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 Judit Somlai
Tibor Kovács
Editors

Neuro-Ophthalmology

123
Neuro-Ophthalmology

• more recent diagnostics and therapies

• modern etiopathomechanisms
• current rehabilitation of people with impaired vision
Judit Somlai • Tibor Kovács
Editors

Neuro-Ophthalmology
Editors
Judit Somlai Tibor Kovács
Unit of Neuro-Ophthalmology Department of Neurology
Department of Neurology and Stroke Semmelweis University
Military Hospital Budapest
Budapest Hungary
Hungary

ISBN 978-3-319-28954-0 ISBN 978-3-319-28956-4 (eBook)

DOI 10.1007/978-3-319-28956-4

Library of Congress Control Number: 2016939129

© Springer International Publishing Switzerland 2016

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software,
or by similar or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a specific statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in
this book are believed to be true and accurate at the date of publication. Neither the publisher nor
the authors or the editors give a warranty, express or implied, with respect to the material
contained herein or for any errors or omissions that may have been made.

Printed on acid-free paper

This Springer imprint is published by Springer Nature

The registered company is Springer International Publishing AG Switzerland
Dedicated to:
László Remenár, M.D.†
(National Neurosurgical Scientific Institute,
Budapest, Hungary)

Márta Hajda, M.D.

(National Neurosurgical Scientific Institute,
Budapest, Hungary)

Prof. Yochanan Goldhammer †

(Tel Hashomer Medical Center, Tel Aviv, Israel)

Prof. Josef Flammer

(University Eye Clinic, Basel, Switzerland)
Preface

Medical specialties are becoming more and more complex. The knowledge of
experts covers more and more circumscripts. Together with several other fac-
tors, this phenomenon enhances the importance of teamwork. Our book also
aims to meet this need. Neuro-ophthalmology falls in a niche between the
two specialties combining their knowledge concerning the sense of sight.
Vision is the most important system which links us to the outside world. Its
complexity in itself requires the expertise of several specialties, not to men-
tion the visual symptoms of numerous diseases of not neurological origin.
Judit Somlai, who has become a household name in Hungarian neuro-
ophthalmology, managed to win over almost 50 experts to cooperate in the
explanation and education of this field.
This book has some antecedents, as since its first publication (1996) it has
already been revised twice. Both revisions (2007 and 2010) incorporated sub-
stantially updated and supplemented content, written by an increasing num-
ber of authors. You can get an idea of the dynamic development of the field
just by considering the shortened time between two revisions and the expan-
sion of the book; the present publication has twice as many pages as the pre-
vious one.
Similarly to the previous book, the rehabilitation of chronic visual impair-
ment and the available aids get extensive coverage.
This book is also an excellent example of integration and work based on a
complex conceptual base, which is frequently lacking in today’s health care.
I would like to congratulate the authors of this book for providing us with
this consistent and trustworthy knowledge, and I would also like to recom-
mend it to experts working in the field of ophthalmology, neurology, neuro-
surgery, traumatology, radiology, as well as cardiovascular medicine and
rehabilitation.

Péter Halász, M.D.

vii
Contents

Part I The Importance and Role of Neuro-Ophthalmology

1 The Importance and Role of Neuro-ophthalmology

in Ophthalmological Clinical Practice . . . . . . . . . . . . . . . . . . . . . . 3
Judit Somlai
2 The Importance of Neuro-ophthalmology in Neurology . . . . . . . . 5
Tibor Kovács

Part II Objectives and Recent Results in the

Neuro-Ophthalmological Clinical Practice

3 Mechanisms of Parallel Information Processing

in the Visual System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
György Benedek
4 A New Direction: Neuro-endocrino-immunology . . . . . . . . . . . . 15
Csaba Balázs
5 Visual and Oculomotor Disorders in Internal Diseases. . . . . . . . 19
György Pfliegler
6 Genetic Aspects of Neuro-ophtalmological Diseases . . . . . . . . . . 27
Mária Judit Molnár
7 Recent Knowledge in the Neurosurgical Practice
Regarding the Visual System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
János Vajda
8 The Role of Gamma Knife Stereotactic Radiosurgery
in the Treatment of Neuro-ophthalmological Diseases . . . . . . . . 37
Gyôrgy T. Szeifert and Jenô Szeifert
9 Neurointerventional Treatment of Diseases Causing
Neuro-ophthalmological Symptoms . . . . . . . . . . . . . . . . . . . . . . . 47
Istvan Szikora
10 Recent Results in Neuropathology: Demyelinating
and Conformational Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Tibor Kovács

