Pediatric Neuro-Ophthalmology

 Content: Note continued: Tuberous Sclerosis --
 Optic Disc Glioma --
 Combined Hamartoma of the Retina and RPE --
 Retrobulbar Tumors --
 Optic Neuritis in Children --
 History and Physical Examination --
 Postinfectious Optic Neuritis --
 Acute Disseminated Encephalomyelitis --
 MS and Pediatric Optic Neuritis --
 Devic Disease (Neuromyelitis Optica) --
 Prognosis and Treatment --
 Course of Visual Loss and Visual Recovery --
 Systemic Prognosis --
 Systemic Evaluation of Pediatric Optic Neuritis --
 Treatment --
 Leber Idiopathic Stellate Neuroretinitis --
 Ischemic Optic Neuropathy --
 Autoimmune Optic Neuropathy --
 Pseudopapilledema --
 Optic Disc Drusen --
 Ocular Disorders Associated with Pseudopapilledema --
 Systemic Disorders Associated with Pseudopapilledema --
 References --
 Optic Atrophy in Children --
 Introduction --
 Epidemiology --
 Optic Atrophy Associated with Retinal Disease --
 Congenital Optic Atrophy Vs. Hypoplasia --
 Causes of Optic Atrophy in Children --
 Compressive/Infiltrative Intracranial Lesions --
 Noncompressive Causes of Optic Atrophy in Children with Brain Tumors --
 Hereditary Optic Atrophy --
 Dominant Optic Atrophy (Kjer Type) --
 Leber Hereditary Optic Neuropathy --
 Recessive Optic Atrophhy --
 Behr Syndrome --
 Wolfram Syndrome (DIDMOAD) --
 Toxic/Nutritional Optic Neuropathy --
 Neurodegenerative Disorders with Optic Atrophy --
 Organic Acidurias --
 Optic Atrophy due to Hypoxia-Ischemia --
 Traumatic Optic Atrophy --
 Miscellaneous Causes --
 Summary of the General Approach to the Child with Optic Atrophy --
 References --
 4. Transient, Unexplained, and Psychogenic Visual Loss in Children --
 Introduction --
 Transient Visual Loss --
 Migraine --
 Epilepsy --
 Posttraumatic Transient Cerebral Blindness --
 Cardiogenic Embolism --
 Nonmigrainous Cerebrovascular Disease --
 Miscellaneous Transient Visual Disturbances in Children. Note continued: Toxic and Nontoxic Drug Effects --
 Summary of Clinical Approach to the Child with Transient Visual Distrubances --
 Laboratory Evaluation of Transient Visual Disturbances in Children --
 Unexplained Visual Loss in Children --
 Causes of Unexplained Visual Loss in Childhood --
 Psychogenic Visual Loss in Children --
 Clinical Profile --
 Neuro-Opthalmologic Findings --
 Categories of Psychogenic Visual Loss in Children --
 Management of Psychogenic Visual Loss in Children --
 Horizons --
 References --
 6. Ocular Motor Nerve Palsies in Children --
 Introduction --
 Oculomotor Nerve Palsy --
 Clinical Anatomy --
 Clinical Features --
 Partial Forms of Oculomotor Palsy --
 Oculomotor Synkinesis --
 Etiology --
 Vascular Third Nerve Palsy in Children --
 Differential Diagnosis --
 Management --
 Trochlear Nerve Palsy --
 Clinical Anatomy --
 Clinical Features --
 Bilateral Trochlear Nerve Palsy --
 Etiology --
 Differential Diagnosis --
 Treatment --
 Abducens Nerve Palsy --
 Clinical Anatomy --
 Clinical Features --
 Causes of Sixth Nerve Palsy --
 Differential Diagnosis --
 Duane Retraction Syndrome --
 Management of Sixth Nerve Palsy --
 Multiple Cranial Nerve Palsies in Children --
 Horizons --
 References --
 7. Complex Ocular Motor Disorders in Children --
 Introduction --
 Strabismus in Children with Neurological Dysfunction --
 Visuovestibular Disorders --
 Neurologic Esotropia --
 Neurologic Exotropia --
 Skew Deviation --
 Gaze Palsies, Gaze Deviations, and Ophthalmoplegia --
 Horizontal Gaze Palsy in Children --
 Congenital Ocular Motor Apraxia --
 Vertical Gaze Palsies in Children --
 Diffuse Ophthalmoplegia in Children --
 Chronic Progressive External Ophthalmoplegia --
 Myasthenia Gravis --
 Olivopontocerebellar Atrophy --
 Botulism --
 Fisher Syndrome: A Variant of Guillain-Barre Syndrome --
 Bickerstaff Brainstem Encephalitis --
 Tick Paralysis. Note continued: Wernicke Encephalopathy --
 Miscellaneous Causes of Ophthalmoplegia --
 Transient Ocular Motor Disturbances of Infancy --
 Transient Neonatal Strabismus --
 Transient Idiopathic Nystagmus --
 Tonic Downgaze --
 Tonic Upgaze --
 Neonatal Opsoclonus --
 Transient Vertical Strabismus in Infancy --
 Congenital Cranial Dysinnervation Syndromes --
 Congenital Ptosis --
 Marcus Gunn Jaw Winking (Trigemino-Oculomotor Synkinesis) --
 Congenital Fibrosis Syndrome --
 Congenital Horizontal Gaze Palsy with Scoliosis --
 Mobius Sequence --
 Monocular Elevation Deficiency, or \"Double Elevator Palsy\" --
 Brown Syndrome --
 Other Pathologic Synkineses --
