Nystagmus In Infancy and Childhood: Current Concepts in Mechanisms, Diagnoses, and Management

Hardcover | January 16, 2013

byRichard W. Hertle, Louis F. DellOsso

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Nystagmus in Infancy and Childhood is a highly-illustrative and thoughtfully written text that provides clinicians and scientists with detailed yet concise information regarding our current understanding, evaluation, and treatments of nystagmus in infancy and childhood. Throughout the text areclinical pearls and narrative observations intended to help the reader appreciate the enormous strides forward in the past 50 years of nystagmus research. Timely and comprehensive, this book is an "everything you need to know" resource, and will provide the reader with:- detailed methodologies of investigation, including analysis software, models of the ocular motor system, and current hypotheses regarding ocular motor oscillations- complementary appendices that can be used for special purposes, i.e., as clinical examination sheets, patient information sheets, and algorithm for computer analysis of nystagmus waveforms- new therapeutic approaches, using relevant eye-movement data and mechanisms- a roadmap toward a more rational, data-driven approach to the medical management of infantile nystagmusAs the only resource effectively comprising the past 50 years of nystagmus research and therapeutic implications, Nystagmus in Infancy and Childhood will be a comprehensive and invaluable guide to for both clinicians and scientists who care for infants and children with nystagmus.

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Nystagmus in Infancy and Childhood is a highly-illustrative and thoughtfully written text that provides clinicians and scientists with detailed yet concise information regarding our current understanding, evaluation, and treatments of nystagmus in infancy and childhood. Throughout the text areclinical pearls and narrative observations ...

Richard W. Hertle MD, FACS, FAAO, FAAP, is Chief of Pediatric Ophthalmology and Director of Adult Motility at The Laboratory of Visual and Ocular Motor Physiology, Children's Hospital Medical Center of Akron, as well as Professor of Ophthalmology at SUMMA Health System, Akron, Ohio and The Northeastern Ohio Universities College of Med...
Format:HardcoverDimensions:336 pages, 10 × 7 × 0.98 inPublished:January 16, 2013Publisher:Oxford University PressLanguage:English

The following ISBNs are associated with this title:

ISBN - 10:0199857008

ISBN - 13:9780199857005

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Table of Contents

1. Relevant Anatomy and Physiology1.1 Infranuclear Ocular Motor Anatomy1.1.1 Extraocular Muscles1.1.2 Extraocular Muscle Pulleys1.1.3 Orbital Tissues1.2 Supranuclear Ocular Motor Anatomy1.2.1 Frontal Eye Fields1.2.2 Superior Colliculus1.2.3 Brainstem Nuclei1.2.4 Vestibular Nuclei1.2.5 Cerebellum1.3 Afferent System1.3.1 Retina/Optic Nerve1.3.2 Optic Nerve1.3.3 Lateral Geniculate1.3.4 Geniculostriate1.3.5 Association Cortex1.3.6 Ocular Motor Proprioception1.4 Efferent System1.4.1 Smooth Pursuit System1.4.2 Saccadic System1.4.3 Vergence System1.4.4 