternating Hemiplegia of Childhood: New Diagnostic Options
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Via Medica Journals n e u r o l o g i a i n e u r o c h i r u r g i a p o l s k a 4 8 ( 2 0 1 4 ) 1 3 0 – 1 3 5 Available online at www.sciencedirect.com ScienceDirect journal homepage: http://www.elsevier.com/locate/pjnns Review article Alternating hemiplegia of childhood: New diagnostic options Aleksandra Gergont *, Marek Kaciński Department of Neurology of Children and Youth, Jagiellonian University, Collegium Medicum, Krakow, Poland a r t i c l e i n f o a b s t r a c t Article history: A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in Received 14 August 2012 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides Accepted 13 May 2013 of the body. Age of onset is before 18 months and the common earliest manifestations are Available online 15 February 2014 dystonic or tonic attacks and nystagmus. Hemiplegic episodes last minutes to days and the frequency and duration tend to decrease with time. Motor and intellectual development is fi Keywords: affected, de cits may also develop later. Epileptic seizures occur in some patients. Neuroimaging of the brain usually reveals no abnormalities. The variability of individual clinical presentations Alternating hemiplegia fi Children and evolution of symptoms have made diagnosis dif cult. Therefore the problems of misdiag- ATP1A3 nosis could account for the low prevalence of this syndrome.
    [Show full text]
  • Crossed Brainstem Syndrome Revealing
    Beucler et al. BMC Neurology (2021) 21:204 https://doi.org/10.1186/s12883-021-02223-7 CASE REPORT Open Access Crossed brainstem syndrome revealing bleeding brainstem cavernous malformation: an illustrative case Nathan Beucler1,2* , Sébastien Boissonneau1,3, Aurélia Ruf4, Stéphane Fuentes1, Romain Carron3,5 and Henry Dufour1,6 Abstract Background: Since the nineteenth century, a great variety of crossed brainstem syndromes (CBS) have been described in the medical literature. A CBS typically combines ipsilateral cranial nerves deficits to contralateral long tracts involvement such as hemiparesis or hemianesthesia. Classical CBS seem in fact not to be so clear-cut entities with up to 20% of patients showing different or unnamed combinations of crossed symptoms. In terms of etiologies, acute brainstem infarction predominates but CBS secondary to hemorrhage, neoplasm, abscess, and demyelination have been described. The aim of this study was to assess the proportion of CBS caused by a bleeding episode arising from a brainstem cavernous malformation (BCM) reported in the literature. Case presentation: We present the case of a typical Foville syndrome in a 65-year-old man that was caused by a pontine BCM with extralesional bleeding. Following the first bleeding episode, a conservative management was decided but the patient had eventually to be operated on soon after the second bleeding event. Discussion: A literature review was conducted focusing on the five most common CBS (Benedikt, Weber, Foville, Millard-Gubler, Wallenberg) on Medline database from inception to 2020. According to the literature, hemorrhagic BCM account for approximately 7 % of CBS. Microsurgical excision may be indicated after the second bleeding episode but needs to be carefully weighted up against the risks of the surgical procedure and openly discussed with the patient.
    [Show full text]
  • Alternating Hemiplegia of Childhood in a Child Misdiagnosed As Intractable Epilepsy
    Published online: 2019-09-25 Letters to the Editor a rare complication of HSV‑1 meningoencephalitis: Case report This is an open access article distributed under the terms of the Creative Commons and review of the literature. Scand J Infect Dis 2006;38:63‑6. Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, 8. Shelley BP, Raniga SB, Al‑Khabouri J. An unusual and build upon the work non-commercially, as long as the author is credited and the new late complication of intracerebral haematoma in herpes creations are licensed under the identical terms. encephalitis after successful acyclovir treatment. J Neurol Sci 2007;252:177‑80. Access this article online 9. Li JZ, Sax PE. HSV‑1 encephalitis complicated by cerebral Quick Response Code: hemorrhage in an HIV‑positive person. AIDS Read Website: www.ruralneuropractice.com 2009;19:153‑5. 10. Takeuchi S, Takasato Y. Herpes simplex virus encephalitis complicated by intracerebral hematoma. Neurol India DOI: 2011;59:594‑6. 10.4103/0976-3147.196436 11. Rodríguez‑Sainz A, Escalza‑Cortina I, Guio‑Carrión L, Matute‑Nieves A, Gómez‑Beldarrain M, Carbayo‑Lozano G, et al. Intracerebral hematoma complicating herpes simplex encephalitis. Clin Neurol Neurosurg 2013;115:2041‑5. How to cite this article: Mahale RR, Mehta A, Shankar AK, Miryala A, 12. Yu W, Lee A, Welch B. Herpes simplex encephalitis presents as Acharya P, Srinivasa R. Bilateral cerebral hemorrhage in herpes simplex large temporal lobe haemorrhage. Neurol Cases 2014;1:12‑5. encephalitis: Rare occurrence. J Neurosci Rural Pract 2016;7:S128-30. Alternating Hemiplegia of Childhood in a Child Misdiagnosed as Intractable Epilepsy Sir, Although she had use two antiepileptic drugs (AEDs) for Neurofibromatosis type 1 (NF1) is an autosomal‑dominant seizures, her attacks continued.