ix
x Contents

Part III Conventional, Novel and Complementary Examinations

in Ophthalmology

Functional Tests of the Visual Pathway System

11 Algorithm of the Neuro-ophthalmological
Examination Used in the International Practice . . . . . . . . . . . . . 67
Judit Somlai
12 Objective and Subjective Examination Methods
of Visual Acuity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
Márta Janáky
13 Examination of Contrast Sensitivity . . . . . . . . . . . . . . . . . . . . . . . 83
Márta Janáky
14 Examination of Color Vision . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Klára Aczél
15 Electroretinography (ERG): Electrophysiological
Examination of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97
Ägnes Farkas
16 Functional Examinations of the Visual Pathway
System with Electrophysiological Methods . . . . . . . . . . . . . . . . 111
Márta Janáky
17 Clinical Importance of Conventional and Modern Visual
Field Tests in the Topographical Diagnostics of Visual
Pathway Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119
Judit Somlai
18 The Differential Diagnosis of Visual Field Deficits
at the Bedside. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133
Gyula Gács and Ildikó Szilvássy
19 The Role of Fluorescein Angiography and Optical
Coherence Tomography in the Examination
of Circulatory Disorders of the Optic Disc . . . . . . . . . . . . . . . . . 145
Zsuzsa Récsán and Zsuzsa Szepessy
20 Optical Coherence Tomography of the Optic Disc
and the Macula in Neurodegenerative Diseases . . . . . . . . . . . . . 157
Gábor Márk Somfai, Erika Tátrai, and Magdolna Simó
Neuro-Ophthalmological Examinations of the Eye Movements
21 Diagnosis, Differential Diagnosis and Treatment
of Congenital Ocular Motor Disorders . . . . . . . . . . . . . . . . . . . . 171
Anna Soproni and Patrícia Domsa
22 Polatest Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197
Judit Pálfia and Ernő Pálfia
Contents xi

23 Physiology and Examination Methods

of the Pupillomotor Pathway . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207
Bernadett Salomváry
24 Neuro-ophthalmological Methods for the Clinical
Analysis of Double Vision . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217
Judit Somlai
Supplementary Test Procedures
25 Duplex Ultrasound Examination of the Carotid
and Vertebral Arteries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223
Péter Barsi
26 Transcranial Doppler Examination. . . . . . . . . . . . . . . . . . . . . . . 231
Gyula Pánczél
27 Color Doppler Ultrasound Examination in Orbital
Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 247
János Németh, Zoltan Harkányi, and Zita Morvay
28 The Role of the Ophthalmologic Ultrasound
in Neuro-ophthalmology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253
Béla Csákány and János Németh
29 The Role of the EMG-ENG in Diagnosing
Neuro-ophthalmologic Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . 257
Mária Judit Molnár
30 Computed Tomography Examinations . . . . . . . . . . . . . . . . . . . . 263
József Kenéz
31 Neuroradiology, Functional MRI . . . . . . . . . . . . . . . . . . . . . . . . 271
György Várallyay and Lajos Rudolf Kozák
32 Novel Information Regarding the Visual
and Eye Movement Systems in Otoneurology . . . . . . . . . . . . . . 283
Ágnes Szirmai
33 Electro-Oculography (EOG) Examination
of Eye Movements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287
Szilvia Gulyás
34 The Importance of Familiar Thrombophilias in the Clinical
Practice. Novel Ways in Anticoagulant Therapy . . . . . . . . . . . . 295
László Nemes
35 Novel Consideration Regarding the Role of Evoked
Potential in Confirming the Diagnosis of Eye Movement
Disorders of Brainstem Origin . . . . . . . . . . . . . . . . . . . . . . . . . . 305
Szabolcs Tóth
xii Contents

Part IV Diseases of Retina and the Optic Nerve (Visual and Sensory
System)

