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The Neuro-Ophthalmology Survival Guide E-Book

The Neuro-Ophthalmology Survival Guide E-Book

Anthony Pane | Neil R. Miller | Mike Burdon

(2017)

Additional Information

Abstract

This book is a practical, symptom-based, ‘how-to’ guide to neuro-ophthalmology and acquired strabismus for all trainee and practising ophthalmologists and optometrists. Each chapter focusses on a specific clinical symptom and includes an introduction to the clinical assessment of a symptom; an examination checklist; a management flowchart to be followed; clinical diagnostic criteria checklists; and further information on the diseases that can cause the symptom with a brief discussion of appropriate management.

  • A practical and accessible handbook for the ophthalmologist or optometrist without a specialist interest in neuro-ophthalmology and acquired strabismus.
  • The text simplifies a subject which is often a cause of litigation against practising ophthalmologists and exam failure for trainees and residents.
  • The book aids diagnosis in a field in which mistakes may lead to medico-legal problems, e.g. brain tumours presenting with blurred or double vision.
  • The book leads the reader from the symptom, through a series of simple but safe flowcharts and check-lists, to the diagnosis or the tests which need to be ordered.
  • The first chapter presents 20 ‘golden rules’ to keep you and your patients out of trouble.
  • A revised text with some new illustrations brings this highly-praised book up to date.
  • New online videos demonstrate abnormal eye movements, eyelid problems and pupil abnormalities.

