- •Contents
- •General Introduction
- •Objectives
- •Introduction
- •1 Neuro-Ophthalmic Anatomy
- •Bony Anatomy
- •Skull Base
- •The Orbit
- •Vascular Anatomy
- •Arterial System
- •Venous System
- •Afferent Visual Pathways
- •Retina
- •Optic Nerve
- •Optic Chiasm
- •Optic Tract
- •Cortex
- •Efferent Visual System (Ocular Motor Pathways)
- •Cortical Input
- •Brainstem
- •Ocular Motor Cranial Nerves
- •Extraocular Muscles
- •Sensory and Facial Motor Anatomy
- •Trigeminal Nerve (CN V)
- •Facial Nerve (CN VII)
- •Eyelids
- •Ocular Autonomic Pathways
- •Sympathetic Pathways
- •Parasympathetic Pathways
- •2 Neuroimaging in Neuro-Ophthalmology
- •Computed Tomography
- •Magnetic Resonance Imaging
- •Vascular Imaging
- •Catheter or Contrast Angiography
- •Magnetic Resonance Angiography and Magnetic Resonance Venography
- •Computed Tomography Angiography and Computed Tomography Venography
- •Metabolic and Functional Imaging Modalities
- •Sonography
- •Retinal and Nerve Fiber Layer Imaging
- •Fundamental Concepts in Localization
- •Crucial Questions in Imaging
- •When to Order
- •What to Order
- •How to Order
- •Negative Study Results
- •Glossary
- •3 The Patient With Decreased Vision: Evaluation
- •History
- •Unilateral Versus Bilateral Involvement
- •Time Course of Vision Loss
- •Associated Symptoms
- •Examination
- •Best-Corrected Visual Acuity
- •Color Vision Testing
- •Pupillary Testing
- •Fundus Examination
- •Visual Field Evaluation
- •Adjunctive Testing
- •Ocular Media Abnormality
- •Retinopathy
- •Vitamin A Deficiency
- •Hydroxychloroquine and Chloroquine Retinopathy
- •Cone Dystrophy
- •Paraneoplastic Syndromes
- •Optic Neuropathy
- •Visual Field Patterns in Optic Neuropathy
- •Anterior Optic Neuropathies With Optic Disc Edema
- •Anterior Optic Neuropathies Without Optic Disc Edema
- •Posterior Optic Neuropathies
- •Optic Atrophy
- •Chiasmal Lesions
- •Visual Field Loss Patterns
- •Etiology of Chiasmal Disorders
- •Retrochiasmal Lesions
- •Optic Tract
- •Lateral Geniculate Body
- •Temporal Lobe
- •Parietal Lobe
- •Occipital Lobe
- •Visual Rehabilitation
- •5 The Patient With Transient Visual Loss
- •Examination
- •Transient Monocular Visual Loss
- •Ocular Causes
- •Orbital Causes
- •Systemic Causes
- •Vasospasm, Hyperviscosity, and Hypercoagulability
- •Transient Binocular Visual Loss
- •Migraine
- •Occipital Mass Lesions
- •Occipital Ischemia
- •Occipital Seizures
- •6 The Patient With Illusions, Hallucinations, and Disorders of Higher Cortical Function
- •The Patient With Visual Illusions and Distortions
- •Ocular Origin
- •Optic Nerve Origin
- •Cortical Origin
- •The Patient With Hallucinations
- •Ocular Origin
- •Optic Nerve Origin
- •Cortical Origin
- •The Patient With Disorders of Higher Cortical Function
- •Disorders of Recognition
- •Disorders of Visual–Spatial Relationships
- •Disorders of Awareness of Vision or Visual Deficit
- •Fundamental Principles of Ocular Motor Control
- •Anatomy and Clinical Testing of the Functional Classes of Eye Movements
- •Ocular Stability
- •Vestibular Ocular Reflex
- •Optokinetic Nystagmus
- •Saccadic System
- •Pursuit System
- •Vergence
- •Clinical Disorders of the Ocular Motor Systems
- •Ocular Stability Dysfunction
- •Vestibular Ocular Dysfunction
- •Optokinetic Nystagmus Dysfunction
- •Saccadic Dysfunction
- •Pursuit Dysfunction
- •Vergence Disorders
- •8 The Patient With Diplopia
- •History
- •Physical Examination
- •Monocular Diplopia
- •Comitant and Incomitant Deviations
- •Localization
- •Supranuclear Causes of Diplopia
- •Skew Deviation
- •Thalamic Esodeviation
- •Vergence Dysfunction
- •Nuclear Causes of Diplopia
- •Internuclear Causes of Diplopia
- •One-and-a-Half Syndrome
- •Infranuclear Causes of Diplopia
- •Third Nerve Palsy
- •Fourth Nerve Palsy
- •Sixth Nerve Palsy
- •Neuromyotonia
- •Paresis of More Than One Cranial Nerve
- •Cavernous Sinus and Superior Orbital Fissure Involvement
- •Neuromuscular Junction Causes of Diplopia
- •Myopathic, Restrictive, and Orbital Causes of Diplopia
- •Thyroid Eye Disease
- •Posttraumatic Restriction