    [Show full text]
  • A Dictionary of Neurological Signs
    FM.qxd 9/28/05 11:10 PM Page i A DICTIONARY OF NEUROLOGICAL SIGNS SECOND EDITION FM.qxd 9/28/05 11:10 PM Page iii A DICTIONARY OF NEUROLOGICAL SIGNS SECOND EDITION A.J. LARNER MA, MD, MRCP(UK), DHMSA Consultant Neurologist Walton Centre for Neurology and Neurosurgery, Liverpool Honorary Lecturer in Neuroscience, University of Liverpool Society of Apothecaries’ Honorary Lecturer in the History of Medicine, University of Liverpool Liverpool, U.K. FM.qxd 9/28/05 11:10 PM Page iv A.J. Larner, MA, MD, MRCP(UK), DHMSA Walton Centre for Neurology and Neurosurgery Liverpool, UK Library of Congress Control Number: 2005927413 ISBN-10: 0-387-26214-8 ISBN-13: 978-0387-26214-7 Printed on acid-free paper. © 2006, 2001 Springer Science+Business Media, Inc. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dis- similar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to propri- etary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omis- sions that may be made.
    [Show full text]
  • High-Yield Neuroanatomy, FOURTH EDITION
    LWBK110-3895G-FM[i-xviii].qxd 8/14/08 5:57 AM Page i Aptara Inc. High-Yield TM Neuroanatomy FOURTH EDITION LWBK110-3895G-FM[i-xviii].qxd 8/14/08 5:57 AM Page ii Aptara Inc. LWBK110-3895G-FM[i-xviii].qxd 8/14/08 5:57 AM Page iii Aptara Inc. High-Yield TM Neuroanatomy FOURTH EDITION James D. Fix, PhD Professor Emeritus of Anatomy Marshall University School of Medicine Huntington, West Virginia With Contributions by Jennifer K. Brueckner, PhD Associate Professor Assistant Dean for Student Affairs Department of Anatomy and Neurobiology University of Kentucky College of Medicine Lexington, Kentucky LWBK110-3895G-FM[i-xviii].qxd 8/14/08 5:57 AM Page iv Aptara Inc. Acquisitions Editor: Crystal Taylor Managing Editor: Kelley Squazzo Marketing Manager: Emilie Moyer Designer: Terry Mallon Compositor: Aptara Fourth Edition Copyright © 2009, 2005, 2000, 1995 Lippincott Williams & Wilkins, a Wolters Kluwer business. 351 West Camden Street 530 Walnut Street Baltimore, MD 21201 Philadelphia, PA 19106 Printed in the United States of America. All rights reserved. This book is protected by copyright. No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. To request permission, please contact Lippincott Williams & Wilkins at 530 Walnut Street, Philadelphia, PA 19106, via email at [email protected], or via website at http://www.lww.com (products and services).
    [Show full text]
  • C1 PAGE.Indd
    Neurology.org/N Child Neurology: A Case-Based Approach Cases from the Neurology® Resident & Fellow Section Child Neurology: A Case-Based Approach Cases from the Neurology® Resident & Fellow Section Editors John J. Millichap, MD Att ending Epileptologist Ann & Robert H. Lurie Children’s Hospital of Chicago Associate Professor of Pediatrics and Neurology Northwestern University Feinberg School of Medicine Chicago, IL Jonathan W. Mink, MD, PhD Frederick A. Horner, MD Endowed Professor in Pediatric Neurology Professor of Neurology, Neuroscience, and Pediatrics Chief, Division of Child Neurology Vice Chair, Department of Neurology University of Rochester Medical Center Rochester, NY Phillip L. Pearl, MD Director of Epilepsy and Clinical Neurophysiology William G. Lennox Chair, Boston Children’s Hospital Professor of Neurology Harvard Medical School Boston, MA Roy E. Strowd III, MEd, MD Assistant Professor Neurology and Oncology Wake Forest School of Medicine Winston Salem, NC © 2019 American Academy of Neurology. All rights reserved. All articles have been published in Neurology®. Opinions expressed by the authors are not necessarily those of the American Academy of Neurology, its affi liates, or of the Publisher. Th e American Academy of Neurology, its affi liates, and the Publisher disclaim any liability to any party for the accuracy, completeness, effi cacy, or availability of the material contained in this publication (including drug dosages) or for any damages arising out of the use or non-use of any of the material contained in this publication. TABLE OF CONTENTS Neurology.org/N Section 2. Pediatric stroke and cerebrovascular disorders 25 Introduction Robert Hurford, Laura L. Lehman, Behnam Sabayan, and Mitchell S.V.