Congenital Diseases of the Retina and the Visual Pathway

36 Hereditary Diseases of the Retina . . . . . . . . . . . . . . . . . . . . . . . . 309
Ágnes Farkas
37 The Roles of Electroretinography (ERG) and Visual
Evoked Potential (VEP) Examinations in the Diseases
of the Retina and/or the Optic Nerve . . . . . . . . . . . . . . . . . . . . . 325
Márta Janáky
38 Congenital Diseases of the Optic Nerve . . . . . . . . . . . . . . . . . . . 333
Márta Janáky
39 Phacomatoses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339
Judit Somlai
Acquired Diseases of the Optic Nerve
Inflammatory Diseases
40 Retrobulbar Optic Neuropathy:
From the Neurologist’s Approach . . . . . . . . . . . . . . . . . . . . . . . . 345
Gábor Lovas
41 Neuromyelitis Optica (Devic’s Disease):
A New Concept for an Old Disease . . . . . . . . . . . . . . . . . . . . . . . 351
Zsolt Illes
42 Acquired Inflammatory Diseases of the Optic Nerve:
From the Neuro-Ophthalmologist’s Approach . . . . . . . . . . . . . 361
Judit Somlai
Circulatory Disorders
43 Vascular Diseases of the Optic Nerve: Internal
Medicine Aspects. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
György Pfliegler
44 The Cardiovascular Background of ‘Intracerebral
Small Vessel Disease’. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377
Éva Nieszner
45 Vascular Diseases of the Optic Nerve:
The Neuro-Ophthalmologist’s Approach . . . . . . . . . . . . . . . . . . 383
Judit Somlai
Compressive Optic Neuropathy
46 Diseases Causing Compression of the Optic Nerve:
The Neurosurgeon’s Perspective . . . . . . . . . . . . . . . . . . . . . . . . . 401
János Vajda
47 Neuro-Ophthalmological Aspects of Tumors Causing
Compression of the Visual Pathway System . . . . . . . . . . . . . . . . 407
Bernadett Salomváry
Contents xiii

Traumatic Optic Neuropathy

48 The Significance of Neuro-ophthalmology
in the Diagnosis and Therapy of Cranial Trauma . . . . . . . . . . . 421
György T. Szeifert and Judit Somlai
Toxic and Deficiency Optic Neuropathy
49 Toxic and Deficiency Optic Neuropathy . . . . . . . . . . . . . . . . . . . 433
Vera Klein
Eye Related Symptoms and Signs of Intracranial Hypertension
50 Ocular Symptoms and Signs of Intracranial
Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437
Judit Somlai
51 Big Blind Spot Syndrome (Papillophlebitis) . . . . . . . . . . . . . . . . 453
Gyula Gács and Ildikó Szilvássy

Part V Neuro-Ophthalmologic Aspects of the Ocular Motor System

Disorders of the Pupillomotor Pathway

52 The Most Important Disorders of the Pupillomotor
Pathway in the Clinical Practice . . . . . . . . . . . . . . . . . . . . . . . . . 463
Judit Somlai
Infranuclear and Nuclear Neurogenic Paresis
53 Congenital Eye Movement Disorders . . . . . . . . . . . . . . . . . . . . . 471
Andrea Deák
54 The Most Important Clinical Syndromes of Acquired
Nuclear and Infranuclear Eye Movement Disorders,
and Their Diagnostic and Therapeutic Options. . . . . . . . . . . . . 483
Judit Somlai
Neurogenic Paresis Due Too Dysfunction of the Brainstem
55 Eye Movement Disorders Related to Brainstem
Dysfunctions -Types, Clinical Significance of Vertical
Localization, Modern Therapeutic Principles . . . . . . . . . . . . . . 493
Judit Somlai
56 The Clinical Significance of Otoneurology
in the Diagnosis of Brainstem Disorders. . . . . . . . . . . . . . . . . . . 507
Ágnes Szirmai
57 Examination of the Eye Movements of the Patient
in Coma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513
György Geréby
xiv Contents

Supranuclear Eye Movement Systems and Their Clinical Significance

58 Supranuclear Regulation of the Eye Movements
and the Significance of Their Disturbances . . . . . . . . . . . . . . . . 523
Szilvia Gulyás
Disorders of the Neuromuscular Junction (MG and OMG),
Non-isoéated Ocular Muscle Paresis and Myogenic Paresis
59 Disorders of the Neuromuscular Junction
and Their Diagnostics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 535
László Sándor Fornádi
60 The Ocular Characteristics and Differential
Diagnostics of Mixed Types Eye Movement
Disorders (Disorders of Ocular Neuromuscular
Junction (OMG), Non Isolated Ocular Muscle Paresis
and Myogenic Paresis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 545
Judit Somlai
61 Endocrine Myopathy and Orbitopathy. . . . . . . . . . . . . . . . . . . . 561
Csaba Balázs