Vestibuloocular System2. Infantile Nystagmus Syndrome2.1 Characteristics of INS2.1.1 History and Background2.1.1.1 Ancient Descriptions and Theories2.1.1.2 Connection to Fixation Attempt2.1.1.3 Modern Physiological Investigation2.1.2 Waveforms, Models, and Mechanisms2.1.2.1 Waveform Types2.1.2.2 Braking and Foveating Saccades2.1.2.3 The Foveation Period2.1.2.4 Foveation Accuracy2.1.2.5 Target Acquisition Time2.1.2.6 Smooth Pursuit2.1.3 The Static Neutral Zone/Region2.1.3.1 Latent Component2.1.4 The Dynamic Neutral Zone/Region2.1.4.1 Asymmetric, (a)Periodic Alternation2.1.4.2 Optokinetic, Pursuit and Vestibuloocular Responses2.1.5 The Null Angle/Zone/Region2.1.6 The Convergence Null2.1.7 The Saccadic Response2.1.8 Static and Dynamic Head Posturing2.1.9 Foveation and Visual Acuity (High Spatial Frequency Vision)2.1.9.1. The eXpanded Nystagmus Acuity Function (NAFX)2.1.9.1.1 NAFX vs. Gaze Angle2.1.9.1.2 LFD and TID2.1.10 Oscillopsia Suppression2.1.10.1 Foveation Dynamics2.1.10.2 Temporal Sampling2.1.10.3 Efference Copy2.1.11 Afferent Stimulation2.1.11.1 Cutaneous Trigeminal Stimulation2.1.11.2 Deep Muscle Stimulation2.1.11.3 Contact Lenses2.1.11.4 Biofeedback2.1.12 Canine Nystagmus (Achiasmatic Belgian Sheepdog)2.1.12.1 See-Saw2.1.12.2 Pendular2.1.12.3 Tenotomy and Reattachment Procedure2.1.13 Canine Model of INS with RPE65 Retinal Degeneration (Briard)2.2 Etiology of INS2.2.1 Familial (Gene Defect)2.2.2 Developmental Disturbance Of Ocular Motor System with Associated Sensory System Deficit.2.2.2.1 Albinism2.2.2.2 Achiasma2.2.2.3 Infantile Strabismus2.2.2.4 Non-vectorial Visual Sensory Deficits2.2.3 The Direct Cause(s) of INS with or without Sensory/Genetic Deficits2.2.3.1 Loss of Smooth-Pursuit Damping2.2.3.2 Tonic Vestibular-Optokinetic Imbalance2.3 Visual Function Deficits and Measurements of INS2.3.1 Static Deficits2.3.1.1 The eXpanded Nystagmus Acuity Function and Longest Foveation Domain Measures2.3.2 Dynamic Deficits2.3.2.1 Target Acquisition Time (Stationary Targets)2.3.2.2 Target Acquisition Time (Moving Targets)2.3.3 Clinical2.3.3.1 Visual Acuity at Different Gaze Angles2.4 Treatment of INS2.4.1 Goals2.4.2 Non-Surgical2.4.2.1 Prisms2.4.2.2 Contact Lenses2.4.2.3 Drugs2.4.2.4 Biofeedback2.4.2.5 Gene-Transfer Therapy2.4.3 Surgical2.4.3.1 Four-Muscle Resection and Recession Procedure (Operation 1) (aka Kestenbaum, Anderson-Kestenbaum, or Anderson+Goto)2.4.3.2 Two-Muscle Recession Procedure (Operation 1A) (aka Anderson)2.4.3.3 Bilateral Medial Rectus Recession Procedure (Operation 8)2.4.3.4 Tenotomy and Reattachment Procedure (Operation 6)3. Fusion Maldevelopment Nystagmus Syndrome3.1 Characteristics of FMNS3.1.1 Waveforms, Models, and Mechanisms3.1.1.1 Types (FMNS plus Nucleus of the Optic Tract)3.1.1.2 The Fixating Eye3.1.1.3 Target Foveation and Dual-Mode Fast Phases3.1.1.4 Foveation Accuracy3.1.2 Variation with Gaze Angle3.1.3 Head Position3.1.4 Foveation, NAFX, and Acuity3.1.5 Efference Copy, Foveation, and Oscillopsia Suppression3.2 Treatment of FMNS3.2.1. Fixation Preference3.2.2. Alexander's Law3.2.3. Eye Muscle Surgery4. Other Types of Nystagmus of Infancy4.1 Nystagmus Blockage Syndrome4.1.1 Characteristics of NBS4.1.1.1 