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Common Neuro-Ophthalmic Pitfalls

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  • 27 b/w illus. 24 tables
  • Page extent: 234 pages
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 (ISBN-13: 9780521713269)

Common Neuro-Ophthalmic Pitfalls
Cambridge University Press
9780521713269 - Common Neuro-Ophthalmic Pitfalls - Case-Based teaching - By Valerie A. Purvin and Aki Kawasaki
Index

Index

14–3–3 protein 168, 169

abducens nerve

See sixth nerve

abduction deficit

acute isolated 77

bilateral, convergence spasm 144–5

incidental 53–4

painful diplopia with isolated 152–3

accommodative spasm 145–6

acetazolamide

adverse effects 192–3

episodic ataxia with nystagmus 205

idiopathic intracranial hypertension 192–3

acetylcholine receptor antibodies 160

acetylcholinesterase (AChE) inhibitors, ocular myasthenia 201–2

acromegaly 72

acute idiopathic blind spot enlargement (AIBSE) 9, 14, 16–17

acute zonal occult outer retinopathy (AZOOR) 16

adduction deficit, farmer with 113–14

Adie syndrome 181

Adie's tonic pupil

acute, vs pharmacologic mydriasis 139–42

chronic, vs Argyll Robertson pupils 142–4

clinical features 181

isolated unilateral mydriasis 180–1

pathophysiology 180

symptomatic treatment 205

adrenergic denervation supersensitivity 66

Alexander's law 110–11

Alzheimer's disease 169–72

spatial organization deficits 169, 171

visual variant 169–72

vs Creutzfeldt–Jakob disease 168, 171

amaurosis fugax 23–4

amitriptyline 192

Amsler grid testing 2, 125

sudden difficulty reading the paper 123, 132

anemia, pernicious 157–8

aneurysms

acute 3rd nerve palsy 163–5

chronic 6th nerve palsy 186

fusiform, basilar artery

See basilar artery, dolichoectasia

posterior communicating artery

See posterior communicating artery (pCOM) aneurysms

angiography

computed tomography (CTA) 72, 164–5

conventional catheter

See catheter angiography

magnetic resonance

See magnetic resonance angiography

See also computed tomography (CT), venography

angiopathy, peripapillary microvascular 138

angiotensin converting enzyme (ACE), serum 155

angle recession, post-traumatic 10

anisocoria

acute painful ophthalmoplegia 91

angle closure glaucoma 12

Horner syndrome 66, 68, 70

anterior choroidal artery occlusion 98, 100

anterior ischemic optic neuropathy (AION) 17–18

arteritic 195–6

arteritic vs non-arteritic 195–6

bilateral sequential 195

non-arteritic

See non-arteritic anterior ischemic optic neuropathy

anticardiolipin antibody syndrome 64

antihypertensive therapy

malignant hypertension 4, 8

overzealous, in NAION 197–9

antineutrophilic cytoplasmic antibodies (ANCA) 155

antiparietal cell antibodies 157

antirecoverin antibodies 22

apraclonidine

testing, Horner syndrome 66, 68

therapy 205

apraxia, constructional 169

aqueductal stenosis 45, 175

Argyll Robertson pupils, vs chronic tonic pupils 142–4

aspergillosis 88

ataxia

episodic, with nystagmus 205

optic 170

atherosclerosis 183

atrial fibrillation 158

aura, migraine 15–16, 186–8

B-cell lymphoma 77

baclofen 205

Balint's syndrome 170

bariatric surgery 147–8, 193

basilar artery, dolichoectasia (fusiform aneurysm) 88–90

bevacizumab 198

Bielschowski three-step test 55–6

bitemporal visual field defects 118, 128

assessing vertical alignment 130

pseudo- (ocular disorders) 119, 128–35

blind spot

acute idiopathic enlargement (AIBSE) 9, 14, 16–17

enlarged, in MEWDS 11, 17

blood pressure

lowering therapy 4, 8

measurement 4, 7–8

overzealous treatment in NAION 197–9

See also hypertension

blurring of vision 1

episodic monocular 23–4

headache and 4

painful mydriasis with 10–13

See also visual loss

botulinum toxin injections 205

bouncing vision 110–13

bovine spongiform encephalopathy 168

“boxer” ptosis 70–2

brain tumor, suspected 4

brainstem

stroke, vs Wernicke's encephalopathy 146–9

syndrome, with negative scan 165–7

breast cancer metastases, orbital apex syndrome 95

brimonidine 198

bruit 108

C-reactive protein (CRP) 195

cancer-associated retinopathy (CAR) 16, 22–3

carbamazepine 205

cardiac glycosides, toxicity 158–60

carotid arteriography

See catheter angiography

carotid artery

dissection 70–2

paraganglioma displacing 67–8

See also internal carotid artery

carotid artery disease

non-arteritic anterior ischemic optic neuropathy 183–4

ocular ischemia 24

carotid-cavernous sinus fistula 107–10

direct or high flow 107

dural (low flow) 107–10

white eyed shunt 108

cat scratch disease, neuroretinitis 104–7

cataract 2–4

catheter angiography

carotid-cavernous sinus fistula 109–10

cerebral aneurysm 163–5

cavernous sinus

sinusitis spreading to 88

thrombosis 109–10

tumors 94, 96–7

cavernous sinus/superior orbital fissure (spheno-cavernous) syndrome 91, 94

etiologies 93

imaging 91–4

plus ipsilateral visual loss and variable proptosis 95

ceco-central scotomas

See scotomas

central gray spot, painless, in a teenager 103–7

central retinal artery occlusion (CRAO) 11, 18–19

hyperacute 18–19

metastatic breast cancer 95

central retinal vein occlusion, carotid-cavernous sinus fistula 109

cerebellar degeneration, downbeat nystagmus 111

cerebellar tonsils

descent, idiopathic intracranial hypertension 86

low 86–7

cerebral edema, hypertensive encephalopathy 6–7

cerebral polyopia 3–4

cerebral venous sinus thrombosis (CVT) 63–6, 81

clinical features 64–5, 81

laboratory evaluation 80

radiographic imaging 64–6, 82

cherry red spot, macular 11, 18–19

chest imaging, Horner syndrome 69

Chiari malformation 45, 111–12

downbeat nystagmus 111

Chiari I malformation 112–13

comitant esotropia 87

downbeat nystagmus 111, 113

oscillopsia 111

symptomatic 112–13

Chiari II malformation 173–4, 176

cholinergic denervation supersensitivity 140–1, 180–1

chorioretinal spots, small yellow-white 105

chronic progressive external ophthalmoplegia (CPEO) 151–2

vs progressive supranuclear palsy 149–52

circumpapillary telangiectatic microangiopathy 138

clinical findings, subtle 103–14

clivus tumors/lesions 77–80

clonazepam 205

cluster headache 70

cobalamin

See vitamin B-12

cocaine testing, Horner syndrome 68

Cogan's lid twitch sign 34

color vision abnormalities

digitalis toxicity 159–60

dominant optic atrophy 132–3

progressive optic neuropathy 60–1

vitamin B-12 deficiency 156

computed tomography (CT)

