- •Contents
- •Foreword
- •Preface
- •The Diagnosis of Optic Neuropathies
- •Optic Neuritis
- •Optic Disc Edema with a Macular Star and Neuroretinitis
- •Nonarteritic Ischemic Optic Neuropathy
- •Traumatic Optic Neuropathy
- •Papilledema
- •Transient Visual Loss
- •Visual Field Defects
- •Diplopia
- •Third Nerve Palsies
- •Fourth Nerve Palsies
- •Sixth Nerve Palsies
- •Ocular Myasthenia Gravis
- •Ptosis
- •Lid Retraction and Lid Lag
- •Index
r Index r
Page numbers in italic indicate that the entry on that page is in a figure or table.
Abducens nerve paresis. See also Sixth nerve palsies (SNP)
divergence insufficiency/paralysis, 229–230
localization, 297
one-and-a-half syndrome, 320–321 Abducens nucleus, anatomy,
312, 314
Aberrant regeneration, third nerve palsy (TNP), 270–271
Abetalipoproteinemia, horizontal gaze palsy, 314–315
Acetazolamide, idiopathic pseudotumor cerebri therapy, 146, 147
Adie’s tonic pupil syndrome clinical features, 441, 444 etiology, 441 neuroimaging studies, 441 therapeutic strategies, 444
Adrenocorticotropic hormone (ACTH), optic neuritis (ON) therapy, 47–49, 48
Alcohol amblyopia, nutritional optic neuropathy, clinical features, 9, 12–14, 17
Alexia, homonymous hemianopsia, optic radiations, 202
Alzheimer’s disease, homonymous hemianopsia, 206
Aneurysm
acquired isolated third nerve palsies (TNP)
neuroimaging protocols, 268–270 normal sphincter
complete extraocular muscle palsy (type 4A TNP), 264–265
incomplete extraocular muscle palsy (type 4B TNP), 266
acquired sixth nerve palsy (SNP), 304 third nerve palsies (TNP),
subarachnoid lesion, 260 Angle-closure glaucoma, horizontal
diplopia, 223–226 Anisocoria
Adie’s syndrome, 441, 444, 444 evaluation protocol, 456, 456 Horner’s syndrome, 445, 445–456
alternating Horner’s syndrome, 448 central Horner’s syndrome, 446, 447 congenital Horner’s syndrome, 449 imaging and evaluation protocols,
455
isolated Horner’s syndrome, 446 isolated postganglionic Horner’s syndrome, 455–456
pediatric patients, 454–455 pharmacologic localization, 452–454,
453
postganglionic Horner’s syndrome, 446–447, 449–451
preganglionic (intermediate) Horner’s syndrome, 446, 448
trauma etiology, 449, 451 intermittent/transient pupillary
phenomenon, 435, 439 iris structural abnormalities, 439,
439–440 isolated etiology, 445
464
isolated anisocoria, clinical evaluation, 445
light-near dissociation, 434, 434 syphilis serology evaluation, 444–445
light reaction, 433 normal pupil, 445
pharmacologic mydriasis/miosis, 435,
437–439
third nerve palsies and, 434–435 tonic pupil, 440–441, 440–443
Anterior choroidal artery occlusion, homonymous hemianopsia, lateral geniculate body lesions, 200
Anterior ischemic optic neuropathy (AION)
associated conditions, 75, 76–78 clinical presentation, 3
atypical features, 75, 78–79, 79 differential diagnosis, 2 evaluation and treatment, 79–80 future research issues, 80–81 giant cell arteritis (GCA)
clinical features, 93, 94
atypical features, 98–99, 100–101 corticosteroid therapy, 107–108 diagnostic criteria, 94, 94–96, 96–98,
98–99
ESR elevation, 99, 105 ESR normal values, 99 evaluation flowchart, 109
hematologic testing, 99, 101, 102 laboratory abnormalities, 99, 102, 105 patient evaluation protocols, 104–105,
105
TAB procedures, 101–104 therapeutic regimen, 106–110
oral vs. IV corticosteroids, 106–108 steroid complications, 108, 110
nonarteritic ischemic optic neuropathy, clinical features, 73–75, 74–75
Anterior ischemic optic neuropathy of the young (AIONY), clinical features, 75
Anterior knee, visual field defects, junctional characteristics, 192–194
Antiacetylcholine receptor antibodies myasthenia gravis (MG) diagnosis, 341
Index 465
ocular myasthenia gravis (OM) diagnosis, 341–342
Anticholinesterase agents, ocular myasthenia gravis (OM) management, 343–344
Aponeurotic ptosis etiology, 415, 415
evaluation protocol, 416, 417 Apraxia of eyelid opening, ptosis
etiology, 410, 411
Argyll Robertson pupil, syphilis serology testing, 444–445
Arnold-Chiari malformation, seesaw nystagmus,
374
Arteriorvenous malformations (AVMs), binocular transient visual loss, 180–184
Aspirin therapy, nonarteritic anterior ischemic optic neuropathy (NA-AION), 81–82
Asymmetric binocular eye oscillations, nystagmus etiology, 368, 370–372, 373
Ataxia, periodic alternating esotropia (PAE), 228
Atherosclerotic disease, monocular TVL with, 171–172
clinical features, 173–179 Atypical optic neuritis, clinical
presentation, 38, 39 Atypical/unexplained optic neuropathy,
14, 22
Basilar aneurysms, anisocoria etiology, 435
Bielschowsky’s test, fourth nerve palsies (FNP), 281
Binasal hemianopsia, visual field defects, 196, 198
Binocular diplopia evaluation protocol, 217
horizontal (esotropia/exotropia), etiology, 219–220,
221–222 vertical diplopia
clinical evaluation, 233–234 etiology, 234, 235
466 Clinical Pathways in Neuro-Ophthalmology, second edition
Binocular symmetric conjugate eye oscillations
etiology, 377–382 evaluation, 376
Binocular symmetric jerk nystagmus eccentric gaze/maneuver induction,
389–391, 392 etiology, 382–385
Binocular transient visual loss clinical signs, 179–183
evaluation protocols, 169–170, 183 Biopsy, horizontal diplopia, 224–226 Bitemporal hemianopsia, visual field
defects, 194, 195–196, 196, 197 Blepharospasm, ptosis, 409–410
Blood pressure monitoring, papilledema evaluation, 133, 135, 135
Botulinum toxin therapy congenital nystagmus, 378 Graves’ ophthalmopathy (GO)
lid retraction, 356 strabismus, 356–357
pendular nystagmus, 381–382 Botulism, vertical diplopia, 238–239 Branstem structures, lid retraction/lid
lag and, 421
Brown’s superior oblique tendon sheath syndrome, vertical diplopia, 241–242
B-scan echography, papilledema differential diagnosis, 130
Calcific emboli, monocular TVL, 173 Carbonic anhydrase inhibitors,
idiopathic pseudotumor cerebri therapy, 146, 147
Carotid artery dissection
Horner’s syndrome, clinical evaluation and etiology, 447, 449
monocular TVL with, 173–179 Carotid endarterectomy, transient visual
loss risk and, 177–178 Cataract surgery, vertical diplopia,
243–244
Cat-scratch disease, neuroretinitis and optic disc edema with macular star (ODEMS), 65–66, 67
Cavernous sinus lesion
fourth nerve palsy (FNP), 283 sixth nerve palsies (SNP), 297, 301 third nerve palsies (TNP), 260–261
Central caudal nucleus (CCN), lid retraction/lid lag, 421
Central disorders convergence spasm, 230–231 horizontal diplopia, 227–228
Central Horner’s syndrome, clinical evaluation, 446, 447
Central retinal artery occlusion (CRAO), giant cell arteritis (GCA)
clinical diagnosis, 95–96, 96–98, 98–99 corticosteroid therapy, 107–108
Central retinal venous occlusion (CRVO), venous stasis retinopathy, differential diagnosis, 171–179
Central venous thrombosis (CVT), pseudotumor cerebri syndrome, 136–137
Cerebral blindness, visual field defects, homonymous hemianopsia, 204–205
Cerebral infarction cerebral polyopia, 217
homonymous hemianopsia, 206 Cerebral ischemia, transient visual loss,
176–179
Cerebral polyopia, monocular diplopia, differential diagnosis, 214, 217
