- •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 10-7 A, Right Adie tonic pupil. B, After instillation of pilocarpine (0.1%), the right pupil becomes miotic, demonstrating supersensitivity. (Courtesy of Lanning B. Kline, MD.)
Patients with tonic pupils may have accommodative symptoms or photophobia but just as often have no symptoms and report that anisocoria was first noticed by a friend or relative. Accommodative symptoms are difficult to treat. Fortunately, they usually resolve spontaneously within a few months of onset. When photophobia from a dilated pupil is a problem, topical dilute pilocarpine (0.1%) may be helpful. With time (months to years), an Adie tonic pupil gets smaller. Histologic examination of the ciliary ganglion in patients with Adie tonic pupil has shown a reduction in the number of ganglion cells.
Kardon RH, Corbett JJ, Thompson HS. Segmental denervation and reinnervation of the iris sphincter as shown by infrared videographic transillumination. Ophthalmology. 1998;105(2):313–321.
Third nerve palsy
Pupillary involvement in third nerve palsy is almost always accompanied by ptosis and limited ocular motility. At times, the motility disturbance may be subtle, requiring careful quantitation with alternate cover testing. Pupillary dysfunction is an important factor in evaluating acute third nerve palsy. When the pupil is involved, an aneurysm at the junction of the internal carotid and posterior communicating arteries must be excluded (see Chapter 2., Fig 2-11). If the pupil is spared and all other functions of the third nerve are completely paretic, an aneurysm can likely be ruled out.
Aberrant regeneration of the oculomotor nerve may cause mydriasis and a synkinetic pupillary reaction. (See images provided by The Neuro-Ophthalmology Virtual Education Library, available at http://novel.utah.edu.) Portions of the pupillary sphincter contract with attempted movement of the eye, especially medially.
For additional discussion of third nerve palsy, see Chapter 8.
Czarnecki JS, Thompson HS. The iris sphincter in aberrant regeneration of the third nerve. Arch Ophthalmol. 1978;96(9):1606– 1610.
Disorders of Pupillary Reactivity: Light–Near Dissociation
Light–near dissociation occurs when a near response exceeds the best pupillary constriction that
bright light can produce. This finding may arise from a variety of causes (Table 10-1).
Table 10-1
Afferent Visual Pathway
Optic neuropathy is the most common cause of light–near dissociation (unilateral or bilateral). In optic neuropathy, light–near dissociation is the result of damage to only the afferent limb of the pupillary light reflex (optic nerve); the central near impulses remain unaffected.
Midbrain
Dorsal midbrain damage can result in midsize pupils with poor light response and preserved near response. Such responses occur when the lesion spares the more ventrally located fibers of the near reflex pathway. Associated findings include bilateral eyelid retraction (Collier sign), vertical gaze palsy, accommodative paresis, and convergence-retraction nystagmus (Parinaud syndrome) (see Chapter 7, Fig 7-5).
The Argyll Robertson pupil occurs in patients with tertiary syphilis involving the central nervous system. Affected patients have small pupils (<2 mm) that are often irregular. The pupils do not react to light, but the near response and subsequent redilation are normal and brisk. This feature distinguishes Argyll Robertson pupils from bilateral chronic tonic pupils, which may also result from neurosyphilis. In addition, iris atrophy frequently occurs, portions of the iris transilluminate, and dilation is poor after instillation of mydriatic eyedrops.
Argyll Robertson–like pupils are observed in widespread autonomic neuropathies such as bilateral tonic pupils (chronic), diabetes mellitus, and chronic alcoholism, as well as in encephalitis and after panretinal photocoagulation. Serologic tests for syphilis, such as the serum fluorescent treponemal antibody-absorption (FTA-ABS) test and the Treponema pallidum hemagglutination assay (TPHA), should be considered in the evaluation of patients with bilateral pupillary light–near dissociation with miosis.
Aberrant Regeneration
Light–near dissociation can also result from aberrant regeneration of damaged nerves that restores the near reflex but not the light reflex. In tonic pupil syndrome, the injured short ciliary nerves resprout and accommodative fibers mistakenly reinnervate the iris sphincter. Similar misdirected growth can