Part VI Diseases of the Orbit

62 Diagnostics and Therapy of Diseases of the Orbit . . . . . . . . . . . 573

Katalin Korányi
63 Traumatic Injuries of the Orbit . . . . . . . . . . . . . . . . . . . . . . . . . . 593
György Pulay

Part VII Neuro-Ophthalmological Considerations

of the Facial Nerve

64 Tumor Lesions of the Facial Nerve . . . . . . . . . . . . . . . . . . . . . . . 637

Ildikó Gádor

Part VIII The Neuro-Ophthalmological Aspects of Headaches

65 Neuro-Opthalmological Aspects of Headaches

from the Neurologist’s Aspect . . . . . . . . . . . . . . . . . . . . . . . . . . . 647
Csaba Ertsey

Part IX Rehabilitation

66 Viewing Down from the Top: Visual Impairments

Developing as a Consequence of Cortical Injury . . . . . . . . . . . . 663
Anna Verseghi and Zita Snagy Nagy
67 Ignored World Without Missing It Neglect . . . . . . . . . . . . . . . . 677
Anna Verseghi and Zita Snagy Nagy
Contents xv

68 Introducing Tools and Services Helping

Life of People with Impaired Vision . . . . . . . . . . . . . . . . . . . . . . 689
Mihaly Szuhaj and Peter Szatmari
69 Elementary and Occupational Rehabilitation
of People with Impaired Vision . . . . . . . . . . . . . . . . . . . . . . . . . . 705
Marta Tolnayne Csattos and Laszlo Joszt
70 The Importance of Rehabilitation and the Options
of a Neuro-ophthalmologist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 715
Judit Somlai

Appendix: Colour Scheme of the Book Content . . . . . . . . . . . . . . . . 719

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 721
Contributors

Klára Aczél, M.D. Outpatient Department of Ophthalmology,

Military Hospital, Budapest, Hungary
Csaba Balázs, M.D. Department of Internal Medicine–Endocrinology,
Budai Hospital of Hospitaller Brothers, Budapest, Hungary
Péter Barsi, M.D., PhD, Habil Szentâgothai Knowledge Center – MR
Research Center, Semmelweis University, Budapest, Hungary
Unit of Neuroradiology, Department of Neurosurgery, University of Pécs,
Pécs, Hungary
György Benedek, M.D., PhD Faculty of Medicine, Institute of Physiology,
Albert Szent-Gyòrgyi Clinical Center, University of Szeged, Szeged,
Hungary
Béla Csákány, M.D., PhD Department of Ophthalmology,
Semmelweis University, Budapest, Hungary
Márta Tolnay Csattos, M.D. Head of National Institution for Blind People
Tiflopedagogist, Rehabilitation Instructor, National Institution for Blind
People, Budapest, Hungary
Andrea Deák, M.D. Orthoptic Unit, Department of Ophthalmology,
Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged,
Hungary
Patricia Domsa, M.D. Ophthalmologist, Pediatric Ophthalmologist,
Madarâsz Street Pediatric Hospital, Budapest, Hungary
Department of Ophthalmology, Semmelweis University, Budapest, Hungary
Rubrica Health Center, Budapest, Hungary
Csaba Ertsey, M.D., PhD Department of Neurology,
Semmelweis University, Budapest, Hungary
Agnes Farkas, M.D., PhD Department of Ophthalmology,
Semmelweis University, Budapest, Hungary
László Fornádi, M.D. Department of Neurology, Jahn Ferenc South
Pest Hospital, Budapest, Hungary