Cerebral structural lesions, binocular transient visual loss, 180
Cerebrospinal fluid (CSF) optic neuritis (ON)
lumbar punctures, 46 neuroimaging studies, 44–46
papilledema, clinical evaluation, 135 pseudotumor cerebri syndrome,
136–137 lumboperitoneal shunt (LSP)
complications and, 148–155,
149
Chiasmal syndromes, visual field defects, 194, 195–196, 196, 197
Childhood strabismus syndromes, esotropia/exotropia etiology, 220, 222, 223
Cholesterol emboli, monocular TVL, 172
Chronic progressive external ophthalmoplegia (CPEO), ptosis etiology, 412–413,
414
Claude’s syndrome, third nerve palsies (TNP), fascicular lesion, 258–259
Cocaine, pharmacological localization of Horner’s syndrome, 452–454,
453
Comatose patients, spontaneous eye oscillations, 396–399,
397–398
Compressive optic neuropathy (CON) clinical presentation, 3, 5, 5–12 Graves’ ophthalmopathy (GO)
imaging findings, 349–350 therapeutic strategies, 353, 357–360,
358–359
Computed tomography (CT) acquired isolated third nerve palsies
(TNP), neuroimaging protocols, 268–270
gaze-evoked transient visual loss (TVL), 170–171
Graves’ ophthalmopathy (GO), 349 horizontal gaze palsies, 312 internuclear ophthalmoplegia (INO),
319
isolated fourth nerve palsies (TNP), 289–290, 290–292
myasthenia gravis (MG) diagnosis, 342 optic neuritis, 44–46
papilledema
clinical evaluation, 133, 135, 135 differential diagnosis, 130
third nerve palsies (TNP), retrospective studies, 271–272
Congenital Horner’s syndrome, evaluation and etiology, 449
Congenital nystagmus, clinical features and classification, 377–378
Congenital ptosis, etiology, 410, 411, 412 Congenital syndromes
fourth nerve palsies (FNP), 287 sixth nerve palsy (SNP), evaluation
protocols, 301 vertical diplopia, 239–245
Consecutive esotropia, etiology, 222
Index 467
Contralateral eyelid retraction, third nerve palsies (TNP), fascicular lesion, 259
Contralesionally beating torsional nystagmus, clinical features and etiology, 385
Convergence insufficiency/paralysis binocular symmetric jerk nystagmus,
390–391
clinical features and etiology, 231 downbeat nystagmus, 385–389, 387 horizontal dysconjugate eye
oscillations, 375,
376
Convergence-retraction nystagmus, horizontal dysconjugate eye oscillations, 375, 376
Convergence spasm, clinical features and etiology, 230–231
Cortical blindness, visual field defects, homonymous hemianopsia, 204–205
Corticosteroids
giant cell arteritis (GCA) complications, 108–110, 111 oral vs. IV therapy, 106–108 treatment protocols, 106
Graves’ ophthalmopathy (GO), 352–353
idiopathic pseudotumor cerebri therapy, 146, 147
nonarteritic anterior ischemic optic neuropathy (NA-AION), 80
ocular myasthenia gravis (OM) management, 344
prognosis, 344–345
optic neuritis (ON) therapy, 47–49, 48 traumatic optic neuropathy (TON)
management, 121–123 Cranial nerve impairment
fourth nerve palsies (FNP) cavernous sinus lesion, 283–284 classifications, 284, 286
clinical features, 281–283, 284 congenital origin, 287 diagnostic criteria, 282–283, 283 etiologies, 283, 285–286 intracranial lesions, 289–290 midbrain lesions, 283
468 Clinical Pathways in Neuro-Ophthalmology, second edition
Cranial nerve impairment (cont.) nonvasculopathic, evaluation
protocols, 287–288, 288–291, 290–292
orbital lesions, 284 subarachnoid space lesion, 283 topographic anatomy, 281 trauma, 287
vasculopathic etiology, 287 horizontal diplopia, 226–227 sixth nerve palsies (SNP)
anatomy, 295, 296–297
cavernous sinus lesion, 298–300, 301 evaluation guidelines, 301–304, 303 localization and classification,
298–300
nonisolated SNP, pontine (lower pons) lesion, 295–296, 298–300
orbital lesion, 301 petrous apex lesion, 297
subarachnoid space lesion, 297, 297 third nerve palsies (TNP)
aberrant regeneration (type 6), 270–271
cavernous sinus lesion, 260–261 classification, 253, 254
clinical features, 253 congenital origins, 262 fascicular lesion, 258–259 isolated acquired TNP
neuroimaging protocols, 268–270 nontraumatic, 262
normal pupillary sphincter, extraocular muscle incomplete palsy (type 4B), 265–266
normal pupillary sphincter, extraocular muscle palsy (type 4A), 264–265
subnormal pupillary sphincter dysfunction, extraocular muscle palsies (type 4C), 267
localization etiologies, 253, 256–257 nonisolated TNP, clinical evaluation,
261–262, 263 nuclear lesion, 254–255 orbital lesion, 261
progressive/unresolved (type 5 TNP), 270
retrospective review, 271–272
subarachnoid lesion, 259–260 trauma, 262
vertical diplopia, 237–238 C-reactive protein, giant cell arteritis
(GCA), diagnosis, 99, 101, 102 ‘‘Crowded’’ hyperoptic disks,
papilledema, differential diagnosis, 131, 131
Cyclic esotropia, clinical features, 228 Cyclodeviation, vertical diplopia,
233–234
Cyclosporin, Graves’ ophthalmopathy (GO) therapy, 353
Dandy criteria, pseudotumor cerebri syndrome, 135
Decompensation syndromes esotropia/exotropia etiology, 220, 222,
223
fourth nerve palsies (FNP), 282 vertical diplopia, 239
Decompressions, Graves’ ophthalmopathy (GO), 358–359, 359–361
Deep sclerotomy with collagen implant (DSCI), transient visual loss, 175
Dental anesthesia, third nerve palsies (TNP), orbital lesion, 261
Devic’s disease, clinical features, 39, 42 Diabetes
acquired isolated third nerve palsies (TNP), normal sphincter, complete extraocular muscle palsy (type 4A TNP), 264–265
papillopathy, anterior ischemic optic neuropathy (AIONY) and, 75,
75
Digoxin, idiopathic pseudotumor cerebri therapy, 147
Diplopia
acquired motor fusion deficiency, 231–232
binocular diplopia, 217
horizontal (esotropia/exotropia), etiology, 219–220, 221–222
childhood strabismus syndromes, esotropia/exotropia, 220, 222,
223
convergence insufficiency and paralysis, 231
convergence spasm, 230–231 cyclic esotropia, 228
divergence insufficiency and paralysis, 228–230
evaluation protocol, 215, 216 foveal displacement syndrome, 245 Graves’ ophthalmopathy (GO), 359,
361
hemifield slide phenomenon, 232 horizontal diplopia
central nerve disorders, 227–228 cranial nerve disorders, 226–227 extraocular muscle disorders,
223–226, 225–226 monocular diplopia, etiology and
evaluation, 215, 217, 217 mysathenia gravis, 218
ocular motor cranial neuropathy, 218 ocular myasthenia gravis (OM),
management of, 342–344 periodic alternating esotropia, 228 phorias and tropias, functional
assessment,
219
restrictive ophthalmoplegia with orbital disease, 218
sensory esotropia/exotropia, 222 supranuclear ophthalmoplegia, 219 thyroid eye disease and, 218–219 transient vs. persistent etiologies, 217,
218 vertical diplopia
cranial nerve impairment, 237–238 etiology, 234, 235–236 examination protocol, 232–234
extraocular muscle disease, 238–240 mechanical misalignment processes,
240–245
neuromuscular junction diseases, 238
supranuclear processes, 234, 236–237 vertical/horizontal deviation, 219 vitreous hemorrhage, secondary
exotropia, 232 Divergence insufficiency/paralysis
esotropia, 228–230 neurologic disease, 229–230
Index 469
Divergence nystagmus, horizontal dysconjugate eye oscillations, 375, 376
Doll’s-head maneuver, diplopia evaluation, supranuclear process, 219