angiography (CTA) 72, 164–5

bilateral occipital stroke 122, 131, 135

bilateral occipital tip strokes 127

carotid-cavernous sinus fistula 108–9

cerebral venous sinus thrombosis 63, 65

increased intracranial pressure 65–6

neck 69

orbital apex syndrome 95

orbits 33

pituitary apoplexy 72–5

skull base 77–9

venography (CTV) 63–6

congenital anomalies 45–57

conjugate gaze palsies

one-and-a-half syndrome 165

progressive supranuclear palsy 149

Wernicke's encephalopathy 147–8

conjunctival injection

angle closure glaucoma 12–13, 22

chronic 107–10

orbital disease 27–8

orbital myositis 152–3

thyroid eye disease 81

conjunctival vessels, dilated and tortuous 107–9

constricted fields after herniation 120–22

constructional apraxia 169

convergence spasm

examination technique 144–6

vs bilateral 6th nerve palsies 144–6

corkscrew vessels 108–9

cornea

decompensation, transient monocular visual loss 23–4

edema, angle closure glaucoma 12–13

surface drying 3

corneal dystrophy, Fuchs 23

coronary artery bypass surgery, visual loss after 125–8

corticosteroids

demyelinating optic neuritis 199–200

giant cell arteritis 195, 197

ocular myasthenia 201–3

optic perineuritis 40

orbital myositis 38–40

pituitary apoplexy 74–5

tapering 197

Tolosa Hunt syndrome 92, 94

See also methylprednisolone, prednisone

cotton-wool spots

giant cell arteritis 196

malignant hypertension 4–6, 8

retinal artery occlusion 13, 18

cough headache 112

cranial nerve palsy

carotid-cavernous sinus fistula 108

vasculopathic 184–6

see also specific palsies

CRAO

See central retinal artery occlusion

Creutzfeldt–Jakob disease (CJD) 168–9

familial 168

Heidenhain variant 168–9

sporadic 168–9

variant (vCJD) 168

vs Alzheimer's disease 168, 171

crossed brainstem syndromes 91

crossed cylinder testing 189

CT

See computed tomography

cup/disc (C/D) ratio

giant cell arteritis 196

glaucoma 8–10, 20

De Morsier's syndrome 51

demyelinating disease

acute one-and-a-half syndrome 165–7

symptomatic treatment 203–5

See also multiple sclerosis, optic neuritis, demyelinating

denervation

Adie's tonic pupil 140–2, 180

supersensitivity

See adrenergic denervation supersensitivity, cholinergic denervation supersensitivity