xvii
xviii Contributors

Gyula Gács, M.D., PhD Outpatient Department, Budapest City Council

Péterfy Sândor Street Hospital, Budapest, Hungary
Ildikó Gádor, M.D. Unit of Otoneurology, Department of
Otorhinolaryngology, National Institute of Neurology, Budapest, Hungary
György Geréby, M.D. Department of Neurology and Stroke Center,
Budapest City Council Saint John Hospital and United Hospitals of North
Buda, Budapest, Hungary
Szilvia Gulyás, M.D., PhD Electrophysiology Unit, Department
of Neurology, Faculty of Medicine, Semmelweis University, Budapest,
Hungary
Zoltan Harkányi, M.D., PhD Department of Radiology, CT Laboratory,
Heim Pál Pediatric Hospital, Budapest, Hungary
Zsolt Illés, M.D. Department of Neurology, University of Pécs, Pécs,
Hungary
Márta Janáky, M.D., PhD, habil. Electrophysiological Unit,
Department of Ophthalmology, Faculty of Medicine, University of Szeged,
Szeged, Hungary
László Joszt Social Worker, Rehabilitation Instructor of Visually Impaired
Patients Physical Accessibility Expert, National Institution for Blind People,
Budapest, Hungary
József Kenéz, M.D., PhD MR Laboratory, Székesfehérvár,
County Hospital, Székesfehérvár, Hungary
Department of Radiology, Semmelweis University, Budapest, Hungary
Department of Radiology, National Traumatology Institute,
Budapest, Hungary
Department of Neurology, Semmelweis University, Budapest, Hungary
Department of Neuroradiology, Haynal Imre Postgraduate University
of Health Sciences, Budapest, Hungary
Vera Klein, M.D. Ophthalmologist, Neuro-ophthalmologist, Health Care
Nonprofit Ltd., Budapest, Hungary
Katalin Korányi, M.D. Neuro-Ophthalmology Section,
Hungarian Ophthalmological Association, National Institute of Oncology,
Budapest, Hungary
Tibor Kovács, M.D., PhD, Habil. Department of Neurology,
Faculty of Medicine, Semmelweis University, Budapest, Hungary
Cerebrovascular and Dementia Unit, Department of General Neurology,
Budapest, Hungary
Dementia and Dystonia Outpatient Department, Semmelweis University,
Budapest, Hungary
Contributors xix

Lajos Rudolf Kozák, M.D., PhD Bolyai Research Fellow, MR Research

Center, Semmelweis University, Budapest, Hungary
Gábor Lovas, M.D., PhD Department of Neurology,
Délpesti Jahn Ferenc Hospital, Budapest, Hungary
Mária Judit Molnár, M.D., PhD, med. habil Department of Molecular
Neurology and Research Center, Center for Rare Neurological Diseases,
Semmelweis University, Budapest, Hungary
Zita Morvay, M.D., PhD Diagnoscan, Szeged, Hungary
Zita Snagy Nagy Department for Multiprofile Rehabilitation of Locomotor
Diseases, National Medical Rehabilitation Institute, Budapest, Hungary
Institute of Psychology, Eötvös Lóránd University, Budapest, Hungary
László Nemes, M.D., PhD National Hemophilia Center and Hemostasis
Outpatient Unit, Military Hospital, Budapest, Hungary
János Németh, M.D. Department of Ophthalmology, Faculty of Medicine,
Semmelweis University, Budapest, Hungary
Éva Nieszner, M.D., PhD Unit Head, Head Physician Internist,
Cardiologist, Diabetology Specialist, Angiology Specialist, Military
Hospital, Budapest, Hungary
Ernő Pálfia, M.D. Ophthalmologist, Pediatric Ophthalmologist, Budapest,
Hungary
Judit Pálfia, M.D. Ophthalmologist, Pediatric Ophthalmologist,
Pálfia Ltd., Budapest, Hungary
Gyula Pánczél, M.D., PhD Department of Neurology, Budapest City
Council Péterfy Sándor Street Hospital and Outpatient Department,
Budapest, Hungary
György Pfliegler, M.D., med.habil Department of Rare Diseases, 2nd
Department of Internal Medicine, Institute for Internal Medicine, Medical
and Health Center, University of Debrecen, Debrecen, Hungary
György Pulay, M.D. Outpatient Department, Accident Center, Budapest
City Council Péterfy Sándor Street Hospital, Budapest, Hungary
Zsuzsa Récsán, M.D., PhD Department of Ophthalmology, Faculty of
Medicine, Semmelweis University, Budapest, Hungary
Bernadett Salomváry, M.D. Head of Department, Head Physician
Ophthalmologist, Neuro-ophthalmologist, Orbital Surgeon, National
Institute of Neurology, Budapest, Hungary
Neuro-Ophthalmology Section, Hungarian Ophthalmological Association,
Budapest, Hungary
Magdolna Simó, M.D., PhD Department of Neurology,
Faculty of Medicine, Semmelweis University, Budapest, Hungary
xx Contributors