Dominant optic atrophy, clinical features, 19, 20
Dorsal mesencephalic supranuclear lesions, lid retraction/lid lag, 422
Dorsal mid-brain syndrome, vertical gaze palsy, 322, 323
Double elevator palsy clinical features, 327–328
vertical diplopia, 234, 236–237 congenital conditions, 240 Double Maddox rod test, vertical
diplopia, 233–234 cranial nerve impairment, 238
Downbeat nystagmus, clinical features and etiology, 370, 385–389, 387
Drug-induced nystagmus, clinical features, 384
Duane’s retraction syndrome, esotropia/ exotropia etiology, 220, 222,
223
Duction movements diplopia assessment, 219
fourth nerve palsies (FNP), clinical evaluation, 281
Dysconjugate bilateral symmetric eye oscillations, etiology, 372
Eccentric gaze, binocular symmetric jerk nystagmus, 389–391, 392
Electrographic status epilepticus, stuporous/comatose patients, 398–399
Electromyography
apraxia of eyelid opening, 410 myasthenia gravis (MG) diagnosis, 341
Electrophysiologic testing, myasthenia gravis (MG) diagnosis, 341
Enophthalmos, lid retraction/lid lag, 425 ‘‘Entomopia,’’ monocular diplopia, 214,
217
Epileptic nystagmus, clinical features, 384–385
470 Clinical Pathways in Neuro-Ophthalmology, second edition
Erythrocyte sedimentation rate (ESR), giant cell arteritis (GCA)
clinical diagnosis, 94, 94–96, 98–99 corticosteroid therapy, 107–108 elevation patterns, 99
marked elevation, evaluation protocols, 105, 105
normal values, 99 Esotropia (ET)
cyclic esotropia, 228
divergence insufficiency/paralysis, 228–230
etiology, 219–220, 221–223 childhood strabismus syndromes,
220, 222, 223 periodic alternating esotropia
(PAE), 228 sensory esotropia, 222
thyroid eye disease, 224–226 Ethambutol exposure, toxic optic
neuropathy, clinical features, 10–12
Exophthalmos, gaze-evoked transient visual loss (TVL), 170–171
Exotropia (XT)
cranial nerve lesions, 227 etiology, 219–220, 221–223
childhood strabismus syndromes, 220, 222, 223
isolated medial rectus paresis, 226 orbital trauma, 226
secondary exotropia, vitrous hemorrhage, 232
sensory exotropia, 222
vitreous hemorrhage, 227–228, 232 Extraocular muscular disorders
acquired isolated third nerve palsies (TNP)
normal sphincter, complete extraocular muscle palsy (type 4A TNP), 264–265
normal sphincter, incomplete extraocular muscle palsy (type 4B TNP), 265–266
subnormal pupillary sphincter dysfunction, extraocular muscle palsies (type 4C TNP), 267
Graves’ ophthalmopathy (GO), radiotherapy, 355–356
Graves’ ophthalmopathy (GO) and, therapeutic strategies, 351, 353
horizontal diplopia, 223–226 myasthemia gravis (MG) vs. ocular
myasthenia gravis (OMG), differential diagnosis, 338–341
vertical diplopia, 238–239
Eyelid nystagmus, etiology, 423–424 Eyelids. See also Lid retraction/lid lag
anatomy, 421
Facial nerve palsy, one-and-a-half syndrome, 319–320
Fallen eye syndrome, vertical diplopia, 242
Fascicular lesion, third nerve palsies (TNP), 258–259
Fisher’s syndrome, vertical diplopia, 238 Fluid-attenuated inversion recovery
(FLAIR) imaging, internuclear ophthalmoplegia (INO), 319
Foster Kennedy syndrome, pseudotumor cerebri syndrome, differential diagnosis, 143–144
Fourth nerve palsies (FNP)
acquired isolated fourth nerve palsies (FNP), evaluation protocols, 288, 288–292
cavernous sinus lesion, 283–284 classifications, 284, 286
clinical features, 281–283, 284 congenital origin, 287 diagnostic criteria, 282–283, 283 etiologies, 283, 285–286 intracranial lesions, 289–290
localization and classification, 284,
285–286 midbrain lesions, 283
nonvasculopathic, evaluation protocols, 287–288, 288–291, 290–292
orbital lesions, 284 subarachnoid space lesion, 283 topographic anatomy, 281 trauma, 287
vasculopathic etiology, 287
Foveal displacement syndrome, binocular diplopia, 245
Frisen papilledema grading scale, 131,
134
Fusion procedures, psoria/tropia management, 219
GABA agonists, vertical nystagmus management, 388–389
Gabapentin, pendular nystagmus therapy, 381–382
Gaze disorders
abducens nucleus anatomy, 312, 314 horizontal conjugate gaze, anatomy,
311 horizontal gaze palsies
clinical evaluation, 312, 315
lesion localization and classification, 311, 312–314
internuclear ophthalmoplegia (INO) clinical features, 314–315, 316 etiology, 316, 317–318
evaluation protocols, 318–319 medial longitudinal fasciculus (MLF)
anatomy, 312, 314 one-and-a-half syndrome
clinical evaluation, 319–321, 321 etiologies, 321, 321
ping-pong gaze, stuporous/comatose patients, 397–399
short-cycle periodic alternating gaze, stuporous/comatose patients, 397–399
skew deviation, clinical features, 327–328
supranuclear monocular elevation paresis, clinical features, 327–328
vertical gaze impairment anatomy, 321
etiology, 322, 324–325
evaluation protocols, 323, 325–326,
326
lesion localization of palsies, 321–322,
322–323
vertical one-and-a-half syndrome, clinical features, 327–328
‘‘Gaze-evoked’’ nystagmus, defined, 367
Index 471
Gaze-evoked nystagmus, etiology, 389–391
Gaze-evoked transient visual loss (TVL), evaluation of, 169, 170–171
Giant cell arteritis (GCA)
binocular transient visual loss, 182–183 clinical features, 93, 94
atypical features, 98–99, 100–101 diagnostic suspicion, 94, 94–96, 96–98,
98–99
ESR elevation, 99, 105 ESR normal values, 99 evaluation flowchart, 109
hematologic testing, 99, 101, 102 laboratory abnormalities, 99, 102, 105 low clinical suspicion, evaluation
criteria, 105, 105
moderate clinical suspicion, evaluation criteria, 104–105
patient evaluation protocols, 104–105,
105
TAB procedures, 101–104 therapeutic regimen, 106–110
complications, 108–110, 111
oral vs. IV corticosteroids, 106–108 steroid complications, 108, 110
transient visual loss, 175
Giant cell myocarditis, horizontal diplopia, 224–226
Glaucoma, optic disc cupping, optic neuropathy, differential diagnosis, 2
Gliomas, anterior visual pathway, optic neuropathy, 5, 9
Glucocorticoids, Graves’ ophthalmopathy (GO) therapy, 354–356, 355
Graves’ ophthalmopathy (GO) clinical features, 349, 350 decompression studies, 358–359 evaluation and treatment flowchart,
360
imaging studies, 349–350 immunosuppressive therapy, 352–354 lid retraction, treatment protocols, 356 lid retraction/lid lag, 424–425
ocular and orbital inflammation, 352 proptosis and/or compressive optic neuropathy, 357, 359
472 Clinical Pathways in Neuro-Ophthalmology, second edition
Graves’ ophthalmopathy (GO) (cont.) proptosis without optic neuropathy,
359, 361 radiotherapy, 354–356
strabismus management, 356–357 systemic thyroid status, 350 therapeutic management, 350–352,
355
vertical diplopia, 241 Guillain-Barre´ syndrome, vertical
diplopia, 238 Guillain-Mollaret triangle lesions,
pendular nystagmus, 382
‘‘Half-moon syndrome,’’ visual field defects, monocular temporal crescent, 192
Hematologic testing, giant cell arteritis (GCA), 99, 101, 102
Hemianopic anosognosia, visual field defects, occipital lesions, 203–205
Hemifacial spasm, ptosis, 409–410 Hemifield slide phenomenon
binocular diplopia, 245 clinical features, 232
Hereditary optic neuropathy, clinical features, 19, 20–22, 22
Hering’s law, pseudo-overaction, fourth nerve palsies (FNP), 282