    [Show full text]
  • Assessment of Acute Motor Deficit in the Pediatric Emergency Room
    J Pediatr (Rio J). 2017;93(s1):26---35 www.jped.com.br REVIEW ARTICLE Assessment of acute motor deficit in the pediatric ଝ emergency room a,∗ b a Marcio Moacyr Vasconcelos , Luciana G.A. Vasconcelos , Adriana Rocha Brito a Universidade Federal Fluminense (UFF), Hospital Universitário Antônio Pedro, Departamento Materno Infantil, Niterói, RJ, Brazil b Associac¸ão Brasileira Beneficente de Reabilitac¸ão (ABBR), Divisão de Pediatria, Rio de Janeiro, RJ, Brazil Received 21 May 2017; accepted 28 May 2017 Available online 27 July 2017 KEYWORDS Abstract Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic Acute weakness; investigation, to children and adolescents who present in the emergency room with acute-onset Motor deficit; Guillain---Barré muscle weakness. syndrome; Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: ‘‘acute,’’ ‘‘weakness,’’ ‘‘motor Transverse myelitis; Child deficit,’’ ‘‘flaccid paralysis,’’ ‘‘child,’’ ‘‘pediatric,’’ and ‘‘emergency’’. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is a fairly common presentation in the pedi- atric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain---Barré syndrome and transverse myeli- tis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness.
    [Show full text]
  • 25. C:\Documents and Settings\Kwang-Il\My
    The human disease network Goh K-I, Cusick ME, Valle D, Childs B, Vidal M, Barabasi′ A-L (2007) Proc Natl Acad Sci USA 104:8685-8690 Disorder Class Bone Coats Cancer Urolithiasise Osteopetrosis disease NDP Caffey van_Buchem Exudative Cardiovascular disease disease vitreoretinopathy Norrie SLC34A1 disease 439 LRP5 Connective tissue disorder Nevo Hyperostosis, syndrome COL1A1 endosteal Dermatological PLOD1 217 PAX9 Oligodontia Osteogenesis Osteoporosis 1164 Developmental Ehlers-Danlos imperfecta syndrome Arthropathy COL3A1 Hypodontia Ear, Nose, Throat Aneurysm, COL1A2 familial_arterial Myasthenic Witkop 733 syndrome Heart syndrome Pseudoachondroplasia Endocrine 3-methylglutaconicaciduria OPA3 WISP3 Optic Marfan block MSX1 atrophy OPA1 Aortic syndrome Paramyotonia Sick_sinus Gastrointestinal aneurysm congenita syndrome 3558 Intervertebral_disc Brugada SCN4A disease syndrome Syndactyly Spondyloepiphyseal COMP COL9A2 Hematological Glaucoma Weill-Marchesani Shprintzen-Goldberg Cramps, SCN5A Zlotogora-Ogur Cleft dysplasia syndrome syndrome potassium-aggravated Myotonia 2785 syndrome palate Parkes_Weber Basal_cell FBN1 congenita Oculodentodigital COL9A3 1432 Immunological 1414 CYP1B1 syndrome nevus_syndrome MASS Hypokalemic Acquired dysplasia Peters long_QT_syndrome Epiphyseal FLNB RASA1 PTCH Keratitis syndrome periodic MATN3 Metabolic SHH anomaly Eye Ectopia Thyrotoxic paralysis dysplasia Atelosteogenesis anomalies Marshall Larson Capillary Basal_cell Holoprosencephaly Coloboma, periodic KCNH2 PVRL1 malformations GJA1 Incontinentia syndrome SLC26A2
    [Show full text]
  • Child Neurology: Benign Nocturnal Alternating Hemiplegia of Childhood Laetitia J.J.C
    RESIDENT & FELLOW SECTION Child Neurology: Section Editor Benign nocturnal alternating hemiplegia Mitchell S.V. Elkind, MD, MS of childhood Laetitia J.J.C. Wagener- Alternating hemiplegia of childhood (AHC) is a dis- parents and told them about the benign and self- Schimmel, MD order of recurrent hemiplegia beginning before age limiting course of this disorder. No specific genetic Joost Nicolai, MD, PhD 18 months and is associated with dystonia, nystagmus, analysis was performed. There has been 1 more at- and progressive cognitive and motor impairment. This tack, making a total of 4 episodes. Motor develop- disorder was first recognized by Verret and Steele1 in ment at the age of 5 years is normal, and he is visiting Correspondence & reprint 1971. Benign nocturnal alternating hemiplegia of child- the regular primary school although there is concern requests to Dr. Nicolai: [email protected] hood, which differs from AHC by its absence of decline about some possible autistic features. Psychiatric in neurologic or cognitive impairment, is very rare or evaluation will start in the next months. possibly only rarely recognized. We describe 2 children The second boy visited our outpatient depart- diagnosed with this syndrome and review the literature ment for the first time at age 20 months. He is the published. In contrast to the severe prognosis of the first son of nonrelated parents and has normal psy- much more known syndrome of AHC,1,2 the prognosis chomotor development. His parents have noted peri- of benign nocturnal alternating hemiplegia of child- ods in which he wakes up crying with a left-sided hood is excellent.
    [Show full text]