Table of Contents

Section Title Page Action Price
Front Cover cover
Inside Front Cover ifc1
The Neuro-ophthalmology Survival Guide i
Copyright Page iv
Table Of Contents v
Video contents vi
Preface ix
Index of key management flowcharts xi
Index of key clinical diagnostic criteria xii
Introduction 1
1. You can’t avoid neuro-ophthalmology! 1
2. Neuro-ophthalmology is special! 1
3. You want your patients to see well! 1
4. You want your patients to stay healthy! 1
5. You want to stay out of trouble! 2
6. You want to pass your exam (if you still have it ahead of you) 2
How can this book help? 2
1 Staying out of trouble 5
Contents 5
Twenty neuro “rules” to keep you out of trouble 5
Patient presentation 6
Examination 7
Blurred vision or field loss 8
Bilateral disc swelling 15
Double vision 17
Giant cell arteritis (GCA) 22
Neuro-imaging 23
Ophthalmic emergencies 24
Three common mistakes 25
2 Blurred vision or field loss 27
Contents 27
Introduction 28
Eye disease 28
Optic nerve disease 28
Optic chiasmal disease 29
Retrochiasmal disease 29
Non-organic visual loss 29
Examination checklist 29
Blurred vision or field loss 29
History 29
Examination 30
Plus: must perform some type of perimetry: 30
Management flowchart 31
Blurred vision or field loss NOT explained by visible ocular disease 31
Optic neuropathy 39
Acute optic neuropathies 40 39
Chronic optic neuropathies 68 39
Optic neuropathy that cannot be diagnosed clinically 39
Acute optic neuropathies 40
Typical optic neuritis 40
Demographic 40
Symptoms 40
Ophthalmic 40
Neurologic 40
Signs 41
Differential diagnosis 42
Investigations 42
Visual prognosis 42
Treatment for the visual loss of typical optic neuritis 42
Systemic prognosis: relationship of typical optic neuritis to multiple sclerosis 43
Treatment to delay development of MS 43
Corticosteroids: 43
Disease-modifying treatments for MS 43
Management recommendations 44
Anterior ischemic optic neuropathy (AION) 44
Causes 44
Risk factors 45
Arteritic AION 45
Non-arteritic AION 45
Symptoms 45
Signs 45
Differential diagnosis 46
Investigations 46
Distinguishing between arteritic and non-arteritic AION 46
Symptoms suggesting GCA 46
Signs suggesting GCA 46
Blood test results suggesting GCA 48
Investigation and treatment of suspected arteritic AION (AION due to GCA) 48
Referral 48
Initial treatment 48
Temporal artery biopsy 49
Further treatment and follow-up 49
Visual prognosis 50
Investigation, treatment and prognosis of non-arteritic AION 50
Investigations 50
Treatment 51
Follow-up 52
Prognosis 52
Note: “diabetic papillopathy” 52
Traumatic optic neuropathy 53
Mechanism 53
Symptoms 53
Signs 53
Investigations 53
Treatment 54
Neuromyelitis optica (NMO) 54
Demographic 55
Symptoms 55
Signs 55
Differential diagnosis 55
Investigations 55
Summary: suspect NMO if a patient with optic neuritis has: 56
Prognosis 57
Treatment 57
Anti-MOG antibody-related optic neuritis 57
Infectious optic neuritis 57
Causes 57
Symptoms 58
Signs 58
3 Swollen disc/s, normal vision 107
Contents 107
Introduction 108
Examination checklist 108
Swollen disc/s, normal vision 108
History 108
Examination 109
Plus: perform perimetry: 109
Management flowchart 110
Swollen disc/s, normal vision 110
Disc pseudo-swelling 114
Definition 114
Causes 114
Characteristics 116
Spontaneous venous pulsations (SVPs) 116
Differentiation from true disc swelling 116
Investigations to help determine if pseudo- or true swelling is present 117
True disc swelling 119
Suggested investigations for swollen disc/s with normal vision 119
1. Review history, examination and perimetry 119
2. Urgent (same-day) MRI of optic nerves and brain with contrast, plus MRV brain, or CT brain and CTV 120
3. Lumbar puncture if MRI and MRV or CT and CTV are normal 120
4. Other investigations in ALL patients 121
Blood tests 121
Chest x-ray and/or chest CT scan 122
Eye disease 122
Optic nerve disease 123
Early or mild optic neuropathy 123
Optic perineuritis 123
Blood disease 124
Blood pressure: severe hypertension 124
Blood glucose: diabetic papillopathy 124
Blood electrolyte disturbance 125
Papilledema 125
Definition 125
Causes 125
Mechanism 126
Incidence and clinical interpretation 127
Not all patients with raised intracranial pressure develop papilledema 127
The severity of disc swelling in papilledema does not necessarily correlate with the level of intracranial pressure 127