- •Post–Cataract Extraction Restriction
- •Orbital Myositis
- •Neoplastic Involvement
- •Brown Syndrome
- •9 The Patient With Nystagmus or Spontaneous Eye Movement Disorders
- •Introduction
- •Early-Onset (Childhood) Nystagmus
- •Infantile Nystagmus Syndrome (Congenital Nystagmus)
- •Fusional Maldevelopment Nystagmus Syndrome (Latent Nystagmus)
- •Monocular Nystagmus of Childhood
- •Spasmus Nutans
- •Gaze-Evoked Nystagmus
- •Rebound Nystagmus
- •Vestibular Nystagmus
- •Peripheral Vestibular Nystagmus
- •Central Forms of Vestibular Nystagmus
- •Acquired Pendular Nystagmus
- •Oculopalatal Myoclonus or Tremor
- •See-Saw Nystagmus
- •Dissociated Nystagmus
- •Saccadic Intrusions
- •Saccadic Intrusions With Normal Intersaccadic Intervals
- •Saccadic Intrusions Without Normal Intersaccadic Intervals
- •Voluntary Flutter (“Nystagmus”)
- •Additional Eye Movement Disorders
- •Convergence-Retraction Nystagmus
- •Superior Oblique Myokymia
- •Oculomasticatory Myorhythmia
- •Eye Movements in Comatose Patients
- •Ocular Bobbing
- •10 The Patient With Pupillary Abnormalities
- •History
- •Pupillary Examination
- •Baseline Pupil Size
- •Pupil Irregularity
- •Anisocoria
- •Anisocoria Equal in Dim and Bright Light
- •Anisocoria Greater in Dim Light
- •Anisocoria Greater in Bright Light
- •Disorders of Pupillary Reactivity: Light–Near Dissociation
- •Afferent Visual Pathway
- •Midbrain
- •Aberrant Regeneration
- •Other Pupillary Disorders
- •Benign Episodic Pupillary Mydriasis
- •11 The Patient With Eyelid or Facial Abnormalities
- •Examination Techniques
- •Ptosis
- •Congenital Ptosis
- •Acquired Ptosis
- •Pseudoptosis
- •Apraxia of Eyelid Opening
- •Eyelid Retraction
- •Abnormalities of Facial Movement
- •Seventh Nerve Disorders
- •Disorders of Underactivity of the Seventh Nerve
- •Disorders of Overactivity of the Seventh Nerve
- •12 The Patient With Head, Ocular, or Facial Pain
- •Evaluation of Headache
- •Migraine and Tension-type Headache
- •Trigeminal Autonomic Cephalgias and Hemicrania Continua
- •Idiopathic Stabbing Headache
- •Inherited Encephalopathies Resembling Migraine
- •Ocular and Orbital Causes of Pain
- •Trochlear Headache and Trochleitis
- •Photophobia
- •Facial Pain
- •Trigeminal Neuralgia
- •Glossopharyngeal Neuralgia
- •Occipital Neuralgia
- •Temporomandibular Disease
- •Carotid Dissection
- •Herpes Zoster Ophthalmicus
- •Neoplastic Processes
- •Mental Nerve Neuropathy
- •Examination Techniques
- •Afferent Visual Pathway
- •Ocular Motility and Alignment
- •Pupils and Accommodation
- •Eyelid Position and Function
- •Management of the Patient With Nonorganic Complaints
- •Immunologic Disorders
- •Giant Cell Arteritis
- •Multiple Sclerosis
- •Myasthenia Gravis
- •Thyroid Eye Disease
- •Sarcoidosis
- •Inherited Disorders With Neuro-Ophthalmic Signs
- •Myopathies
- •Neurocutaneous Syndromes
- •Posterior Reversible Encephalopathy Syndrome
- •Lymphocytic Hypophysitis
- •Cerebrovascular Disorders
- •Transient Visual Loss
- •Vertebrobasilar System Disease
- •Cerebral Aneurysms
- •Arterial Dissection
- •Arteriovenous Malformations
- •Cerebral Venous Thrombosis
- •Neuro-Ophthalmic Manifestations of Infectious Diseases
- •Human Immunodeficiency Virus Infection
- •Herpesvirus
- •Mycobacterium
- •Syphilis
- •Progressive Multifocal Leukoencephalopathy
- •Toxoplasmosis
- •Lyme Disease
- •Fungal Infections
- •Prion Diseases
- •Radiation Therapy
- •Basic Texts
- •Related Academy Materials
- •Requesting Continuing Medical Education Credit
Figure 1-12 Vertebrobasilar arterial system and major arteries with common variations of the cortical branches of the posterior cerebral artery. A, Lateral view. B, Anteroposterior view. Vertebral artery (VA) and basilar artery (BA) branches: anterior inferior cerebellar artery (AICA); posterior inferior cerebellar artery (PICA); superior cerebellar artery (SCA). Posterior cerebral artery (PCA) and its branches: calcarine artery (CalcA); parieto-occipital artery (ParOccipA); posterior choroidal artery (PChoA); posterior temporal (PTempA); posterior communicating artery (PCoA). (Illustration b y Dave Peace.)