1H-magnetic resonance spectroscopy, Graves’ ophthalmopathy (GO), 350
Homonymous hemianopsia, visual field defects, 198
lateral geniculate body lesion, 199–200 normal neuroimaging, 205–207 occipital lesions, 202–205
optic radiation, 201–202 optic tract lesions, 198–199 therapeutic management, 207
Horizontal diplopia
binocular horizontal diplopia, etiology, 219–220, 221–222
central nerve disorders, 227–228 cranial nerve disorders, 226–227 extraocular muscle disorders, 223–226,
225–226
Horizontal dysconjugate eye oscillations, evaluation and etiology, 375,
376
Horizontal gaze palsies clinical evaluation, 312, 315
lesion localization and classification, 311, 312–314
one-and-a-half syndrome, 320–321 Horizontal nystagmus, etiology,
382–383
Horizontal pendular nystagmus, diagnosis and etiology, 380
Horizontal smooth pursuit defects, lesion localization, 311, 312–314
Horner’s syndrome anisocoria, 445, 445–456
alternating Horner’s syndrome, 448
central Horner’s syndrome, 446, 447 congenital Horner’s syndrome, 449 imaging and evaluation protocols,
455
isolated Horner’s syndrome, 446 isolated postganglionic Horner’s syndrome, 455–456
pediatric patients, 454–455 pharmacologic localization, 452–454,
453
postganglionic Horner’s syndrome, 446–447, 449–451
preganglionic (intermediate) Horner’s syndrome, 446, 448
trauma etiology, 449, 451
fourth nerve palsy (FNP), cavernous sinus lesion, 283
monocular TVL with, 173–179 ptosis etiology, 413, 414, 415
Hydrocephalus, periodic alternating esotropia (PAE), 228
Hydroxyamphetamines, pharmacological localization of Horner’s syndrome, 452–454, 453
Hyperdeviation, vertical diplopia, congenital conditions, 239–240
Hyperglobus, ptosis etiology, 410 Hyperthyroidism, Graves’
ophthalmopathy (GO), therapeutic strategies, 351
Hypertropia
fourth nerve palsies (FNP), 281–283,
284 ptosis etiology, 410
thyroid eye disease, 224–226 Hypoglobus, lid retraction/lid lag, 425
Ice-pack test, myasthenia gravis (MG) diagnosis, 340–341
Immunoglobulin therapy Graves’ ophthalmopathy (GO),
353–354 optic neuritis (ON), 49
Immunosuppressive therapy Graves’ ophthalmopathy (GO),
352–353
saccadic intrusions, classification and etiology, 395
Infectious disease
optical disc edema with macular star (ODEMA)
clinical features, 64
differential diagnosis, 64–66, 67 saccadic intrusions, classification and
etiology, 393–395
Inferior rectus fibrosis syndrome, vertical diplopia, 243
Inferior rectus paresis, isolated, third nerve palsies (TNP), 254–255
Infiltrative optic neuropathy, clinical features, 5–6, 8, 13–14
Inflammatory optic neuropathy, clinical features, 56, 8, 13–14
‘‘Insect eye,’’ monocular diplopia, 214, 217
Interferon therapy, optic neuritis (ON), 49
Intermittent angle closure glaucoma monocular transient visual loss,
175–176 reading-evoked TVL, 171
Intermittent LP inhibition (ILPI), apraxia of eyelid opening, 410
Internal carotid artery (ICA), atherosclerotic disease, monocular TVL with, 173–179
Internuclear ophthalmoplegia (INO) clinical features, 314–315, 316 etiology, 316, 317–318, 318
Index 473
evaluation protocols, 318–319, 320 horizontal gaze palsy, 314 nystagmus etiology, 370
Internuclear ophthalmoplegia (INO) of abduction, 315
Intracranial lesions, isolated fourth nerve palsies (TNP), 289–290, 290291
Intracranial pressure
papilledema evaluation, 132–133, 135,
135
pseudotumor cerebri syndrome, 136–137
lumboperitoneal shunt (LSP) complications and, 148–155,
149
vertical diplopia, 237–238 Ipsilateral ptosis, third nerve palsies
(TNP), fascicular lesion, 259 Iris abnormalities, structural
abnormalities, anisocoria, 439,
439–440
Jakob-Creutzfeldt disease, Heidenhain variant, homonymous hemianopsia, 205–206
Junctional scotoma, visual field defects, 193–194
Kearns-Sayre syndrome, ptosis etiology, 412–413, 414
Kjer’s hereditary optic neuropathy, clinical features, 19, 20
Latent nystagmus, diagnosis and management, 378–379
Lateral geniculate body lesions, homonymous hemianopsia, 199–200
Leber’s hereditary optic neuropathy (LHON), clinical features, 19,
20–21, 22
Levator palpebrae superioris (LPS)
lid retraction/lid lag and, 421, 423–424 vertical diplopia and spasm of, 236
Levodopa, nonarteritic anterior ischemic optic neuropathy (NA-AION) therapy, 82–83
Lewy body disease, homonymous hemianopsia, 206
474 Clinical Pathways in Neuro-Ophthalmology, second edition
Lid retraction/lid lag evaluation protocol, 427, 428 eyelid anatomy, 421
Graves’ ophthalmopathy (GO), management options, 356
lower eyelid retraction, etiology, 428–429, 429
miscellaneous etiologies, 425, 426–427, 427
neurogenic etiology, 422–424 neuromuscular/myopathic etiology,
424–425 Light-near dissociation
anisocoria, etiology, 434, 434 pupillary abnormalities, syphilis
serology testing, 444–445 Light reaction, anisocoria, 433
normal pupil, 445
Locked-in syndrome, clinical evaluation, 327–328
Lower eyelid retraction, etiologies, 428–429, 429
Lumbar punctures
optic neuritis (ON), indications for, 46 papilledema, clinical evaluation, 135 progressive/unresolved sixth nerve
palsy (SNP), 304 pseudotumor cerebri therapy, 147
Lumboperitoneal shunt (LPS), pseudotumor cerebri therapy, 148–155, 149
Lutz posterior INO. See INO of abduction
Lyme disease, neuroretinitis and optic disc edema with macular star (ODEMS), 66
Macro-square-wave jerks, saccadic intrusions, classification and etiology, 393–395
Magnetic resonance imaging (MRI) acquired isolated third nerve palsies
(TNP), neuroimaging protocols, 268–270
gaze-evoked transient visual loss (TVL), 170–171
Graves’ ophthalmopathy (GO), 349 horizontal gaze palsies, 312 Horner’s syndrome evaluation, 455
internuclear ophthalmoplegia (INO), 319
isolated fourth nerve palsies (TNP), 289–290, 290–292
monocular TVL assessment, 172 nonarteritic anterior ischemic optic
neuropathy (NA-AION), 80–81
optic neuritis, 44–46
multiple sclerosis risk assessment, 51–53
papilledema, clinical evaluation, 133, 135,
135
pseudotumor cerebri syndrome, 136–137
third nerve palsies (TNP), retrospective studies, 271–272
transient visual loss assessment, 178–179
vertical gaze palsy, 325–326 Medial longitudinal fasciculus (MLF)
anatomy, 312, 314
lid retraction/lid lag etiology, 422–424 one-and-a-half syndrome, 319–321
Medial rectus paresis, isolated exotropia (XT), 226
third nerve palsies (TNP), 254–255 Meningiomas
compressive optic neuropathy, clinical features, 5, 67
monocular TVL with, 171–172 Meso-diencephalon disorders, vertical
gaze palsy, 322 Methylprednisolone
giant cell arteritis (GCA) therapy, 106–108
optic neuritis (ON) therapy, 47–49, 48 traumatic optic neuropathy (TON),
122–123
Meyer’s loop, homonymous hemianopsia, lateral geniculate body lesions, 199–200
Midbrain lesion
fourth nerve palsy (FNP), 283 ptosis etiology, 413, 414, 415
Migraines
binocular transient visual loss, 179–184
monocular TVL with, 171–172 third nerve palsies (TNP),
subarachnoid lesion, 256–257, 259260
transient visual loss, vasospasm, 175–176
vertical diplopia, 237–238
Miller Fisher syndrome, one-and-a-half syndrome, 320–321
Morbidity