“Dead discs can’t swell” 127
Timecourse 127
Symptoms 127
Neurologic symptoms 128
Ophthalmic symptoms 128
Signs 128
Disc appearance 129
Complications 131
Neurologic 131
Ophthalmic 131
Differential diagnosis 132
Investigations 132
Treatment 132
Diseases that can cause papilledema 133
Brain tumors 133
Demographic 133
Mechanism 133
Symptoms 133
Signs 134
Investigations 134
Treatment 134
Hydrocephalus 135
Definition 135
Demographic 135
Mechanism and types 135
Symptoms 135
Signs 135
Investigations 135
Treatment 136
Meningitis 136
Causes 136
Mechanism 136
Symptoms 136
Signs 136
Investigations 136
Treatment 137
Dural venous sinus thrombosis 137
Demographic 137
Mechanism 137
Causes 137
Symptoms 138
Signs 138
Investigations 138
Treatment 138
Extracranial venous outflow obstruction 139
Mechanism 139
Causes 139
Symptoms and signs 139
Investigations 139
Treatment 139
Secondary pseudotumor cerebri syndrome (pseudotumor cerebri of known cause other than obesity) 139
Definition 139
Demographic 141
Mechanism 141
Possible causes 141
Symptoms and signs 141
Investigations 141
Treatment 142
Idiopathic intracranial hypertension (IIH; also known as primary pseudotumor cerebri syndrome) associated with obesity 142
Definition 142
Terminology 143
Demographic 143
Mechanism 144
Symptoms 144
Signs 144
Complications 144
Differential diagnosis 146
Investigations 146
Treatment 146
Observation without treatment other than weight loss 146
Weight loss 147
Medical treatment 147
Oral acetazolamide (Diamox) 147
Oral diuretics 148
Oral topiramate (Topamax) 148
Surgical treatment 149
Follow-up 150
4 Transient visual loss 151
Contents 151
Introduction 152
Monocular transient visual loss 152
Binocular transient visual loss 153
Examination checklist 153
Transient visual loss 153
History 153
Examination 154
Plus: perform perimetry: 154
Management flowchart 155
Transient visual loss 155
Monocular transient visual loss 159
Binocular visual loss mistakenly attributed to one eye 159
Eye disease 159
Optic nerve disease 160
Vascular disease 160
Giant cell arteritis (GCA) 160
Ocular ischemic syndrome 161
Symptoms 161
Signs 161
Investigations 161
Treatment 161
Embolic monocular transient visual loss (“amaurosis fugax”) 162
Causes 162
Symptoms 162
Signs 162
Investigations 162
Treatment 163
Vasospastic monocular transient visual loss (“retinal migraine”) 163
Symptoms 164
Signs 164
Investigations and treatment 164
Binocular transient visual loss 164
Monocular visual loss mistakenly attributed to both eyes 164
Bilateral simultaneous eye or optic nerve disease 164
Retrochiasmal disease 164
Seizure activity from a tumor or arteriovenous malformation (AVM) 164
Migraine 165
Mechanism 165
Symptoms 165
Signs 166
Investigations 166
Treatment 166
Vascular disease 167
Giant cell arteritis 167
Vertebrobasilar insufficiency 167
Mechanism 167
Causes 167
Symptoms 167
Signs 168
Investigations 168
Treatment 168
5 Double vision 169
Contents 169
Introduction 170
Eye disease 170
Orbit or extraocular muscle disease 171
Neuromuscular junction disease 171
Ocular motor nerve disease 171
Brain disease 171
Examination checklist 172
Diplopia 172
History 172
Examination 172
Plus: perform perimetry if: 173
Management flowchart 174
Double vision 174
Monocular diplopia 185
Causes 185
Symptoms 185
Signs 186
Investigations and treatment 186
Binocular diplopia 186
Muscle or orbit disease 186
Restrictive myopathy 186
Causes 186
Symptoms 187
Signs 187
Investigations 187
Treatment 188
Paretic myopathy 188
Causes 188
Symptoms 188
Signs 188
Investigations 188
Treatment 189
Acute orbitopathy 189
Causes 190
Symptoms 190
Signs 190
Investigations 192
Treatment 193
Neuromuscular junction disease 193
Myasthenia gravis 193
Mechanism 193
Causes 193
Symptoms 193
Diplopia 194
Ptosis 194
Systemic symptoms 194
Signs 194
Abnormal ocular motility (Figs. 5.5 and 5.6, Videos 5.3 and 5.4) 194
Ptosis (see also Chapter 10, p. 300) 196
Other ophthalmic signs of myasthenia 196
Systemic signs 197
Clinical tests 197
Sleep/rest test 197
Ice test 197
Tensilon test 197
Prostigmin test 199
Investigations 199
Natural history 200
Treatment 200
Optical treatment 200
Medical treatment 200
Surgical treatment 201
Nerve disease 201
Third nerve palsy 201
Causes 201
Symptoms 202
Signs 202