 Internuclear Ophthalmoplegia --
 Cyclic, Periodic, or Aperiodic Disorders Affecting Ocular Structures --
 Ocular Neuromyotonia --
 Ocular Motor Adaptations and Disorders in Patients with Hemispheric Abnormalities --
 Eye Movement Tics --
 Eyelid Abnormalities in Children --
 Congenital Ptosis --
 Excessive Blinking in Children --
 Hemifacial Spasm --
 Eyelid Retraction --
 Apraxia of Eyelid Opening --
 Pupillary Abnormalities --
 Congenital Bilateral Mydriasis --
 Accommodative Paresis --
 Adie Syndrome --
 Horner Syndrome --
 References --
 8. Nystagmus in Children --
 Introduction --
 Infantile Nystagmus --
 Clinical Features --
 Onset of Infantile Nystagmus --
 Terminology --
 History and Physical Examination --
 ERG --
 Hemispheric Visual Evoked Potentials --
 Overlap of Infantile Nystagmus and Strabismus --
 Eye Movement Recordings in Infantile Nystagmus --
 Contrast Sensitivity and Pattern Detection Thresholds in Infantile Nystagmus --
 Theories of Causation --
 Visual Disorders Precipitating Infantile Nystagmus --
 When to Obtain Neuroimaging Studies in Children with Nystagmus --
 Treatment --
 Spasmus Nutans --
 Russell Diencephalic Syndrome of Infancy --
 Monocular Nystagmus --
 Nystagmus Associated with Infantile Esotropia. Note continued: Torsional Nystagmus --
 Horizontal Nystagmus --
 Latent Nystagmus --
 Nystagmus Blockage Syndrome --
 Treatment of Nystagmus Blockage Syndrome --
 Vertical Nystagmus --
 Upbeating Nystagmus in Infancy --
 Congenital Downbeat Nystagmus --
 Hereditary Vertical Nystagmus --
 Periodic Alternating Nystagmus --
 Seesaw Nystagmus --
 Congenital versus Acquired Seesaw Nystagmus --
 Saccadic Oscillations that Simulate Nystagmus --
 Convergence-Retraction Nystagmus --
 Opsoclonus and Ocular Flutter --
 Voluntary Nystagmus --
 Ocular Bobbing --
 Neurological Nystagmus --
 Leigh Subacute Necrotizing Encephalomyelopathy --
 Pelizaeus-Merzbacher Disease --
 Joubert Syndrome --
 Santavuori-Haltia Disease --
 Infantile Neuroaxonal Dystrophy --
 Carbohydrate-Deficient Glycoprotein Syndromes --
 Down Syndrome --
 Hypothyroidism --
 Maple Syrup Urine Disease --
 Nutritional Nystagmus --
 Epileptic Nystagmus --
 Cobalamin C Methylmalonic Aciduria and Homocystinuria --
 Familial Vestibulocerebellar Disorder --
 Summary --
 References --
 Torticollis and Head Oscillations --
 Introduction --
 Torticollis --
 Ocular Torticollis --
 Head Tilts --
 Head Turns --
 Vertical Head Positions --
 Refractive Causes of Torticollis --
 Neuromuscular Causes of Torticollis --
 Systemic Causes of Torticollis --
 Head Oscillations --
 Head Nodding with Nystagmus --
 Head Nodding without Nystagmus --
 Visual Disorders --
 Otological Abnormalities --
 Systemic Disorders --
 References --
 Neuro-Ophthalmologic Manifestations of Neurodegenerative Disease in Childhod --
 Introduction --
 Neuronal Disease --
 Neuronal Ceroid Lipofuscinosis --
 Lysosomal Diseases --
 Gangliosidoses --
 Mucopolysaccharidoses --
 Subacute Sclerosing Panencephalitis --
 White Matter Disorders --
 Metachromatic Leukodystrophy --
 Canavan Disease (Spongy Degeneration of Cerebral White Matter) --
 Krabbe Disease. Note continued: Pelizaeus-Merzbacher Disease --
 Cockayne Syndrome --
 Alexander Disease --
 Sjogren-Larsson Syndrome --
 Cerebrotendinous Xanthomatosis --
 Peroxisomal Disorders --
 Zellweger Syndrome --
 Adrenoleukodystrophy --
 Basal Gangila Disease --
 Pantothenate Kinase-Associated Neurodegeneration --
 Wilson Disease --
 Aminoacidpathies and Other Biochemical Defects --
 Maple Syrup Urine Disease --
 Homocystinuria --
 Abetalipoproteinemia --
 Mitochondrial Encephalomyelopathies --
 Chronic Progressive External Ophthalmoplegia (CPEO) --
 Leigh Subacute Necrotizing Encephalomyelopathy --
 Mitochondrial Encephalomyelopathies and Stroke-Like Episodes (MELAS) --
 Myoclonic Epilepsy and Ragged Red Fibers (MERRF) --
 Mitochondrial Depletion Syndrome --
 Congenital Disorders of Glycosylation --
 Horizons --
 References --
 Neuro-Ophthalmologic Manifestations of Systemic and Intracranial Disease --
 Introduction --
 Phakomatoses --
 Neurofibromatosis (NF1) --
 Neurofibromatosis 2 (NF2) --
 Tuberous Sclerosis --
 Struge-Weber Syndrome --
 Von Hippel-Lindau Disease --
 Ataix Telangiectasia --
 Linear Nevus Sebaceous Syndrome --
 Klippel-Trenauney-Weber Syndrome --
 Brain Tumors --
 Suprasellar Tumors --
 Arachnoid Cystsa --
 Cavernous Sinus Lesions --
 Hemispheric Tumors --
 Posterior Fossa Tumors --
 Brainstem Tumors --
 Tumors of the pineal Region --