idiopathic pseudotumor cerebri, weight fluctuation and therapy for, 145–147, 146
pseudotumor cerebri syndrome, visual field loss, 144
Motor fusion deficiency, acquired motor fusion deficiency, clinical features, 231–232
Mu¨ ller muscle contraction, lid retraction/lid lag and, 424
Multiple sclerosis (MS)
internuclear ophthalmoplegia (INO), 316, 317–318, 318
optic neuritis and
lumbar puncture results, 46 neuroimaging studies, 44–46 risk assessment, 50–53, 51–52 therapeutic strategies, 47–49, 48
pendular nystagmus, 379–381 vertical diplopia syndromes, 236–237
Myasthenia gravis (MG). See also Ocular myasthenia gravis
acquired isolated third nerve palsies (TNP), normal sphincter, incomplete extraocular muscle palsy (type 4B TNP), 266
antiacetylcholine receptor antibody testing, 341
diplopia and, 218 horizontal diplopia, 225 lid retraction/lid lag, 425
non-pharmacologic diagnosis, 340–341
ocular myasthenia gravis, differential diagnosis, 337–341
one-and-a-half syndrome, 320–321 ptosis etiology, 415
thymoma, CT imaging for, 342, 343 vertical diplopia, 238–239
Index 475
Myasthenic pseudo-INO clinical features, 318 evaluation protocols, 318
Myopathies
lid retraction/lid lag and, 424–425 ptosis etiology, 412–413, 414
Nerve palsies, convergence spasm, 230–231
Neurofibromatosis-2, papilledema, 135–136
Neurogenic disorders
lid retraction/lid lag, 422–424 ptosis etiology, 413, 414, 415
Neuroimaging
acquired isolated third nerve palsies (TNP)
normal sphincter, complete extraocular muscle palsy (type 4A TNP), 264–265
normal sphincter, incomplete extraocular muscle palsy (type 4B TNP), 266
subnormal pupillary sphincter dysfunction, extraocular muscle palsies (type 4C TNP), 267
fourth nerve palsy (FNP), subarachnoid space lesion, 283
homonymous hemianopsia, 205–207 isolated fourth nerve palsies (TNP),
289–290,
290–292
isolated third nerve palsies (TNP), 268–270
Neuromuscular junction diseases ptosis etiology, 415
vertical diplopia, 238 Neuromyelitis optica. See Devic’s
disease Neuroretinitis
clinical features, 63–64 evaluation flowchart, 67, 68 infectious agents, 64–66, 67
optical disc edema with macular star (ODEMS), differential diagnosis, 64–67, 65, 67–68
476 Clinical Pathways in Neuro-Ophthalmology, second edition
Nonarteritic anterior ischemic optic neuropathy
(NA-AION)
clinical features, 73–75, 74–75 atypical features, 75, 78–79, 79
evaluation and treatment, 78–79 future research issues, 80–81
giant cell arteritis (GCA), differential diagnosis, 95–96, 96–98, 98–99
therapeutic management aspirin therapy, 81–82
experimental treatments, 82–83 flowchart for, 84, 85
optic nerve sheath fenestration, 83, 83–85
Nonketotic hyperglycemia, homonymous hemianopsia, 206
Nuclear complex of the posterior commissure (NPC), lid retraction/lid lag, 422–424
Nuclear lesion, third nerve palsies (TNP), 254–255
Nutritional optic neuropathy clinical features, 9, 12–14, 17
Leber’s hereditary optic neuropathy (LHON), differential diagnosis, 22
Nylen-Barany maneuver, binocular symmetric jerk nystagmus, 391
Nystagmoid jerking, stuporous/ comatose patients, 398–399
Nystagmus
acquired binocular pendular nystagmus, diagnosis and etiology, 379–380
asymmetric binocular oscillations, etiology, 368, 370–372, 373
binocular/symmetric, binocular/ asymmetric, or monocular oscillations, 368
binocular symmetric conjugate oscillations, 376
binocular symmetric jerk nystagmus eccentric gaze/maneuvered
induction, 389–391 etiology, 382–385
binocular symmetric pendular conjugate oscillations, etiology, 377–382, 383 clinical features, 367–368, 369
downbeat nystagmus, 385–387, 387 dysconjugate bilateral symmetric eye
oscillations, 372
horizontal dysconjugate oscillations, etiology, 375, 376
monocular oscillations, etiology, 368, 370–372, 373
periodic alternating nystagmus (PAN),
384,
384–385
predominantly vertical jerk nystagmus, 385–389
saccadic intrusions, 391–395
seesaw nystagmus, etiology, 373–374,
374
spontaneous oscillations, stuporous and comatose patients, 396–399, 397
upbeat nystagmus, 388, 388–389
Obesity, idiopathic pseudotumor cerebri, 140–142, 141
therapeutic management protocols, 145–147, 146
Occipital epilepsy, idiopathic, binocular transient visual loss, 181
Occipital hypoperfusion, binocular transient visual loss, migraine and, 180
Occipital ischemia, bilateral, binocular transient visual loss, 182
Occipital lesions, visual field defects, 202–205
Occipital lobe tumors, binocular transient visual loss, 180–181
Ocular bobbing
one-and-a-half syndrome, 319–320 stuporous/comatose patients, 398–399
Ocular flutter, saccadic intrusions, classification and etiology, 393–395
Ocular hypoperfusion, monocular TVL and, 173–179
Ocular inflammation, Graves’ ophthalmopathy (GO), 352
Ocular ischemic syndrome, transient visual loss and, 175–179
Ocular motor cranial neuropathy, diplopia and, 218
Ocular myasthenia gravis (OM) evaluation flowchart, 342, 343 management protocols, 342–344 myasthemia gravis (MG), differential
diagnosis, 337–341 non-pharmacologic diagnosis,
340–341 prognosis, 344–345 testing summary, 341–342
thymectomy, indications for, 344 thymoma, CT imaging for, 342, 343
Ocular neuromyotonia (ONM), horizontal diplopia, 227
Ocular tilt reaction (OTR) vertical diplopia, 234, 236–237 vertical gaze disorders, 328
Ocular torsion, vertical diplopia, 233–234 Oculomasticatory myorhythmia,
horizontal dysconjugate eye oscillations, 375,
376
Oculomotor nerve. See also Third nerve palsies (TNP)
acquired isolated third nerve palsies (TNP), normal sphincter, complete extraocular muscle palsy (type 4A TNP), 264–265
anatomy, 253 Oculopalatal myoclonus
one-and-a-half syndrome, 319–320 pendular nystagmus, 380
One-and-a-half syndrome clinical features, 319–321
etiologies and evaluation protocols,
320–321, 321
vertical gaze palsy with, 327–328 Ophthalmoplegia, bilateral preganglionic
internal, third nerve palsies (TNP), fascicular lesion, 258–259
Ophthalmoplegic migraine third nerve palsies (TNP),
subarachnoid lesion, 256–257, 259–260
vertical diplopia, 237–238
Index 477
Opsoclonus-myoclonus syndrome (OMS), saccadic intrusions, classification and etiology, 394–395, 396
Optic chiasm, visual field defects chiasmal syndromes, 194, 195–196, 196,
197
junctional characteristics, 192
Optic disc cupping, optic neuropathy diagnosis, 2
Optic disc drusen evaluation protocols, 130
papilledema differential diagnosis, 130
Optic disc edema differential diagnosis,
pseudopapilledema, 131, 134 evaluation protocol, 154 papilledema, 129
differential diagnosis, optic neuropathy, 131, 131
pseudopapilledma, differential diagnosis, 131, 131
Optic disc edema with macular star (ODEMS)
clinical presentation, 3, 63–64, 64 evaluation flowchart, 67, 68 infectious agents, 64–66, 67 neuroretinitis, differential diagnosis,
64–67, 65, 67–68 prognosis, 67, 69
Optic glioma, compressive optic neuropathy, clinical features, 5, 7–8
Optic nerve, visual field defects, junctional characteristics, 192–194
Optic nerve sheath fenestration (ONSF) nonarteritic anterior ischemic optic
neuropathy (NA-AION), 83, 8385
pseudotumor cerebri therapy, 148–155,
153
complications, 152–154, 153 Optic neuritis (ON)