Gábor Márk Somfai, M.D., PhD Department of Ophthalmology,

Faculty of Medicine, Semmelweis University, Budapest, Hungary
Judit Somlai, M.D. Head of Unit of Neuro-Ophthalmology, Department
of Neurology and Stroke, Military Hospital, Budapest, Hungary
Unit of Neuro-Ophthalmology, Hungarian Ophthalmological Association,
Budapest, Hungary
Trustee, NOSZA Foundation (for the Prevention and Screening
of Ocular Storke), Budapest, Hungary
Anna Soproni, M.D. Ophthalmologist, Pediatric Ophthalmologist, Private
Medical Office, Budapest, Hungary
Péter Szatmári “Informatics for People with Visual Injury” Foundation,
Budapest, Hungary
György T. Szeifert, M.D., med. habil Unit for Neurosurgical Injuries,
Péterfy Accident Center, Semmelweis University, Budapest, Hungary
Jenő Szeifert, M.D. Faculty of Education and Psychology,
Institute of Health Promotion and Sport Sciences, Eötvös
Lóránd University, Budapest, Hungary
Zsuzsa Szepessy, M.D., PhD Department of Ophthalmology,
Faculty of Medicine, Semmelweis University, Budapest, Hungary
István Szikora, M.D., PhD Neurointerventional Unit, National Institute
of Neurology, Budapest, Hungary
Ildikó Szilvássy Districts 2 and 14 Outpatient Department,
Budapest, Hungary
Ágnes Szirmai, M.D., PhD Department of Otorhinolaryngology,
Head and Neck Surgery, Faculty of Medicine, Semmelweis University,
Budapest, Hungary
Mihály Szuhaj “Informatics for People with Visual Injury” Foundation,
Budapest, Hungary
Erika Tátrai, M.D., PhD student Department of Ophthalmology,
Faculty of Medicine, Electrophysiologist, Budapest, Hungary
Szabolcs Tóth, M.D. University Professor, DMSC Neurosurgeon,
Electrophysiologist, Budapest, Hungary
János Vajda, M.D., PhD Head Physician Neurosurgeon, National Institute
of Neurology, Budapest, Hungary
György Várallyay, M.D. Head Physician Radiologist, Neuroradiologist,
Szentagothai Knowledge Center – MR Research Center, Semmelweis
University, Budapest, Hungary
Anna Verseghi, M.D. Rehabilitation Unit for Patients with Skull
and Brain Injury, National Medical Rehabilitation Institute,
Budapest, Hungary
 The eternal dilemma concerning which is harder:
to imagine a world one has never seen
or
to lose a world that has once been familiar with
and
to accept that it will never return?
We must never decide, just let empathy guide us.

Hippocates graphic illustration was created by †János Kass (1927–2010), Hungarian

Graphic Sculptor
 Part I
The Importance and Role of
Neuro-Ophthalmology

1. The Importance and Role of Neuro-ophthalmology in Ophthalmological

Clinical Practice
2. The Importance of Neuro-ophthalmology in Neurology
 The Importance and Role
of Neuro-ophthalmology 1
in Ophthalmological Clinical
Practice

Judit Somlai

Neuro-ophthalmology (NO) is a new of neuro-ophthalmological assessment. Our

diagnostic sub-specialty on the periphery empirical knowledge will probably inform us
between neurology and ophthalmology that of the etiology of the disorder as well. The
encompasses the ophthalmological symp- tests will give us information about:
toms of internal diseases with neurological
or neurosurgical complications. The visual • the localization
pathways (between the retina and the visual • the extent of the damage
cortex) and the oculomotor system (between • disorders that cause only loss of function at
the eye muscles and the cortical centers) have the onset without morphological changes
direct contact with almost the whole length detectable on CT or MRI scans
of the central nervous system. Therefore, if • the efficiency of the treatment and the prog-
we note any disorder in these functions, we ress of the disease
can draw conclusions regarding the extent • the qualitative and objective assessment of
and the location of the damage with the help care and rehabilitation.