The distal 2 sets of circumferential arteries consist of the superior cerebellar artery (SCA) followed by the PCA, representing the terminal branches of the BA at the level of the midbrain. Perforators from the proximal SCA partially supply the nucleus of CN III and its fascicles. In addition, small branches often supply the CN V root. CN III exits between the SCA and PCA, where it may be compressed by an aneurysm.
Perforators from the proximal PCA (P1 segment) supply the rostral portion of the midbrain (involved in vertical gaze) and part of the LGN. A large branch, the artery of Percheron, often supplies both sides of the midbrain from one of the PCAs. Because thalamostriate arteries originate from P1, infarcts related to the internal ICA–MCA spare the thalamus. The P1 segment ends with the PCoA, which joins the vertebrobasilar circulation to the carotid circulation anteriorly. The connecting PCoA parallels the course of CN III, which explains the high frequency of CN III palsy with PCoA aneurysms (see Chapters 2 and 8). As the distal PCA courses around the brainstem, it gives off a parieto-occipital branch before terminating in the calcarine branch, which supplies the primary visual cortex (Fig 1-13).
Figure 1-13 The occipital cortex and its blood supply. Areas V1, V2, and V3 keyed by color. CalcA = calcarine artery; CC = corpus callosum; MCA = middle cerebral artery; PCA = posterior cerebral artery. (Illustration b y Craig A. Luce.)
Venous System
Ocular venous outflow begins in the arcade retinal veins, which exit into the central retinal vein (CRV) and in the choroidal veins, which exit the sclera through the vortex veins. Anteriorly, the episcleral venous plexus collects both blood from the anterior uveal circulation and aqueous percolating through the Schlemm canal. These 3 primary venous drainage pathways (Fig 1-14) empty mainly into the superior ophthalmic vein, which runs posteriorly within the superior medial orbit to the orbital apex, where it crosses laterally to enter the cavernous sinus posterior to the superior orbital fissure.
Figure 1-14 Venous drainage of the orbit. A, Anterior view of the superficial venous system of the eyelids. B, Sagittal view of the venous circulation of the orbit and globe. v = vein. (Illustrations b y Christine Gralapp.)
Microscopic collaterals variably exist between these venous beds. In rare instances, shunts connecting retinal veins to choroidal veins are present within the retina. More commonly (usually in association with central retinal vein occlusion or optic nerve sheath meningioma), optociliary shunt vessels (retinochoroidal collateral vessels) may appear on the disc surface. At a more macroscopic level, the superior ophthalmic vein is variably connected anteriorly to the angular and facial veins and inferiorly to the inferior ophthalmic vein and pterygoid venous plexus. These collaterals may become important, particularly in patients with elevated venous pressure (usually related to a carotid cavernous fistula).
Intracranially, the superficial cortical venous system drains mainly superiorly and medially to the superior sagittal sinus running in the sagittal midline (Fig 1-15). In addition to the cortical drainage, the superior sagittal sinus absorbs CSF through the arachnoid villi and the pacchionian granulations. Thus, obstruction to venous outflow results in decreased CSF absorption and elevated intracranial pressure. The superior sagittal sinus runs posteriorly to terminate at the torcular Herophili (confluence of the venous sinuses) at the level of the tentorium separating the cerebellum from the occipital lobes. The transverse sinuses run anteriorly from the connection of the tentorium and the skull to the petrous pyramid, where they turn to run caudally as the sigmoid sinus down to the jugular bulb, where the internal jugular vein exits the skull.
Figure 1-15 Illustration of the cerebral venous sinus system. (Illustration b y Christine Gralapp.)
Inferior superficial cortical venous drainage is carried directly down to the transverse and sigmoid sinuses through the vein of Labbé and the basilar vein of Rosenthal. The deep drainage of the supratentorial diencephalon and mesencephalon begins as deep draining veins (often in relationship to the ventricular system). These deep veins drain together to form the vein of Galen,
which runs posteriorly to drain into the straight sinus. This runs within the tentorium to drain, along with the superior sagittal sinus, into the torcular Herophili.
Some anterior cerebral venous drainage may access the cavernous sinus. The 2 cavernous sinuses are joined by variable connections through the sella and posteriorly through a plexus of veins over the clivus. The cavernous sinus drains primarily caudally into the jugular bulb via the inferior petrosal sinus, which traverses the Dorello canal with CN VI under the petroclinoid ligament. Alternatively, drainage may occur laterally along the petrous apex through the superior petrosal sinus to the junction of the transverse and sigmoid sinuses (Fig 1-16). Small veins may drain through the foramen rotundum and foramen ovale as well as through the pterygoid plexus to anastomose with the facial venous system (external jugular vein).
Figure 1-16 Anatomy of the cavernous sinus drainage system. (Illustration b y Christine Gralapp.)
Veins of the eyelids anastomose medially between the angular vein and branches of the superior ophthalmic vein (particularly at the superior medial orbit in the region of the trochlea). Facial veins drain inferiorly and laterally to form the external jugular vein, which eventually joins the internal jugular in the neck.
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