anterior ischemic optic neuropathy (AION), differential diagnosis, 73–75, 74–75
associated disorders, 38, 40–42
478 Clinical Pathways in Neuro-Ophthalmology, second edition
Optic neuritis (ON) (cont.) atypical optic neuritis, clinical
presentation, 38,
39
clinical presentation, 3, 35, 36–38 evaluation protocol, 39, 42 lumbar punctures, indications
for, 46
multiple sclerosis (MS) and lumbar puncture results, 46 neuroimaging studies, 44–46 risk assessment, 50–53, 51–52 therapeutic strategies, 47–49, 48
neuroimaging techniques, 44–46 pediatric patients, clinical features,
38–39, 43 treatment strategies
immunoglobulin therapy, 49 interferon therapy, 49 steroids, 47–49, 48
vision prognosis, 49–50 visual field defects, 35–36
visually evoked potentials (VEPs), 47 Optic neuritis treatment trial (ONTT)
study
clinical results, 43–44, 44 multiple sclerosis risk assessment,
50–53, 51–52 neuroimaging techniques, multiple
sclerosis lesions, 44–46 therapeutic strategies, 47–49, 48 vision prognosis measurements, 49–50
Optic neuropathies diagnosis
anterior ischemic optic neuropathy (AION), 3
atypical/unexplained optic neuropathy, 14, 22
clinical evaluation flowchart, 4 clinical features and etiology, 1, 12 compressive optic neuropathy
(CON), 3, 5, 58 hereditary optic neuropathy, 19,
20–22, 22 infiltrative/inflammatory optic
neuropathy, 56, 7, 8, 13 optic disc edema with macular star
(ODEMS), 3 optic neuritis, 3
radiation exposure (RON), 14, 16–17,
18–19
toxic/nutritional optic neuropathy, 914, 15–17
traumatic optic neuropathy (TON), 9 papilledema, differential diagnosis,
131, 131
visual field defects, 191–192 Optic radiations, homonymous
hemianopsia, 201–202 Optic tract lesions, homonymous
hemianopsia, visual field defects, 198–199
Orbital blow-fracture, vertical diplopia, 242
Orbital disease
diplopia and, restrictive ophthalmoplegia, 218
horizontal diplopia, orbital pseudotumor/orbital myositis, 223–226
Orbital fourth nerve palsies (FNP), clinical evaluation, 283
Orbital inflammation, Graves’ ophthalmopathy (GO), 352
Orbital lesion
fourth nerve palsies (FNP), 284 sixth nerve palsies (SNP), 301 third nerve palsies (TNP), 261
Orbital myositis
differential diagnosis, 224–226, 226 horizontal diplopia, 223–226
Orbital pseudotumor differential diagnosis, 224, 225 horizontal diplopia, 223–226
Orbital trauma. See also Traumatic optic neuropathy (TON)
exotropia (XT), orbital trauma, 226 horizontal diplopia, 225–226
orbital blow-fracture, vertical diplopia, 242
third nerve palsy (TNP), 262
Palatal myoclonus, pendular nystagmus, 380
Papilledema bilateral
clinical evaluation, 132–133, 135, 135 etiology, 131, 132
clinical features, 130–131, 131–134 defined, 129
differential diagnosis
optic neuropathy, 131, 131 pseudopapilledema, 129–130
etiology, 132
evaluation protocols, 130 monocular TVL with, 171–172 neuroimaging studies, 132–133, 135,
135
pseudotumor cerebri syndrome clinical signs, 143–144 defined, 135–137
stages of, 131, 133
unilateral papilledema, etiology, 131
Papilllomacular bundle, visual field defects, optic neuropathies, 191–192
Papillomacular involvement, optic neuritis (ON), 36, 38
Paramedian pontine reticular formation (PPRF)
horizontal gaze palsy, 314 one-and-a-half syndrome, 319–321
Paroxysmal superior rectus, vertical diplopia, 236
Pediatric patients
Horner’s syndrome evaluation, 454–455
optic neuritis, clinical features, 38–39,
43
pseudotumor cerebri syndrome, optic nerve sheath fenestration (ONSF) therapy in,
151–155
transient visual loss assessment, 179 Pendular nystagmus
acquired monocular variation, etiology, 370
diagnosis and management, 379–382,
383
Pentoxifylline, Graves’ ophthalmopathy (GO) therapy, 354
Periodic alternating esotropia (PAE), clinical features, 228
Periodic alternating nystagmus (PAN), clinical features and etiology, 383–385, 384
Index 479
Peripheral ganglion cells, visual field defects, optic neuropathies, 191–192
Peripheral vestibular disease, horizontal nystagmus, 382–383
Peripheral visual defects, clinical presentation, 36
Pernicious anemia, nutritional optic neuropathy, clinical features, 9, 12–14, 17
Petrous apex lesion, sixth nerve palsies (SNP), 297
Pharmacological localization, Horner’s syndrome, 452–454, 453, 455
Pharmacological mydriasis/miosis, anisocoria, 436, 437–439 Phoria syndromes, vertical diplopia,
239
Phorias, diplopia evaluation, 219 ‘‘Pie-in-the-sky’’ defects, homonymous
hemianopsia, optic radiations, 201–202
‘‘Pie-on-the-floor’’ defects, homonymous hemianopsia, optic radiations, 201–202
Pilocarpine testing anisocoria, 435
tonic pupil, 440–441, 442–443 Ping-pong gaze, stuporous/comatose
patients, 397–399
Pinhole device, monocular diplopia, 215, 217, 217
Platelet-fibrin emboli, monocular TVL, 173
Plus-minus syndrome, third nerve palsies (TNP), 259
Polymerase chain reaction (PCR), vertical gaze palsy evaluation, 326
Pontine (lower pons) lesion horizontal gaze palsies, 312, 315 sixth nerve palsies (SNP), 295–296,
298–300
vertical gaze palsy, locked-in syndrome, 327–328
Posterior cerebral artery infarction, visual field defects, homonymous hemianopsia, 204–205
480 Clinical Pathways in Neuro-Ophthalmology, second edition
Posterior ischemic optic neuropathy (PION)
associated conditions, 75, 76–78 clinical features, 74, 7475
Postganglionic Horner’s syndrome clinical evaluation and etiology,
446–447, 450–451 evaluation protocols, 455–456 pharmacologic etiology, 454
Postprandial transient visual loss, evaluation, 174
Prednisone
Graves’ ophthalmopathy (GO) therapy, 351, 353
optic neuritis (ON) therapy, 47–49, 48 Preganglionic (intermediate) Horner’s
syndrome
clinical evaluation and etiology, 446,
448 pharmacologic etiology, 454
Pretarsal motor persistence (PMP), apraxia of eyelid opening, 410
Pretectal pseudobobbing, stuporous/ comatose patients, 399
Proptosis, Graves’ ophthalmopathy (GO), treatment alternatives, 357–358, 358–360, 361
Prostigmin test, myasthemia gravis (MG) vs. ocular myasthenia gravis (OMG), 338–341
Proton density imaging (PDI), internuclear ophthalmoplegia (INO), 319
Pseudo-internuclear ophthalmoplegia (INO), nystagmus etiology, 370
Pseudo-one-and-a-half syndrome, clinical features, 321
Pseudopapilledema, differential diagnosis
papilledema, 129–130
true optic disc edema, 131, 134 Pseudoptosis, ptosis, differential
diagnosis, 409, 409 Pseudotumor cerebri syndrome
associated systemic disease, 136,
138–139, 140 clinical signs, 143–144 defined, 135–137
drug toxicity and, 137 etiologies, 138–139 evaluation protocols, 144–145
gaze-evoked transient visual loss (TVL), 170–171
idiopathic pseudotumor cerebri, 140, 141
diagnostic criteria, 140, 141 risk factors and clinical
characteristics, 140–142 symptoms, 142, 143
therapeutic management protocols, 145–147, 146
symptoms, 142, 143
therapeutic management protocols, 145–147, 146
lumbar punctures, 147 surgical procedures, 148–155,
149, 154
Pseudo-von Graefe phenomenon, third nerve palsy (TNP), aberrant regeneration, 270–271
Ptosis
acquired vs. congenital, 410, 411, 412
acquired, etiology, 410, 411, 412 aponeurotic ptosis, 417