J. Somlai, M.D.
Head of Unit of Neuro-Ophthalmology,
Department of Neurology and Stroke,
Military Hospital, Budapest, Hungary
Unit of Neuro-Ophthalmology, Hungarian
Ophthalmological Association, Budapest, Hungary
Trustee, NOSZA Foundation (for the Prevention
and Screening of Ocular Stroke), Budapest, Hungary
e-mail: [email protected]; http://somlaijudit.hu

© Springer International Publishing Switzerland 2016 3

J. Somlai, T. Kovács (eds.), Neuro-Ophthalmology, DOI 10.1007/978-3-319-28956-4_1
4 J. Somlai

Drawing from the era of Islamic medicine. Their ideas of the visual pathways were not far from reality, especially
regarding the chiasmatic region

Topography, a.k.a. ‘localization diagnostics’ progression of the disease. If necessary,

can help us to identify the location and extent of patients should be followed up for life by
pathological changes anywhere in the visual experts from the affected and related fields on
and oculocomotor pathways, e.g., in the case regular consultations.
of loss of visual field or paralytic strabismus. Similar to almost all fields of clinical medi-
Examinations enable us to quantify the extent of cine, cutting-edge diagnostic techniques are at
the abnormality, but they can also help us to the forefront of neuro-ophthalmology.
exclude pathological processes. This feature can be explained by the fact that
Disease processes that result in loss of the earlier a disease is diagnosed, the more effi-
vision can evade even the most modern diag- cient the appropriate therapy and the lower the
nostic tools for a relatively long period of time. chance of residual symptoms will be.
On the other hand, some functional disorders, Medical treatment is complemented by com-
such as transient loss of vision and/or diplopia prehensive rehabilitation performed by our assis-
can indicate or herald an imminent or actual tants with special qualifications. This way we can
disease process. The results of conservative not only alleviate patients’ symptoms, but we can
and surgical treatments can be evaluated using also assist their recovery and resumption of regu-
neuro-ophthalmological tests, e.g., an increased lar activities with the help of modern diagnostic
visual field defect can indicate relapse or techniques and useful advice.
 The Importance of Neuro-
ophthalmology in Neurology 2
Tibor Kovács

Content and the layers of the eyeball are related to the

References ............................................................. 6
meninges. The retina and the brain are supplied
by a common artery. The orbits and the intracra-
nial structures feature strong anatomical connec-
The eye is the mirror of the soul, or it is rather a tions. The visual system has some contact with
mirror of the brain, a window to the brain, at least all regions of the brain from the retina to the
for neurologists. In neurology, the examination occipital cortex. The anatomy and the vestibular
of the optic nerve and eye movements are part of connections of oculomotor regulation constitute
the physical examination. Fundoscopy is vital in some of the most difficult topics both in medical
emergency neurology. Visual field testing is per- and specialty training. Structures related to vision
formed both during neurological and ophthalmo- can be found almost everywhere in the nervous
logical examinations, but its most accurate system (one-third of supratentorial structures
assessment is best performed with ophthalmo- plays a role in vision); thus, they can be affected
logical devices. Eye movement and pupil reac- by nearly all neurological diseases; the descrip-
tion tests are also carried out by both specialties; tion of the disorder can help to identify the trig-
however, the criteria for these evaluations might ger factor (Newman et al. 2003).
be the most different in these tests. As a result, it is easy to understand why oph-
The interdependence of neurology and oph- thalmologists and neurologists often point at
thalmology is justified by the complexity of the each other when faced with several diseases of
visual system. The eyes are ultimately part of the the visual and oculomotor system. In our every-
brain, the retina develops from the diencephalon, day practice, most of us have seen patients with
anterior ischemic optic neuropathy pushed to and
fro by neurological and ophthalmological wards
T. Kovács, M.D., PhD, Habil. and outpatient clinics. Neuro-ophthalmology
University Associate Professor, Department of is a field where the specialist has to be an oph-
Neurology, Faculty of Medicine, Semmelweis
University, Budapest, Hungary
thalmologist and a neurologist at the same time,
which is, of course, not achievable. As a result
Cerebrovascular and Dementia Unit, Department
of General Neurology, Semmelweis University,
of the system of education, everybody quali-
Budapest, Hungary fies as an ophthalmologist or a neurologist first,
Dementia and Dystonia Outpatient Department,
which means that we are going to have neu-
Semmelweis University, Budapest, Hungary rologists who are good at ophthalmology and
e-mail: [email protected] ophthalmologists who are good at neurology; a