Multiple Types of Nystagmus4.1.1.2 Waveforms and Mechanisms4.1.1.2.1 Target Foveation4.1.1.2.2 Foveation Accuracy4.1.1.3 Purposive Esotropia4.1.1.4 Head Position4.1.1.5 Blockage Syndrome Types I and II4.1.1.6 Foveation, NAFX, and Acuity4.1.1.7 Efference Copy, Foveation, and Oscillopsia Suppression4.1.2 Treatments of NBS4.1.2.1. Fixation Preference4.1.2.2. Alexander's Law4.1.2.3. Surgical4.1.2.3.1. Fixating Eye4.2 Spasmus Nutans Syndrome4.2.1 Characteristics of SNS4.2.1.1 Waveforms and Mechanisms4.2.1.2 Variable Interocular Phase4.2.1.3 Head Nodding4.2.2 Treatment of SNS5. Differential Diagnosis of Nystagmus In Infancy and Childhood5.1 Nystagmus without Associated Neurological Disease - "Benign"5.1.1. Infantile Nystagmus Syndrome5.1.1.1 Association with Strabismus5.1.1.2 Clinical Signs and Symptoms5.1.1.3 Differential Diagnosis5.1.1.4 Reverse-Cover and Gaze-Angle Cover Tests5.1.2 Fusion Maldevelopment Nystagmus Syndrome5.1.2.1 Association with Strabismus5.1.2.2 Clinical Signs and Symptoms5.1.2.3 Differential Diagnosis5.1.2.4 Alternate-Cover and Gaze-Angle Cover Tests5.1.3 Nystagmus Blockage Syndrome5.1.3.1 Association with Strabismus5.1.3.2 Clinical Signs and Symptoms5.1.3.3 Differential Diagnosis5.1.4 Spasmus Nutans Syndrome5.1.4.1 Association with Strabismus5.1.4.2 Clinical Signs and Symptoms5.1.4.3 Differential Diagnosis5.1.5 Nystagmus and Strabismus5.2 Nystagmus with Associated Neurological Disease - "Symptomatic"5.2.1 Vestibular Nystagmus5.2.1.1 Peripheral Vestibular Imbalance5.2.1.2 Central Vestibular Imbalance5.2.1.3 Central Vestibular Instability (Periodic Alternating)5.2.2 Gaze-holding Deficiency Nystagmus5.2.2.1 Eccentric Gaze, Gaze-evoked, Rebound5.2.2.2 Gaze Instability ("Run-away")5.2.3 "Vision-Loss" Nystagmus5.2.3.1 Pre-chiasmal, Optic Chiasm, Post-chiasmal Vision Loss5.2.4 Other Pendular Nystagmus Associated with Diseases of Central Myelin5.2.4.1 Oculopalatal Tremor or "Myoclonus"5.2.4.2 Pendular Vergence Nystagmus Associated with Whipple's Disease5.2.5 Convergence/Convergence Evoked Nystagmus5.2.6 Upbeat Nystagmus5.2.7 Downbeat Nystagmus5.2.8 Torsional Nystagmus5.2.9 "See-Saw" Nystagmus5.2.10 Lid Nystagmus5.3 Saccadic Intrusion/Oscillations5.3.1 Square Wave Jerks And Oscillations5.3.2 Square-Wave Pulses5.3.3 Staircase Saccadic Intrusions5.3.4 Macrosaccadic Oscillations5.3.5 Saccadic Pulses (Single And Double)5.3.6 Convergence Retraction "Nystagmus"5.3.7 Dissociated Ocular Oscillations5.3.8 Dysmetric Saccades5.3.9 Ocular Flutter5.3.10 Flutter Dysmetria5.3.11 Opsoclonus5.3.11.1 Opsoclonus-Myoclonus5.3.12 Superior Oblique Myokymia5.3.13 Ocular bobbing5.3.13.1 Typical5.3.13.2 Monocular5.3.13.3 Atypical5.3.14 Psychogenic (Voluntary) Flutter6. Afferent Visual System - Clinical Examination Procedures6.1 Subjective Testing6.1.1 Teller Acuity Card Procedure6.1.2 Visual Acuity Testing (High Spatial Frequency Vision)6.1.3 Stereo Testing6.1.4 Color-Vision Testing6.1.5 Contrast-Sensitivity Testing6.1.6 Gaze- and Time-Dependent Acuity Testing6.1.7 Visual Field Testing6.2 Objective Testing6.2.1 Visual Evoked Potentials6.2.2 Electroretinography6.2.3 Optical Coherence Tomography6.2.4 Fundus Photography7. Treatment7.1 Medical7.1.1 Optical7.1.1.1 