 Meningiomas --
 Epidermoids and Dermoids --
 Gliomatosis Cerebri --
 Metastasis --
 Complications of Treatment of Intracranial Tumors in Children --
 Hydrocephalus --
 Hydrocephalus due to CSF Overproduction --
 Noncommunicating Hydrocephalus --
 Communicating Hydrocephalus --
 Common Causes of Hydrocephalus in Children --
 Clinical Features of Hydrocephalus --
 Effects and Complications of Treatment --
 Vascular Lesions --
 AVMs --
 Cavernous Angiomas --
 Intacranial Aneurysms --
 Isolated Venous Ectasia. Note continued: Craniocervical Arterial Dissection --
 Strokes in Children --
 Cerebal Venous Thrombosis --
 Cerebral Dysgenesis and Intracranial Malformations --
 Destructive Brain Lesions --
 Malformations Due to Abnormal Stem Cell Proliferation or Apoptosis --
 Malformations Due to Abnormal Neuronal Migration --
 Malformations Secondary to Abnormal Cortical Organization and Late Migration --
 Anomalies of the Hypothalamic-Pituitary Axis --
 Encephaloceles --
 Cerebellar Malformations --
 Miscellaneous --
 Congenital Corneal Anesthesia --
 Reversible Posterior Leukoencephalopathy --
 Cerebroretinal Vasculopathies --
 Syndromes with Neuro-Ophthalmologic Overlap --
 Proteus Syndrome --
 PHACE Syndrome --
 Goldenhar Syndrome (Oculoauriclovertebral) Dysplasia) --
 Delleman (Oculocerebrocutaneous) Syndrome --
 Encephalocraniocutaneous Lipomatosis --
 Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) --
 References.
 Abstract:             \"Due to the generous representation of the afferent visual system within the brain, neurological disease may disrupt vision as a presenting symptom or as a secondary effect of the disease. Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis. The signs and symptoms of neurological disease are elusive by their very nature, presenting a confounding diagnostic challenge. Neurological medications and neurosurgical treatments can produce neuro-ophthalmological dysfunction that can be difficult to distinguish from disease progression. Affected patients may experience substantial delays in diagnosis, and are often subjected to extensive (and expensive) diagnostic testing. Scientific articles pertaining to specific disorders are scattered throughout medical subspecialty journals. These children continue to \"fall through the cracks\" of our medical education system. The increasing recognition that pediatric neuro-ophthalmology comprises a distinct set of diseases from those seen in adults has led to its emergence as a dedicated field of study. \"Since the original publication of Pediatric Neuro-Ophthalmology nearly fourteen years ago, interest in the field has burgeoned. Pediatric ophthalmology and pediatric neurology subspecialty conferences often include symposia dedicated to recent advances in pediatric neuro-ophthalmology. Technical advances in neuroimaging have given rise to a more integrated mechanistic classification of neuro-ophthalmological disease in children. Our understanding of neurodevelopmental disorders of the visual system has expanded, longstanding monoliths have been dissembled into component parts, basic molecular mechanisms have taken center stage, and genetic underpinnings have become definitional. Evolutionary alterations can now be observed at the level of the gene, adding a new dimension to our understanding of disease pathogenesis. New classifications now encompass clinically disparate conditions. Descriptive definitions have been supplanted by mechanistic ones, and clinical definitions superseded by genetic ones. Our concept of disease pathogenesis has been revised and in some cases overturned. Bearing witness to these remarkable advancements has compelled me to enhance and expand the first edition of Pediatric Neuro-Ophthalmology into this new and revised one. \"In the first edition of this book, our goal was to present the clinical characteristics, diagnostic evaluation, and therapeutic options for the common neuro-ophthalmologic disorders of childhood. In so doing, we designed the book to be provide a narrative journey through the thought processes involved in the clinical management of these disorders. In this edition, I have retained the basic narrative format of original book, while expanding the exploration of these complex visual disorders in the context of the many new scientific advancements and discoveries that have come to light. These conditions are fun to diagnose, fascinating to understand, and gratifying to manage.\" --from the Preface to the 2nd Edition