© Springer International Publishing Switzerland 2016 5

J. Somlai, T. Kovács (eds.), Neuro-Ophthalmology, DOI 10.1007/978-3-319-28956-4_2
6 T. Kovács

situation which has both advantages and disad- fibrosis of the extraocular muscles. Nowadays
vantages. The most significant consequences of the group has extensive neurological literature
this separation are apparent in the case of rare as well, as the cause of oculomotor disorders has
diseases. In 1992, a child was admitted to the proved to be a developmental disorder of cranial
Department of Neurology in Boston, USA (Engle nerve motor neurons (congenital cranial dysin-
2007) with bilateral ptosis and downward gaze. nervation disorders) rather than fibrosis. There
Investigations for myasthenia, mitochondrial dis- are many other ‘surprising’ diseases like this;
ease, and congenital myopathy yielded no results. therefore, this book might serve to decrease the
The result of ophthalmological consultation was number of such surprises for both ophthalmolo-
somewhat surprising for the neurologists as the gists and neurologists.
ophthalmologists diagnosed the case as congeni-
tal fibrosis of the extraocular muscles. This dis-
ease entity was unknown for the neurologists in References
spite of the fact that it had been first described in
ophthalmology in the 1800s, as belonging to the Engle EC. Oculomotility disorders arising from disrup-
tions in brainstem motor neuron development. Arch
group of ocular fibroses, the most well-known
Neurol. 2007;64:633–7.
manifestation of which is Duane syndrome. In Newman NJ et al. Neuro-ophthalmology update.
ophthalmological literature, the cause of this Continuum Am Acad Neurol. 2003;9:11–78.
group of diseases was thought to be the primary
 Part II
Objectives and Recent Results in the
Neuro-Ophthalmological Clinical Practice

3. Mechanisms of Parallel Information Processing in the Visual System

4. A New Direction: Neuro-Endocrino-Immunology
5. Visual and Oculomotor Disorders in Internal Diseases
6. Genetic Aspects of Neuro-Ophtalmological Diseases
7. Recent Knowledge in the Neurosurgical Practice Regarding the Visual
System
8. The Role of Gamma Knife Stereotactic Radiosurgery in the Treatment
of Neuro-ophthalmological Diseases
9. Neurointerventional Treatment of Diseases Causing Neuro-
ophthalmological Symptoms
10. Recent Results in Neuropathology: Demyelinating and Conformational
Diseases
 Mechanisms of Parallel
Information Processing 3
in the Visual System

György Benedek

Contents of parallel, independent processing of somatic

Interrelations of Focal and Global Vision ............ 12 sensations.
Clinical Evidence .................................................... 12 As a pioneer of the principle of parallel pro-
Glaucoma ................................................................ 13 cessing in the visual system, Bishop made an
Alzheimer’s Disease ................................................ 13
observation in 1933 about distinct groups of optic
Amblyopia ................................................................ 13
fibers of frogs and rabbits based on their conduc-
Further Readings ................................................... 14 tion speed. This has led him to the conclusion
that these different fiber groups transmit different
components of visual perception similarly to
Visual information encoded on the retina is pro- somatosensory pathways. However, while it was
cessed in two anatomically independent pathway quite easy to recognize the submodalities pro-
systems in the central nervous system. This par- cessed in a parallel fashion in the somatosensory
allel character of the structure and functioning of system, the differentiation of the visual submo-
the visual system has long since been known, dalities in the visual system has been shown to be
however, researchers have only recognized its significantly more difficult. The main reason for
physiological and clinical importance in the past this was that none of the fiber groups could be
years. linked to the receptor populations known at that
The original concept of Aristotle has matched time (cones and rods).
the five senses: sight, sound, smell, taste and The publication of Enroth-Cugell and Robson
touch with independent sensory organs. In 1844, was considered as a breakthrough, as they
Volkmann suggested that the different sensory described two different types of ganglion cells in
modalities perceived through the skin (touch, the retina of the cat: the Y and the X cells. The
vibration, cold, warm, pain) are transmitted by authors found that the stimuli on the relatively
distinct populations of neurons in the central ner- big receptive field of the Y-cells are not summed
vous system. This idea has founded the principle linearly, while X cells added stimuli linearly in
their small receptive field. X cells were in the
majority around the central area, while Y cells
were mostly found in the peripheries (Fig. 3.1).
G. Benedek, M.D., PhD The importance of the two different cell groups
Faculty of Medicine, Institute of Physiology, were particularly highlighted when a great differ-
Albert Szent-Györgyi Clinical Center,
University of Szeged, Szeged, Hungary ence was discovered in the conduction speed of
e-mail: [email protected] the axons pertaining to these two cell types.

© Springer International Publishing Switzerland 2016 9

J. Somlai, T. Kovács (eds.), Neuro-Ophthalmology, DOI 10.1007/978-3-319-28956-4_3
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