Version Prisms7.1.1.2 Vergence Prisms7.1.1.3 Contact Lenses7.1.1.4 Correction of Ammetropia/Anisometropia7.1.1.5 Intraocular Lenses7.1.1.6 Refractive Surgery7.1.2 Pharmacological7.1.2.1 Sedatives/Hypnotics7.1.2.2 Neuroleptics7.1.2.3 Psychoactive Medications7.1.2.4 Antiseizure Medications7.1.2.5 Botulinum7.1.2.6 Anticholinesterases7.2 Eye-Muscle Surgery7.2.1 General Principles7.2.2 Classification7.2.3 Preoperative Evaluation7.2.3.1 Visual Acuity7.2.3.2 Ocular Motor and Standard Clinical Evaluations7.2.3.3 Strabismus7.2.3.4 Eye-Movement Recordings7.2.3.5 Head-Posture Measurements ("Null Zones")7.2.3.6 Laboratory and Special Tests7.2.4 Results7.2.4.1 Visual Acuity7.2.4.2 Strabismus7.2.4.3 Eye-Movement Recordings7.2.4.4 Head-Posture Measurements7.2.5 Complications7.3 Other7.3.1 Biofeedback7.3.2 Acupuncture7.3.3 Cutaneous Stimulation7.3.4 Gene Transfer Therapy7.3.5 Mind-Body Stress Reduction-Mindful Medication Techniques7.3.6 Occupational and Vision Therapy7.3.7 Educational Assistance7.4 Assessing Therapeutic Outcomes (Post-Therapy)7.4.1 Direct Outcome Measures (NAFX, LFD, and Lt)7.4.1.1 Post-Tenotomy and Reattachment7.4.1.2 Post-Convergence/Bimedial Rectus Recession7.4.1.3 Post-Four-Muscle Recession and Resection or Two-Muscle Recession + Tenotomy and Reattachment7.4.1.4 Soft Contact Lenses7.4.1.5 Systemic Acetazolamide and Topical Brinzolamide7.4.2 Indirect/Clinical Outcome Measures7.4.2.1 Visual Acuity at Different Gaze Angles7.5 Estimating Therapeutic Outcomes (Post-Therapy)7.5.1 Direct Outcome Measures (NAFX, LFD, and Lt)7.5.1.1 Tenotomy and Reattachment (INS without Afferent Visual Deficits)7.5.1.2 Tenotomy and Reattachment (INS with Afferent Visual Deficits)7.5.1.3 Prisms/Bimedial Rectus Recession7.5.1.4 Soft Contact Lenses7.5.2 Indirect/Clinical Outcome Measures7.5.2.1 Visual Acuity at Different Gaze Angles8. Summary and ConclusionsEpilogueAppendicesAppendix A. Eye-Movement Recording Systems and CriteriaA. 1 Recording MethodsA.1.1 Contact ElectrooculographyA.1.2 Infrared ReflectionA.1.3 Scleral Search CoilA.1.4 High-Speed Video OculographyA.2 Research CriteriaA.2.1 NAFXA.2.1.1 MethodologyA.2.1.2 Estimating Visual Function ImprovementsA. 3 Clinical CriteriaA.3.1 Waveform TypesA.3.2 Null and Neutral ZonesA.3.3 PAN and APANA.3.4 Symmetry, Conjugacy, Vergence, and Monocular EvaluationA.4 Calibration TechniquesA.4.1 Adults and ChildrenA.4.1.1 Infantile NystagmusA.4.1.2 Fusion Maldevelopment NystagmusA.4.2 InfantsAppendix B. Clinical ExaminationB.1 General Clinical Examination FormB.2 Nystagmus Examination FormB.3 Strabismus Examination FormB.4 Clinical PearlsB.5 Ophthalmological Myths and FactsAppendix C. Illustrative Cases and TreatmentC.1 Infantile Nystagmus SyndromeC.1.1 Gaze-Angle Null OnlyC.1.1.1 Version PrismsC.1.1.2 Soft Contact LensesC.1.1.3 Four-Muscle Resection, Recession, and Tenotomy and ReattachmentC.1.1.3.1. Fine Tuning with PrismsC.1.1.3.2. Soft Contact LensesC.1.2 Convergence Null OnlyC.1.2.1 Vergence Prisms with Negative SpheresC.1.2.2 Soft Contact LensesC.1.2.3 Bimedial RecessionC.1.3 Both Gaze-Angle and Convergence NullsC.1.3.1 Convergence Gaze-AngleC.1.3.1.1 Composite Prisms and Negative SpheresC.1.3.1.2 Base-out