Partial third nerve palsy (Figs. 5.8 and 5.9, Videos 5.5–5.8) 204
Complete third nerve palsy (Fig. 5.10, Video 5.9) 206
“Pupil-sparing” third nerve palsy (Fig. 5.11, Video 5.10) 207
Differential diagnosis 208
Investigations 208
Treatment 208
Fourth nerve palsy 209
Causes 210
Symptoms and signs 210
Acquired unilateral fourth nerve palsy (Fig. 5.14, Video 5.11) 212
Acquired bilateral fourth nerve palsy 213
Congenital unilateral fourth nerve palsy (Fig. 5.15, Video 5.12) 213
Differential diagnosis 213
Investigations 215
Treatment 215
Sixth nerve palsy 215
Causes 216
Symptoms 216
Signs 217
Differential diagnosis 217
Investigations 220
Treatment 220
Unilateral multiple nerve palsies 221
Cavernous sinus syndrome 221
Causes 222
Symptoms 222
Signs (Video 5.14) 222
Investigations 222
Treatment 222
Orbital apex syndrome 223
Bilateral multiple nerve palsies 223
Meningitis 223
Guillain–Barré syndrome and Miller Fisher variant 224
Symptoms and signs 224
Wernicke encephalopathy 225
Brain disease 225
Internuclear ophthalmoplegia (INO) 225
Causes 225
Mechanism (Fig. 5.21) 225
Symptoms 225
Signs 226
Unilateral INO (Fig. 5.22) 226
Bilateral INO (Video 5.16) 227
Differential diagnosis 227
Investigations 227
Treatment 227
Skew deviation 227
Causes 227
Symptoms 227
Signs (Fig. 5.23, Video 5.17) 227
Differential diagnosis 228
Investigations 228
Treatment 228
Supranuclear ophthalmoplegias 228
Dorsal midbrain syndrome 229
Causes 229
Mechanism 229
Symptoms 229
Signs (Fig. 5.24 and Video 5.18) 230
Investigations 230
Horizontal gaze palsy (Videos 5.19 and 5.20) 230
Horizontal saccadic palsy 231
6 “Seeing things” 241
Contents 241
Introduction 242
Eye disease 242
Optic nerve disease 242
Brain disease 242
Examination checklist 243
“Seeing things” 243
History 243
Examination 243
Plus: perform perimetry: 244
Visual illusions (Table 6.1 and Fig. 6.1) 245
Metamorphopsia (Fig. 6.2) 246
7 Abnormal movement or orientation of the visual world 253
Contents 253
Introduction 254
Inner ear disease 254
Brain disease 254
Types of eye movement disorders causing abnormal movement of the visual world 255
Examination checklist 255
Abnormal movement of the visual world 255
History 255
Examination 256
Plus: perform perimetry if: 256
Oscillopsia 256
Definition 256
Causes 257
Acquired nystagmus 257
Definition 257
Types 257
Common types of acquired nystagmus 257
Central vestibular nystagmus 257
Convergence retraction nystagmus (Video 7.16) 258
See-saw nystagmus (Video 7.6) 259
Periodic alternating nystagmus (Video 7.7) 259
Acquired pendular nystagmus (Video 7.15) 259
Rebound nystagmus (Videos 7.18 and 7.19) 259
Voluntary nystagmus (Video 7.20) 259
Saccadic intrusions 259
Types and significance 259
Square-wave jerks (Video 7.10) 259
Macrosquare-wave jerks (Video 7.11) 260
Macrosaccadic oscillations (Video 7.12) 260
Opsoclonus (Video 7.13) 260
Ocular flutter (Video 7.14) 260
Superior oblique myokymia (Video 7.9) 260
Abnormal vestibulo-ocular reflex 260
Treatment of oscillopsia 260
Akinetopsia 261
Definition 261
Types 261
Etiology 261
Testing 261
Manifestations 261
Associated deficits 262
8 Abnormal eye movements without visual symptoms 265
Contents 265
Introduction 266
Examination checklist 266
Abnormal eye movements without visual symptoms 266
History 266
Examination 267
Plus: perform perimetry if: 267
Misaligned eyes without diplopia 267
Poor vision or childhood-onset strabismus 267
Abnormal voluntary eye movements without diplopia 268
Normal age-related loss of upgaze 268
Gaze palsy 268
Horizontal gaze palsy 268
Signs 268
9 Unequal pupils 275
Contents 275
Introduction 276
Iris disease 276
Parasympathetic chain disease (brain, third nerve, ciliary ganglion) 276
Sympathetic chain disease (brain, neck, upper chest) 276
Examination checklist 277
Unequal pupils 277
History 277
Examination 277
Plus: perform perimetry if: 278
Management flowchart 279
Unequal pupils 279
Approach to anisocoria 280
Definition 280
Etiology 280
Rule 280
Question 280
Answer 280
Explanation 280
Anisocoria with normally reactive pupils 280
Differential diagnosis 280
Physiologic anisocoria (Fig. 9.1) 280
Horner syndrome (Fig. 9.2 and Video 9.1) 281
Signs 281
Pharmacologic testing to confirm the diagnosis of Horner syndrome (Table 9.1) 281
Causes 284
Clinical clues to the location of the lesion 285
Pharmacologic testing to determine the location of the lesion 285