developmental delay 51–3

Devic's disease (neuromyelitis optica) 191

dexamethasone, giant cell arteritis 197

diabetes mellitus

fungal sinusitis 88

offspring of mothers with 51, 53

diethylstilbestrol 17–18

digoxin (digitalis) toxicity 158–60

diplopia

acute, in pregnancy 146–7

acute isolated horizontal 76–7

acute painless, brainstem syndrome 165–6

acute unilateral painful ophthalmoplegia 91

atypical pseudotumor cerebri syndrome 80

intermittent horizontal

chronic pink eye 107

empty sella 85

low cerebellar tonsils 86–7

monocular 2–4

painful, with isolated abduction deficit 152–3

painless horizontal

dolichoectatic basilar artery 88–9

oculomotor nerve palsy with aberrant regeneration 96–8

progressive horizontal, sphenoid sinus mucocele 87

progressive supranuclear palsy 151

and ptosis

painful 37–40

painless 160–1, 200–1

slowly progressive 75–6

symptomatic treatment 203–5

vertical

causes 55

intermittent 54–7

mild radiographic changes 81–3

painless 32–4

dolichoectatic basilar artery 88–90

Doll's head maneuver, progressive supranuclear palsy 150–1

Dorello's canal 75–6

dorsal midbrain (Parinaud's) syndrome 143, 173–4, 177

dot/blot hemorrhages 6–7

double-ring (halo) sign 51–2

Drance hemorrhage 10, 20

driving, diplopia during 75–6

drusen, optic disc 46–50

buried 45–50

confirming diagnosis 46–7, 49–50

dry eyes 3

Duane's syndrome

subtypes 54

type I 53–4

dural carotid-cavernous sinus fistula 107–10

dysmetria 112

Edinger–Westphal nucleus 143–4

edrophonium chloride test

See Tensilon test

electroencephalography (EEG), Creutzfeldt-Jakob disease 169

electromyography (EMG), single fiber 160

electrophysiologic tests, hypovitaminosis A 21

electroretinography (ERG) 14

acute idiopathic blind spot enlargement 9, 14

cancer-associated retinopathy 22–3

hypovitaminosis A 16, 19–21

retinitis pigmentosa 118, 120

rod-cone dystrophy 125, 134

elevation deficit, incidental 27–32

Elschnig spots 6

embolic stroke, twinkling after 158–60

embolism

ocular circulation 183

retinal 23–4, 70–2

empty delta sign 65

empty sella (syndrome) 85–6

primary 86

secondary 86

enophthalmos 27

episcleral vessels

corkscrew appearance 108–9

dilation and tortuosity 107–9

episodic scintillating scotoma 186–8

erythrocyte sedimentation rate (ESR) 195

esotropia

comitant, low cerebellar tonsils 86–7

convergence spasm 144–6

dolichoectatic basilar artery 88–9

sixth nerve palsy 146

symptomatic treatment 203–5

Wernicke's encephalopathy 148

essential thrombocytosis 81

exercise, Chiari I malformation 112

extra-ocular muscle enlargement

carotid-cavernous sinus fistula 107–9

orbital myositis 38–9, 152

thyroid eye disease 33, 81, 83

extra-ocular muscle surgery 203–4

eye pain

acute unilateral ophthalmoplegia with 91–4

angle closure glaucoma 12

optic neuritis without 137–8

optic neuropathy 40–3

orbital disease 27

postganglionic Horner syndrome 70

ptosis and diplopia with 37–40

transient monocular visual loss (TMVL) with 24

See also headache, pain

eyelids

crease position 34–5

drooping

See ptosis

normal position 27–8

retraction

See lid retraction

facial asymmetry 57, 85

failure to provide symptomatic treatment 203–5

false negative test results 155–77

family album tomography (FAT scan) 57

farmer with adduction deficit 113–14

fatal familial insomnia 168

fibromuscular dysplasia 70–1

flame hemorrhages 6, 8

fluorescein angiography 14

hypovitaminosis A 21

optic disc drusen 50

fogging examination techniques, non-organic visual loss 189

food-cobalamin malabsorption syndrome 157

foramen ovale, patent 123

fourth nerve palsy

3-step test 55–6

causes 55

congenital 54–7

decompensated 57

