- •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
Sensory and Facial Motor Anatomy
Although the importance of CNs II, III, IV, and VI is obvious, CN V and CN VII also have important impacts on normal ophthalmic function and are frequently involved in neuro-ophthalmic disorders. For example, proper functioning of CN V is essential for preventing corneal damage. In addition, complete loss of corneal sensation may be accompanied by abnormal corneal epithelial growth (neurotrophic keratitis associated with loss of neural secreted growth factors).
Trigeminal Nerve (CN V)
The sensory nerve terminates within the trigeminal nucleus. The nuclear complex of CN V extends from the midbrain to the cervical spinal cord and includes a main sensory nucleus, a mesencephalic nucleus, and a spinal nucleus (Fig 1-36). The main sensory nucleus is located within the pons lateral to the motor nucleus (the most rostral portion of the trigeminal complex except for the mesencephalic nucleus) and receives light-touch information from the skin of the face and the mucous membranes. The mesencephalic nucleus serves proprioception and deep sensation from the facial muscles, including those of mastication and the EOMs. In addition, a spinal nucleus extends caudally to the level of the C4 vertebra, receiving pain and temperature information. The various sensory nuclei of CN V project to the contralateral thalamus and from there to the postcentral gyrus. The motor nucleus of CN V lies in the pons, medial to the sensory nucleus. The motor nucleus sends signals to the muscles of mastication (temporalis, pterygoid, and masseter), the tensor tympani (which dampens sound by tensing the tympanic membrane within the middle ear as a reflex response to loud noises), the tensor veli palatini (which orients the uvula), the mylohyoid muscle, and the anterior belly of the digastric muscle (both of which are strap muscles in the neck).
Figure 1-36 Diagram of the central pathways and peripheral innervation of CN V. (Used with permission from Kline LB. Neuro-
Ophthalmology Review Manual. 6th ed. Thorofare, NJ: Slack; 2008:174.)
The fascicles of CN V enter the brainstem ventrally in the pons and extra-axially traverse the subarachnoid space to penetrate the dura just over the petrous pyramid. Within the subarachnoid space, the trigeminal root often comes in contact with the superior cerebellar artery. This proximity may be a cause of trigeminal neuralgia (atypical facial pain, discussed in Chapter 12) and is the anatomical basis for microvascular decompression. The 3 divisions of CN V synapse in the trigeminal (gasserian) ganglion, located in an extradural space at the floor of the middle cranial fossa (Meckel cave) (Fig 1-37).
Figure 1-37 Lateral view of the orbit, showing its sensory nerves. (Illustration b y Dave Peace.)
The ophthalmic division (V1) is the most anterior branch exiting the trigeminal ganglion. It runs forward within the lateral wall of the cavernous sinus just below CN IV. As it approaches the superior orbital fissure extradurally, it divides into 3 major branches: lacrimal, frontal, and nasociliary. In addition, small branches innervate the dura of the anterior middle cranial fossa, including the cavernous sinus, the parasellar region, the tentorium, and the dura of the petrous apex. These branches also innervate the floor of the anterior cranial fossa, including the falx and the major blood vessels at the skull base.
The lacrimal and frontal nerves enter the orbital apex outside the annulus of Zinn. At its terminus, the frontal nerve divides into supraorbital and supratrochlear branches, which innervate the forehead, frontal sinus, and upper eyelid (including the conjunctiva). The lacrimal nerve also runs anteriorly in the superior lateral orbit just above the lateral rectus to innervate the lacrimal gland and some skin just superotemporal to the orbit. The nasociliary branch is the only branch entering the intraconal space through the annulus of Zinn. The nasociliary branch runs through the ciliary ganglion and anteriorly to innervate the globe through the short and long posterior ciliary nerves. Prior to reaching the globe, branches from the nasociliary division pass through the anterior and posterior ethmoidal foramina to innervate part of the ethmoidal sinuses, the lateral wall of the nose, and the skin of the nose to the nasal tip. This co-innervation of the globe and the nasal skin is the reason behind the development of the Hutchinson sign in patients with zoster ophthalmicus.
The maxillary division (V2) runs forward at the inferior lateral base of the cavernous sinus to enter the foramen rotundum, located just below the superior orbital fissure. Just before entering the canal, V2 gives off the middle meningeal nerve, which supplies the dura of the lateral middle cranial fossa. On the anterior end of the foramen rotundum, V2 enters the pterygomaxillary area. Two large pterygopalatine nerves supply sensation to the nasopharynx, hard and soft palate, and portions of the nasal cavity. Posterior alveolar nerves supply sensation to the upper gums and molars. The zygomatic nerve enters the orbit through the inferior orbital fissure and divides into the zygomaticofacial and the
zygomaticotemporal nerves, which supply sensation to the lateral face (see Fig 1-37). The maxillary nerve continues anteriorly within a canal between the orbit above and the maxillary sinus below to exit through the infraorbital foramen (as the infraorbital nerve) just below the inferior orbital rim. It subsequently divides into palpebral, nasal, and labial branches. The sensation of the cheek as well as the lower eyelid and upper teeth and gums is provided by this division.
The mandibular division (V3) enters through the foramen ovale, lateral to the foramen lacerum and medial to the foramen spinosum (carrying the MMA). V3 innervates the skin of the jaw and carries the motor division of the trigeminal nerve to the muscles of mastication and neck. Motor paralysis results in contralateral deviation of the jaw when it is closed (weakness of the temporalis) and ipsilateral deviation when protruded (because of weakness in the lateral pterygoid).
Facial Nerve (CN VII)
The facial nerve (CN VII) is responsible for the movement of the facial muscles. Voluntary facial movements originate along with other motor activity in the precentral gyrus. White matter tracts pass through the internal capsule and cerebral peduncles along with the other corticobulbar fibers. The motor neurons destined for the upper face receive information from both sides (bilateral innervation), whereas the lower facial musculature receives information only from the contralateral cortex (Fig 1- 38). The CN VII nucleus receives additional information from basal ganglia extrapyramidal connections, which are largely responsible for involuntary blinking. Abnormal blinking, as present in basal ganglia disorders such as Parkinson disease, is probably mediated through alterations in inhibition of the supranuclear control of the blink reflex.