apraxia of eyelid opening, 410, 411 blepharospasm, 409–410 evaluation protocol, 416 hemifacial spasm, 409–410 hypertropia/hyperglobus, 410 isolated, classification, 412, 412 isolated, etiology, 415, 415 mechanical etiology, 412, 413 myasthemia gravis (MG) vs. ocular
myasthenia gravis (OMG), differential diagnosis, 338–341
myogenic etiology, 412–413, 414 neurogenic etiology, 413, 414, 415 neuromuscular junction disease, 415 nonisolated, etiology, 412, 412 ocular myasthenia gravis (OM),
management of, 342–344 pseudoptosis, differential diagnosis,
409, 409 steroid-induced, 417 trauma and, 415
Pupillary abnormalities intermittent/transient phenomenon,
etiology, 436, 439 syphilis serology testing, 444–445
Pupillary sphincter, acquired isolated third nerve palsies (TNP)
complete extraocular muscle palsy (type 4A TNP), 264–265
incomplete extraocular muscle palsy (type 4B TNP), 265–266
subnormal dysfunction, extraocular muscle palsies (type 4C TNP), 267
Purtscher’s retinopathy, venous stasis retinopathy, transient visual loss, differential diagnosis, 174
Quadrantanopias, homonymous hemianopsia
occipital lesions, 202–205 optic radiations, 201–202
Quadruple sectoranopia, homonymous hemianopsia, lateral geniculate body lesions, 200
Radiation optic neuropathy (RON), clinical features, 14, 16–17,
18–19, 19
Radioactive iodine (RAI) therapy, Graves’ ophthalmopathy (GO), 351, 353
Radiotherapy, Graves’ ophthalmopathy (GO), 354–356, 355
Reading-evoked transient visual loss (TVL), evaluation, 171
Rebound nystagmus, etiology, 389–391 Relative afferent pupillary defect
(RAPD), optic tract lesions, homonymous hemianopsia, 198–199
Repetitive divergence, stuporous/ comatose patients, 398–399
Restrictive ophthalmopathy, vertical diplopia, 240–242
Restrictive ophthalmoplegia, orbital disease, diplopia and, 218 Retinal emboli, monocular TVL and,
172–173, 177, 177–179
Index 481
Retinal fibers, visual field defects, optic neuropathies, 192
Retinal lesions, visual field defects, 189, 191, 191
Rubeosis iridis, transient visual loss and, ocular ischemic syndrome, 175–179
Saccadic intrusions, classification and etiology, 391–395, 394
Scintillating scotoms, binocular transient visual loss, 180–182
Seesaw nystagmus, clinical features and etiologies, 373–374, 374
Seizure disorders
binocular transient visual loss, 180–181 epileptic nystagmus, 384–385
lid retraction/lid lag and, 423–424 Sensory esotropia, etiology, 222 Sensory exotropia, etiology, 222 Short-cycle periodic alternating gaze,
stuporous/comatose patients, 397–399
Shy-Drager syndrome, alternating Horner’s syndrome, 448
Silent sinus syndrome, vertical diplopia, 242
Single-fiber electromyography (SFEMG) abnormalities
myasthenia gravis (MG) diagnosis, 341 ocular myasthenia gravis (OM)
diagnosis, 341–342
Single photon emission tomography (SPECT), binocular transient visual loss, migraine and, 180
Sixth nerve palsies (SNP) anatomy, 295, 296–297
cavernous sinus lesion, 298–300, 301 evaluation guidelines, 301–304, 303 isolated vasculopathic, evaluation
protocols, 301–302 localization and classification, 296,
298–300
nonisolated SNP, pontine (lower pons) lesion, 295–296, 298–300
nonvasculopathic, evaluation protocols, 302
orbital lesion, 301 petrous apex lesion, 297
482 Clinical Pathways in Neuro-Ophthalmology, second edition
Sixth nerve palsies (SNP) (cont.) progressive/unresolved SNP,
evaluation protocols, 302 subarachnoid space lesion, 297, 297 unilateral isolated SNP, diagnostic
criteria, 296
Skew deviation, clinical evaluation, 327–328
‘‘Sleep test,’’ myasthenia gravis (MG) diagnosis, 340–341
Small-cell lung cancer (SCLC), saccadic intrusions, classification and etiology, 394–395
Smoking
Graves’ ophthalmopathy (GO) therapy, 352
nutritional optic neuropathy, clinical features, 9, 12–14, 17
Spasmus nutans, nystagmus etiology, 368, 370
Spontaneous anterior chamber hemorrhage, transient visual loss, 175
Spontaneous eye oscillations, stuporous/ comatose patients, 396–399,
397–398
Spontaneous jerk nystagmus, clinical features and etiology, 385
Square-wave jerks, saccadic intrusions, classification and etiology, 391–395, 394
Steroids
Graves’ ophthalmopathy (GO) therapy, 354–356, 355
ptosis etiology, 417 Strabismus
divergence insufficiency/paralysis, 228–230
Graves’ ophthalmopathy (GO), treatment alternatives, 356–357
vertical diplopia, 239–245
Striate cortex lesions, visual field defects, homonymous hemianopsia, 204–205
Stroke patients, transient visual loss risk and, 176–179
Stuporous patients, spontaneous eye oscillations, 396–399, 397–398
Subarachnoid space lesion fourth nerve palsy (FNP), 283
sixth nerve palsies (SNP), 297, 297 third nerve palsies (TNP), 256–257,
259–260
neuroimaging protocols, 269–270 Superior oblique click syndrome, vertical
diplopia, 241–242 Superior oblique myokymia (SOM)
nystagmus etiology, 370–372 vertical diplopia, 237–238
Superior oblique (SO) palsy fascicular lesion, 259 pseudo-overaction, fourth nerve
palsies (FNP), 282
vertical diplopia, 236
Superior rectus palsy, isolated, vertical diplopia, 238–239
Supranuclear gaze disorders abducens nucleus anatomy, 312,
314
horizontal conjugate gaze, anatomy, 311
horizontal gaze palsies clinical evaluation, 312, 315
lesion localization and classification, 311, 312–314
internuclear ophthalmoplegia (INO) clinical features, 314–315, 316 etiology, 316, 317–318
evaluation protocols, 318–319 medial longitudinal fasciculus (MLF)
anatomy, 312, 314 one-and-a-half syndrome
clinical evaluation, 319–321, 321 etiologies, 321, 321
skew deviation, clinical features, 327–328
supranuclear monocular elevation paresis, clinical features, 327–328
vertical gaze impairment anatomy, 321
etiology, 322, 324–325
evaluation protocols, 323, 325–326,
326
lesion localization of palsies, 321–322,
322–323
vertical one-and-a-half syndrome, clinical features, 327–328 Supranuclear monocular elevation paresis, clinical features,
327–328 Supranuclear ophthalmoplegia
diplopia and, 219
vertical diplopia, 234, 236–237 Surgical procedures
Graves’ ophthalmopathy (GO) lid retraction treatments, 356
proptosis and/or compressive optic neuropathy (CON), 353, 357–358, 358–359, 361
traumatic optic neuropathy (TON), 124, 125, 126
Syphilitic meningitis, neuroretinitis and optic disc edema with macular star (ODEMS), 65–66
Systemic disease, pseudotumor cerebri syndrome, 136–137
Systemic lupus erythematosus, transient visual loss, 175
Systemic thyroid status, Graves’ ophthalmopathy (GO), 350
Temporal artery biopsy (TAB), giant cell arteritis (GCA)
clinical diagnosis, 94, 94–96, 96–98, 98–99
indications for, 101–104
therapeutic complications, 108–110, 111 unilateral vs. bilateral TAB, 101–104
Tensilon test, myasthemia gravis (MG) vs. ocular myasthenia gravis (OMG), differential diagnosis, 338–341
Third nerve palsies (TNP)
aberrant regeneration (type 6), 270–271 acquired isolated TNP
neuroimaging protocols, 268–270 nontraumatic (type 4), 262 normal pupillary sphincter,
extraocular muscle incomplete palsy (type 4B), 265–266
normal pupillary sphincter, extraocular muscle palsy (type 4A), 264–265
Index 483