ipsilateral, and contralateral Horner syndrome 91

Fuchs corneal dystrophy 23

fundus photography, optic disc drusen 47, 50

fungal sinusitis 88

furosemide 192–3

fusional amplitudes, vertical 55, 57

fusional capacity, congenital 4th nerve palsy 55–7

gait disorder, progressive supranuclear palsy 150–1

gastric bypass surgery/gastroplasty 19, 21, 193

Gerstmann–Straussler–Scheinker disease 168

“ghost” images 3

giant cell arteritis (GCA)

anterior ischemic optic neuropathy 195–6

evaluation and treatment 194–7

negative temporal artery biopsies 155, 195

ocular ischemia 24

glaucoma

acute angle closure 12–13, 22

optic disc changes 8–10, 20

optic neuropathy 8–10, 18, 19

globe position 27–8

Goldmann perimetry 14

abnormal field and night blindness 118

abnormal field and photopsias 124, 134

abnormal fields and temporal disc pallor 123, 133

central fields 127

constricted field after herniation 121, 129

incidental field defect 119, 130

post-cardiac bypass visual loss 127, 134

retinitis pigmentosa 117, 127

gonioscopy 2, 10

Graves’ disease

euthyroid 33–34

orbitopathy

See thyroid eye disease

gray spot, painless central, in a teenager 103–7

growth failure 51–3

halo (double-ring) sign 51–2

halos around lights 10–13

hard exudates 4, 6, 8

See also macular star

head tilt

longstanding pre-existing 55, 57

test (3-step test) 55–6

headache

acute angle closure glaucoma 12–13

atypical pseudotumor cerebri syndrome 80

bilateral disc edema with 5, 8

bilateral third nerve palsy and 72–5

Chiari I malformation 112

cluster 70

cough 112

disc elevation with 45–50

increased intracranial pressure 4

low cerebellar tonsils and 86–7

migraine visual aura without 187

migrainous 187

papilledema and 63–6

recurrent, with upgaze deficit 27, 29, 30

third nerve palsy and 162–5

See also eye pain, pain

hematologic malignancies, fungal sinusitis 88

hemeralopia (degradation of vision in light) 1, 21–3

hemodialysis, fungal sinusitis 88

Holmes–Adie syndrome 181

homocysteine, serum 157

homonymous hemianopia

bilateral 127–8, 134

with negative neuroimaging 168–71

small right central 123–5, 132, 133

Horner syndrome 67–9

central 68

etiologies 68

ipsilateral, and contralateral 4th nerve palsy 91

neck imaging 67–9

pharmacologic testing 66, 68

postganglionic 68

acute painful 70–2

painless 66–9

preganglionic 68

ptosis 34, 67–8

symptomatic treatment 205

Tolosa Hunt syndrome 93

hummingbird sign 151

hydrocephalus

constricted fields after herniation 120, 129

dorsal midbrain syndrome 174

shunt failure 173, 175–7

hydrocortisone, pituitary apoplexy 74–75

hydroxyamphetamine test 66, 68–70

hydroxycobalamin therapy 157

hypertension

malignant 4–8

overzealous control in NAION 197–9

See also blood pressure

hypertensive encephalopathy 6–7

hypertensive retinopathy 4–6, 8

hypopituitarism 51–3

hypotension 183, 198–9

hypovitaminosis A 15, 16, 19–21

ice test, myasthenia gravis 160–1

idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) 63–4

atypical 66, 80–1

empty sella 85–6

imaging investigations 65–6

management 191–4

minor radiographic abnormalities 86

secondary 64

surgical management 192–3

immunosuppressed patients, fungal sinusitis 88

immunosuppressive therapy, ocular myasthenia 203

incidental findings 85–90

inferior ophthalmic vein, shunting of blood through 108

inferior rectus muscle entrapment, orbital floor fracture with 29–32

internal carotid artery (ICA)

aneurysm 186

dissection 70–2

See also carotid artery

internuclear ophthalmoplegia (INO)

myasthenia simulating 113–14

one-and-a-half syndrome 165–6

saccadic velocity 114

intra-saccadic fatigue 114

intracranial hypertension, idiopathic (IIH)