Investigations 286
Intermittent unilateral pupillary mydriasis (sympathetic form) (Fig. 9.7) 286
Anisocoria with one pupil that is poorly reactive or non-reactive to light 287
Differential diagnosis 287
Evaluation 287
Iris sphincter damage (traumatic mydriasis) (Fig. 9.8) 287
Pharmacologic blockade (Fig. 9.9) 288
Topical parasympatholytic agents (causing dilated pupil/s) 288
Topical parasympathomimetic agents (causing constricted pupil/s) 289
Tonic pupil (Fig. 9.10 and Video 9.2) 289
Signs 289
Etiology 289
Causes 290
Pharmacologic testing for tonic pupil (Fig. 9.10) 291
Third nerve palsy (Fig. 9.11) 291
Intermittent unilateral pupillary mydriasis (parasympathetic form) (Fig. 9.12) 292
10 Ptosis 293
Contents 293
Introduction 294
Examination checklist 294
Ptosis 294
History 294
Examination 295
Management flowchart 297
Aponeurotic ptosis 298
Cause 298
Demographic 298
Symptoms 298
Signs 298
Investigations 298
Treatment 298
Caution 298
Ptosis due to levator myopathy 299
Causes 299
Demographic 299
Symptoms 299
Signs 299
Investigations 299
Treatment 299
Ptosis due to myasthenia gravis 300
Demographic 300
Symptoms and signs 300
Investigations and treatment 301
Ptosis due to partial third nerve palsy 301
Caution 301
Causes 301
Demographic 301
Symptoms and signs 301
Investigations 302
Treatment 302
Ptosis due to Horner syndrome 303
Causes 303
Demographic 303
Symptoms and signs 303
Pharmacologic testing 303
Investigations 304
Treatment 304
11 Facial weakness or spasm 305
Contents 305
Introduction 306
Facial weakness 306
Involuntary facial movements 306
Examination checklist 307
Facial weakness or spasm 307
History 307
Examination 307
Facial weakness 308
Facial nerve palsy 308
Causes 308
Symptoms and signs (Figs. 11.1 and 11.2, Video 11.1) 308
Ophthalmic 308
Other symptoms of facial nerve dysfunction 308
Possible localizing symptoms and signs 309
Differential diagnosis 311
Investigations 311
Ophthalmic complications 311
Ophthalmic treatment 311
Involuntary facial movements 312
Orbicularis oculi myokymia 312
12 Unexplained eye pain, orbital pain or headache 317
Contents 317
Introduction 318
Examination checklist 318
Unexplained eye pain or headache 318
History 318
Examination 319
Ophthalmic causes of headache or facial pain 319
Headache due to angle-closure glaucoma 320
Headache due to herpes zoster ophthalmicus 320
Headache due to refractive error 320
Headache due to heterophoria or heterotropia 320
Ophthalmic symptoms and signs of an intracranial or systemic cause of headache or facial pain 320
Giant cell arteritis (GCA) 321
Raised intracranial pressure 321
Pituitary apoplexy 322
Internal carotid artery (ICA) dissection 322
Causes 322
Symptoms and signs 322
Investigations 322
Treatment 322
Trigeminal neuralgia 322
Causes (Fig. 12.1) 322
Symptoms 323
Signs 323
Investigations 323
Treatment 323
Common “benign” headache patterns with ophthalmic features 324
Migraine 324
Trigeminal autonomic cephalalgias (TACs) 325
When to refer other patients for further investigation 325
13 Neuro-ophthalmic history and examination 327
Contents 327
Introduction 328
History 328
Patient Details 328
Presenting complaint 328
Other ophthalmic symptoms 329
Ophthalmic history 329
Medical history 329
Medications 329
Family history 329
Social and occupational history 329
Symptoms of giant cell arteritis (GCA) 330
Systems review questions 330
General 330
Neurologic 330
Ear, nose, throat 330
Respiratory 330
Cardiovascular 331
Gastrointestinal 331
Genitourinary 331
Musculoskeletal 331
Skin 331
Infectious risk factors 331
Examination 331
Visual acuity (VA) 333
Color vision (Video 13.1) 333
Subjective color desaturation (Fig. 13.1) 333
Color plate tests (e.g. Ishihara or Hardy-Rand-Rittler pseudoisochromatic color plates) 333
Visual fields to confrontation (Fig. 13.2 and Video 13.2) 334
Descriptive 335
Finger counting in quadrants 335
Red target for central field 335
Red target for hemianopic defect 335
Perimetry 336
Fixation, alignment and eye movements (Video 13.3) 336
Suggested reading 365
Appendix: Checklists 367
Neuro-ophthalmic history checklist 367
Neuro-ophthalmic examination checklist 367
Index 369
A 369
B 371
C 371
D 373
E 374
F 375
G 376
H 377
I 378
J 379
K 379
L 379
M 380
N 382
O 383
P 385
Q 387
R 387
S 388
T 389
U 391
V 391
W 392
Z 392