subnormal pupillary sphincter dysfunction, extraocular muscle palsies (type 4C), 267
anisocoria, 434–435 bilateral, nuclear lesions, 255
cavernous sinus lesion, 260–261 classification, 253, 254
clinical features, 253 congenital origins, 262 fascicular lesion, 258–259
lid retraction/lid lag and, 423–424 localization etiologies, 253, 256–257 monocular elevator paresis,
subarachnoid lesion, 259–260 nonisolated TNP, clinical evaluation,
261–262, 263 nuclear lesion, 254–255 orbital lesion, 261
progressive/unresolved (type 5 TNP), evaluation protocols, 270
ptosis etiology, 413, 414, 415 retrospective review, 271–272 subarachnoid lesion, 259–260 trauma, 262
unilateral TNP, nuclear lesions, 255 Thromboembolic disease, monocular TVL and, 172–179
Thymectomy, ocular myasthenia gravis (OM), indications for, 344
Thymoma evaluation, myasthenia gravis (MG) diagnosis, 342
Thyroid eye disease diplopia and, 218–219 Graves’ ophthalmopathy
clinical features, 349, 350 decompression studies, 358–359 evaluation and treatment flowchart,
360
imaging studies, 349–350 immunosuppressive therapy, 352–354 lid retraction, treatment protocols,
356
ocular and orbital inflammation, 352 proptosis and/or compressive optic neuropathy, 357, 359
proptosis without optic neuropathy, 359, 361
radiotherapy, 354–356 strabismus management, 356–357
484 Clinical Pathways in Neuro-Ophthalmology, second edition
Thyroid eye disease (cont.) systemic thyroid status, 350
therapeutic management, 350–352,
355
horizontal diplopia, 224–226, 226 lid retraction/lid lag, 424–425 vertical diplopia, 241
Thyroid ophthalmopathy, binocular transient visual loss, 182
Tilted optic disc, papilledema, differential diagnosis, 131, 131
Tonic pupil
Adie’s tonic pupil syndrome clinical features, 441, 444 etiology, 441 neuroimaging studies, 441 therapeutic strategies, 444
anisocoria, 440–441, 442–443 isolated, differential diagnosis, 441,
442–443
syphilis serology testing, 444–445 Toxic optic neuropathy, clinical features,
914, 15–16
Transient diplopia, etiology, 217, 218 Transient visual loss (TVL)
binocular TVL etiology, 179–183
evaluation, 167, 169–170, 183 etiology, 171–179, 177 monocular TVL
duration of episodes, reading-evoked TVL, 171–179
gaze positions, 168, 168169, 171 patient history, 167
prolonged reading, 171
Transurethral prostatic resection (TURP) syndrome, binocular transient visual loss, 183
Traquair’s junctional scotoma, visual field defects, 193–194
Trauma. See Orbital trauma fourth nerve palsies (FNP), 287
Horner’s syndrome and, 449, 451 ptosis etiology, 415
sixth nerve palsy (SNP), evaluation protocols,
301
Traumatic optic neuropathy (TON) clinical features, 9, 119, 119
evaluation and treatment flow chart,
125
grading and classification, 120, 121 neuroimaging, 119–120 pathogenesis, 119, 120
therapeutic management strategies, 120–126, 125
corticosteroid therapy, 122–123 protocols and classification, 123, 123 surgical management, 124–126
Trigemino-oculomotor synkinesis, lid retraction/lid lag and, 423–424
Trochlear nerve. See also Fourth nerve palsies (FNP)
localization, 284
Tropias, diplopia evaluation, 219 T2-weighted imaging, internuclear
ophthalmoplegia (INO), 319
Upbeat nystagmus, clinical features and etiology, 386–389, 388
Uveitis-glaucoma-hyphema (UGH) syndrome, transient visual loss, 175, 177
Valsalva maneuver
binocular symmetric jerk nystagmus, 391
gaze-evoked transient visual loss (TVL), 170–171
Vasculopathic fourth nerve palsies (FNP), evaluation protocols, 287
Vasculopathic sixth nerve palsy (SNP) evaluation protocols, 301–302
risk factors, 302
Vasospasm, transient visual loss, 175–176 Venereal Disease Research Laboratory
(VDRL) test, papilledema, clinical evaluation, 135
Venous occlusive disease, pseudotumor cerebri syndrome, evaluation protocols, 144–145
Venous stasis retinopathy, transient visual loss, 174–179
Vertebrobasilar transient ischemic attacks, binocular transient visual loss, 182
evaluation protocol, 183 Vertical diplopia
cranial nerve impairment, 237–238 etiology, 234, 235–236 examination protocol, 232–234
extraocular muscle disease, 238–240 mechanical misalignment processes,
240–245
monocular elevator paresis, 234, 236–237
neuromuscular junction diseases, 238 supranuclear processes, 234, 236–237
Vertical gaze impairment anatomy, 321
etiology, 322, 324–325
evaluation protocols, 323, 325–326, 326 lesion localization of palsies, 321–322,
322–323
Vertical jerk nystagmus, clinical features and etiology, 385–389
Vertical ocular myoclonus, stuporous/ comatose patients, 399
Vertical pendular nystagmus, etiology, 370
Vertigo, binocular symmetric jerk nystagmus, 390–391
Vistibular tone imbalance, nystagmus etiology, 367368 i368
Visual acuity
optical disc edema with macular star (ODEMA), 64
pseudotumor cerebri syndrome, optic nerve sheath fenestration (ONSF) and, 150–155
traumatic optic neuropathy (TON), 121–122
Visual field defects
bilateral defects, clinical features, 192,
193
binasal hemianospia, 196, 198 bitemporal hemianopsia, 194, 195–196,
196
evaluation protocols, 190 homonymous hemianopsia, 198
lateral geniculate body lesion, 199–200
normal neuroimaging, 205–207 optic radiation, 201–202
optic tract lesions, 198–199
Index 485
therapeutic management, 207 junctional defects, 192–194 monocular temporal cresent, 192 occipital lesions, 202–205
optic neuritis (ON), 35–36 optic neuropathy, 191–192
pseudotumor cerebri syndrome, 144 retinal lesions, 189, 191, 191 topographical diagnosis, 189, 190 unexplained defects, 207–208 unilateral defects, 189
Visual loss
giant cell arteritis (GCA), clinical diagnosis, 95–96, 96–98, 98–99
monocular nystagmus, 370 transient visual loss (TVL)
binocular TVL etiology, 179–183
evaluation, 167, 169–170, 183 etiology, 171–179, 177
monocular TVL, duration of episodes, 171–179
monocular TVL, gaze positions, 168, 168–169, 171
patient history, 167 prolonged reading, 171
Visually evoked potentials (VEPs), optic neuritis (ON), indications for, 47
Visual prognosis
nonarteritic anterior ischemic optic neuropathy (NA-AION), 79
levodopa therapy, 82–83
optic nerve sheath fenestration, 83, 83–85
optic disc edema with macular (ODEMS), 67, 69
optic neuritis (ON), 49–50 Visual recovery
giant cell arteritis (GCA), therapeutic protocols, 106–108
optic neuritis (ON), 49–50 traumatic optic neuropathy (TON)
corticosteroid therapy, 122–123 natural history and progression,
120–122
Vitamin A, idiopathic pseudotumor cerebri etiology, 140–142, 141
486 Clinical Pathways in Neuro-Ophthalmology, second edition
Vitamin E deficiency syndrome, horizontal gaze palsy, 314–315
Vitreous hemorrhage, secondary exotropia, 232
Voluntary nystagmus, diagnosis and management, 379
Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome, horizontal gaze palsy, 314
Weber’s syndrome, third nerve palsies (TNP), fascicular lesion, 258–259
Weight reduction programs, idiopathic pseudotumor cerebri therapy, 145–147, 146
Wernicke’s syndrome, vertical diplopia, 236
Whipple’s disease, vertical gaze palsy, 325–326
Wilbrand’s knee, visual field defects, junctional characteristics, 192–194