Prisms and Negative SpheresC.1.3.1.3 Soft Contact LensesC.1.3.1.4 Bimedial RecessionC.1.3.2 Gaze-Angle ConvergenceC.1.3.2.1 Version PrismsC.1.3.2.2 Soft Contact LensesC.1.3.2.3 Four-Muscle Resection, Recession and Tenotomy and ReattachmentC.1.4 No NullsC.1.4.1 Soft Contact LensesC.1.4.2 Four-Muscle Tenotomy and ReattachmentC.1.4.2.1 Tenotomy and Reattachment with Augmented Tendon SutureC.1.4.2.2 Augmented Tendon Suture Procedure sans Tenotomy and ReattachmentC.1.4.3 FadenC.2 Infantile Nystagmus Plus StrabismusC.2.1 Gaze-Angle Null OnlyC.2.1.1 Four-Muscle Resection, Recession and Tenotomy and ReattachmentC.2.2 Vertical and Torsional NullsC.2.3 No NullsC.2.3.1 Four-Muscle Tenotomy and Reattachment and StrabismusC.3 Fusion Maldevelopment Nystagmus SyndromeC.3.1 Uniocular FixationC.3.2 Alternating FixationC.3.3 Alexander's Law ThresholdC.4 Nystagmus Blockage SyndromeC.4.1 Bimedial Recession (plus Tenotomy and Reattachment)C.4.2 Recession and Resection plus Tenotomy and ReattachmentC.4.3 Four-Muscle Tenotomy and ReattachmentAppendix D. Diagnosis and Treatment Flow ChartsD.1 Waveform-Based DiagnosisD.2 Therapeutically Exploitable Waveform CharacteristicsD.3 Clinically Based Diagnoses and LimitationsD.4 Thrapeutically Exploitable Clinical CharacteristicsD.5 Analysis GraphsAppendix E. Included Compact Disk and On-Line Access VideosE.1 "Eyeballs 3D" Eye-Motion and Waveform VideosMV1 INS (PPfs) 1 cycle 1/20-speed illustrating foveation periodMV2 INS (Jef) 4 cycles 1/3-speed with phase planeMV3 INS (PPfs) 3 cycles speed illustrating well-developed foveationMV4 INS (PPfs) 3 cycles 1/5-speed with phaseMV5 INS (PPfs) speed 3-dimensional motion with subclinical SSNMV6 INS 1/5-speed Subclinical SSN motion amplifiedMV7 INS PPfs speed from OMS model and patientMV8 INS (Pfs) step responses OMS modelMV9 INS (PPfs) speed pulse responses OMS modelMV10 INS (PPfs) speed OMS model ramp and step-ramp responsesMV11 FMNS gaze-angle effect OMS modelMV12 FMNS speed alternate cover test OMS modelMV13 FMNS adducting eye fixation OMS modelMV14 INS damping with rapid convergenceMV15 INS speed pre-TandRMV16 INS speed post-TandRMV17 INS speed RPE65-deficient canine pre-gene therapyMV18 INS speed RPE65-deficient canine pre-gene therapy scan pathMV19 INS speed RPE65-deficient canine post-gene therapyMV20 INS speed RPE65-deficient canine post-gene therapy scan pathMV21 Oscillopsia simulationMV22 Uniocular APN pre- and post-therapyMV23 INS (alternating J) OMS modelE.1.1 PowerPoint FilesE.1.1 E1_INS Dynamic VAE.1.2 E1_INS Latency PosterE.1.3 E1_ INS LatencyE.1.4 E1_ INS Model PosterE.2 Canine Videos (plus others)CV1 Normal BrittanyCV2 Achiasmatic Belgian Sheepdog: pre-TandRCV3 Achiasmatic Belgian Sheepdog: post-TandRCV4 RPE65-deficient canine: pre-gene therapy behaviorCV5 RPE65-deficient canine: post-gene therapy behaviorCV6 RPE65-deficient canine: pre-gene therapy eye movementsCV7 RPE65-deficient canine: post-gene therapy eye movementsCV8 Cat with INSCV9 Goat with SSNE.3 Patient VideosPV1 Saccadic Initiation FailurePV2 Brainstem Tumor: Tonic Gaze DeviationPV3 Acquired Downbeat NystagmusPV4 Acquired Gaze-Evoked (Gaze-Holding Failure) NystagmusPV5 Acquired Unidirectional