- •Preface
- •Transient Binocular Visual Loss
- •Migraine with Typical Visual Aura
- •Congenitally Elevated Optic Disc
- •Optic Disc Coloboma
- •Optic Pit
- •Morning Glory Optic Disc Anomaly
- •Optic Disc Hypoplasia
- •Typical Optic Neuritis
- •Atypical Optic Neuritis
- •Arteritic Anterior Ischemic Optic Neuropathy
- •Posterior Ischemic Optic Neuropathy
- •Hypotensive Ischemic Optic Neuropathy
- •Toxic Optic Neuropathy
- •Dominantly Inherited Optic Neuropathy
- •Leber Hereditary Optic Neuropathy
- •Sphenoid Meningioma
- •Optic Nerve Sheath Meningioma
- •Craniopharyngioma
- •Pituitary Adenoma
- •Optic Glioma (Pilocytic Astrocytoma of Optic Nerves or Chiasm)
- •Anterior Visual Pathway Intracranial Aneurysm
- •Traumatic Optic Neuropathy
- •Radiation Optic Neuropathy
- •Graves Optic Neuropathy
- •Papilledema
- •Hypertensive Optic Neuropathy
- •Paraneoplastic Optic Neuropathy
- •Diabetic Papillopathy
- •Homonymous Hemianopia
- •Cerebral (Retrogeniculate, Cortical) Blindness
- •Visual Agnosia
- •Visual Spatial And Attentional Disturbances
- •Orbital Myositis
- •Graves Disease
- •Genetic Extraocular Myopathy
- •Myasthenia Gravis
- •Third Cranial Nerve Palsy
- •Fourth Cranial Nerve Palsy
- •Sixth Cranial Nerve Palsy
- •Unilateral Ophthalmoplegia
- •Bilateral Ophthalmoplegia
- •Direct Carotid–cavernous Fistula
- •Indirect (Dural) Carotid–cavernous Fistula
- •Internuclear Ophthalmoplegia
- •Skew Deviation
- •Dorsal Midbrain (Pretectal) Syndrome
- •Paramedian Thalamic or Midbrain Syndrome
- •Paramedian Pontine Syndrome
- •Dorsolateral Medullary (Wallenberg) Syndrome
- •Acute Upgaze Deviation
- •Acute Downgaze Deviation
- •Omnidirectional Slow Saccades
- •Omnidirectional Saccadic Pursuit
- •Congenital Ocular Motor Apraxia
- •Acute Horizontal Gaze Deviation
- •Ping Pong Gaze
- •Physiologic Nystagmus
- •Congenital Motor Nystagmus
- •Congenital Sensory Nystagmus
- •Monocular Pendular Nystagmus of Childhood
- •Spasmus Nutans
- •Peripheral Vestibular Nystagmus
- •Acquired Binocular Pendular Nystagmus
- •Sidebeat, Upbeat, and Downbeat Nystagmus
- •Epileptic Nystagmus
- •Convergence–retraction “Nystagmus”
- •Voluntary “Nystagmus”
- •Superior Oblique Myokymia
- •Square Wave Jerks
- •Ocular Flutter and Opsoclonus
- •Ocular Dysmetria
- •Ocular Bobbing
- •Tonic Pupil
- •Argyll Robertson Pupils
- •Tectal (Dorsal Midbrain) Pupils
- •Pharmacologically Dilated Pupil
- •Mydriatic Pupil of Third Cranial Nerve Palsy
- •Mydriatic Pupil of Traumatic Iridoplegia
- •Episodic Pupil Dilation
- •Horner Syndrome
- •Ptosis
- •Lid Retraction
- •Apraxia of Eyelid Opening
- •Benign Essential Blepharospasm
- •Hemifacial Spasm
- •Index
Disorders• 8 SECTIONMotor Ocular Brainstem
Omnidirectional Slow Saccades
Key Facts
•Very slow refixational eye movements in both eyes in all directions
•In alert patients, usually caused by damage to saccadic generator in the pons
•Common causes:
• multiple sclerosis • pontine infarction • spinocerebellar ataxias (especially type 2) • progressive supranuclear palsy • genetic extraocular myopathies
Clinical Findings
•Very slow saccadic eye movements in both eyes in all directions
•In neurodegenerative conditions, may also find:
•saccadic intrusions • nystagmus • skew deviation • esotropia • cognitive decline • parkinsonism • spasticity • dysautonomia
Ancillary Testing
•MRI shows brainstem atrophy in some neurodegenerative states, orbital signs in orbitopathies, but is normal in genetic extraocular myopathies
•Blood for genetic testing of inherited ataxias
•Muscle biopsies for mitochondrial myopathies
•Serum for phenytoin level
•PCR for Whipple antigen
Differential Diagnosis
• Common causes:
•multiple sclerosis • pontine infarction • spinocerebellar ataxias (especially type 2) • progressive supranuclear palsy • genetic extraocular myopathies
•Uncommon causes:
•Friedreich ataxia • Huntington disease • phenytoin toxicity • Whipple disease
• paraneoplastic syndromes • ocular motor cranial nerve palsies • Fisher syndrome • myasthenia gravis • multiple system atrophies • Graves disease
• orbital myositis • orbital trauma
•Also consider sedative medication, psychogenic lack of cooperation
Treatment
• Depends on underlying condition
Prognosis
• Depends on underlying condition
150
Fig. 8.19 Omnidirectional slow saccades. When the patient attempts refi xational movements, the eyes move very slowly (indicated by dotted lines).
Saccades Slow Omnidirectional
Fig. 8.20 Hereditary cerebellar degeneration. Axial FLAIR
MRI shows severe atrophy of the cerebellar folia (arrow).
151
Disorders• 8 SECTIONMotor Ocular Brainstem
Omnidirectional Saccadic Pursuit
Key Facts
•As the eyes follow a moving target, their movement is fragmented by fast catchup movements
•Found in very elderly, inattentive, tired, sedated, and sleepy patients
•Otherwise a sensitive but non-specific sign of brain dysfunction
•Prominent finding in cerebellar disorders
Clinical Findings
•Small jerky eye movements (saccades) replace smooth tracking movements (pursuit) when the patient is asked to follow a target moving at <40º/s in any direction
Ancillary Testing
• MRI often negative in degenerative, toxic, metabolic, or inattentive states
Differential Diagnosis
•Toxic, metabolic, or degenerative encephalopathy
•Poor attention
Treatment
• Depends on underlying condition
Prognosis
• Depends on underlying condition
152
Fig. 8.21 Omnidirectional saccadic pursuit. When the eyes follow a moving target, they fall behind and must catch up by using saccades (indicated by dashed lines).
Pursuit Saccadic Omnidirectional
153
Section 9
Cerebral Gaze Disorders
Congenital Ocular Motor Apraxia |
156 |
Acquired Ocular Motor Apraxia (Supranuclear Gaze Palsy) |
158 |
Acute Horizontal Gaze Deviation |
160 |
Spasm of the Near Reflex |
162 |
Ping Pong Gaze |
164 |