Gaze-Evoked (Gaze-Holding Failure) NystagmusPV6 Ocular Motor Neuromyotonia of Cranial Nerve IIIPV7 Ocular Motor Neuromyotonia of Cranial Nerve VIPV8 Infant with Opsoclonus (Ocular Flutter - "Saccadomania")PV9 Acquired Saccadic Oscillations-1PV10 Acquired Saccadic Oscillations-2PV11Acquired Saccadic Oscillations-3PV12 Acquired Nystagmus + Saccadic OscillationsPV13 Acquired Upbeat Nystagmus with Wiernecke's EncephalopathyPV14 Acquired Vertical Pendular NystagmusPV15 Fusion Maldevelopment Nystagmus-1PV16 Fusion Maldevelopment Nystagmus-2PV17 Down Syndrome and Infantile NystagmusPV18 Achiasma + See-Saw Nystagmus + Infantile NystagmusPV19 Octogenarian with Infantile NystagmusPV20 Infantile Nystagmus: Aperiodically Changing Intensity (Not Direction)PV21 Infantile Nystagmus: Aperiodically Changing Direction (Not Intensity)PV22 Infantile Nystagmus: Unequal-1PV23 Infantile Nystagmus: Unequal-2PV24 Infantile Nystagmus: Unequal-3PV25 Infantile Nystagmus: Jerk with Extended FoveationPV26 Infantile + NOT Nystagmus: Dual JerkPV27 Infantile Nystagmus: Pre- and Post-OperativePV28 Optic Nerve Dysplasia and Infantile Nystagmus: MultiplanarPV29 Infantile Nystagmus: Jerk with Extended FoveationPV30 Infantile Nystagmus: Unequal with a "latent component"PV31 Albinism and Infantile NystagmusPV32 Optic Nerve Dysplasia and Infantile Nystagmus: MultiplanarPV33 Albinism, Up-gaze Null, and Infantile Nystagmus: EqualPV34 Retinal Dystrophy and Infantile Nystagmus: MultiplanarPV35 Albinism and Infantile Nystagmus: Pre- and Post-Operative Horizontal NullPV36 Albinism and Infantile Nystagmus: MultiplanarPV37 Infantile Nystagmus: Periodic AlternatingPV38 Infantile Nystagmus: Asymmetric Aperiodic AlternatingPV39 Albinism and Infantile Nystagmus: Pre- and Post-Operative Vertical NullPV40 Albinism, Up-gaze Null, and Infantile NystagmusPV41 Albinism and Infantile NystagmusPV42 "Vertical" Infantile Nystagmus-1PV43 Retinal Dystrophy and "Vertical" Infantile Nystagmus-2PV44 Spasmus Nutans Nystagmus-1PV45 Spasmus Nutans Nystagmus-2PV46 Voluntary Ocular FlutterAppendix F. Included Compact Disk and On-Line AccessF.1 Omlab ReportsF.1.1 #011105 Recording and Calibrating the Eye Movements of Nystagmus SubjectsF.1.2 #111005 Using the NAFX for Eye-Movement Fixation Data Analysis and DisplayF.1.3 #111905 Nystagmus Therapies: Types, Sites, and MeasuresF.1.4 #090506 Original Ocular Motor Analysis of the First Human with Achiasma: Documentation of Work Done in 1994F.1.5 #123007 The Benefits of Extraocular Muscle Surgery in INSF.1.6 #030509 How Someone "Sees" the World and How to Clinically Assess Therapeutic Improvements in Visual FunctionF.2 Patient HandoutsF.2.1 INS InformationF.2.2 INS TreatmentsF.2.3 Tenotomy and ReattachmentF.2.4 INS and AcuityF.2.5 INS MiscellaneousF.3 Physician/Research Scientist WorksheetsF.3.1 Recession and Resection Surgical CalculationF.3.2 Estimating Improvement in Peak NAFXF.3.3 Estimating Improvement in LFDF.3.4 NAFX vs. Visual Acuity (Motor and Sensory Components)F.4 Clinical Examination FormsF.4.1 General Clinical Examination FormF.4.2 Nystagmus Examination FormF.4.3 Strabismus Examination FormF.5 Analysis SoftwareF.5.1 OMtoolsF.5.2 OMS Model GUI User Guide