See idiopathic intracranial hypertension

intracranial pressure (ICP), increased

cerebral venous sinus thrombosis 63–5

imaging investigations 65–6

neck imaging 69

vs malignant hypertension 4, 7

intraocular pressure (IOP), raised

angle closure glaucoma 12–13

carotid-cavernous sinus fistula 107

glaucomatous optic neuropathy 10

iris sphincter

damage 180

postganglionic parasympathetic denervation 140–2

sectoral palsy 140–2

ischemic optic neuropathy 17–18

anterior

See anterior ischemic optic neuropathy

posterior (PION) 17–18, 127

jaw claudication 194

kuru 168

lateral choroidal artery occlusion 98–9

lateral geniculate body (LGB)

anatomy 98–9

stroke 98–100

Leber's hereditary optic neuropathy (LHON)

vs idiopathic optic neuritis 137–9

Wallace mutation 137

levator palpebrae superioris

dehiscence of aponeurosis 34–7

fatigability testing 34

function, measurement 35

lid retraction 27

on adduction 96–8

on downgaze 97–8

thyroid eye disease 28, 81

light, degradation of vision in

See hemeralopia

light-near dissociation (LND)

Adie's pupil 181

bilateral 142–4

causes and mechanisms 143

monocular 140–1

light reflex, pupillary 143–4

liver disease, vitamin A deficiency 21

look-alike diagnoses 137–53

lumbar puncture, serial 193–4

lumbo-peritoneal shunting, idiopathic intracranial hypertension 193

lung carcinoma

apical 69

small cell 22

lymphoma, B-cell 77

macro square wave jerks 151

macrocytosis 156–7

macropsia 1

macular disorders

diagnostic tests 2

malignant hypertension 6

neuroretinitis 104

occult 14

macular edema, malignant hypertension 4, 6

macular star

differential diagnosis 106

malignant hypertension 6

neuroretinitis 104–7

mad cow disease (bovine spongiform encephalopathy) 168

magnetic resonance angiography (MRA)

carotid-cavernous sinus fistula 109

cerebral aneurysms 163–5

internal carotid artery dissection 70–2

vertebrobasilar dolichoectasia 90

magnetic resonance imaging (MRI)

acute painful unilateral ophthalmoplegia 91, 92

atypical pseudotumor cerebri syndrome 80–2

bilateral idiopathic 6th nerve palsy 77

carotid-cavernous sinus fistula 108–9

cavernous sinus/superior orbital fissure syndrome 91–4

cerebral venous sinus thrombosis 63–5, 81–2

Chiari I malformation 111

clivus lesions 77–80

contrast-enhanced

increased intracranial pressure 65–6

optic nerve sheath meningiomas 60–3

sphenoid wing meningioma 63

Creutzfeldt-Jakob disease 169

demyelinating disease 165–7

demyelinating optic neuritis 199

dolichoectatic basilar artery 88–9

empty sella 85

head and neck 69

idiopathic intracranial hypertension 86

increased intracranial pressure 65–6, 86

internal carotid artery dissection 70–2

low cerebellar tonsils 86–7

multiple sclerosis 203, 204

neck 67–8

optic perineuritis 40, 42

orbital disease 33, 38

orbital myositis 152–3

petrous ridge meningioma 75–76, 78

pituitary apoplexy 72, 74

postganglionic Horner syndrome 66–9, 71

progressive supranuclear palsy 151

right-sided visual field loss 98–9

shunt malfunction 173–6

skull base 77–9

sphenoid sinus mucocele 87

thyroid eye disease 81, 83

Tolosa Hunt syndrome 91–4

twinkling after embolic stroke 158, 159

Wernicke's encephalopathy 148–9

magnetic resonance venography (MRV) 65–6

malabsorption 21

malignant hypertension 4–8

management 191–205

medial longitudinal fasciculus (MLF) 147, 165, 166

meningiomas

optic nerve sheath 61–3

petrous ridge 75–6

sphenoid wing 63

mental status changes

hypertensive encephalopathy 6

Wernicke's encephalopathy 148

Mestinon

See pyridostigmine

metamorphopsia 1

metastatic disease, orbital apex syndrome 95

methylmalonic acid, serum 157

methylprednisolone

demyelinating optic neuritis 199–200

giant cell arteritis 197

multiple sclerosis 203

MEWDS (multiple evanescent white dot syndrome) 11, 17

microangiopathy, circumpapillary telangiectatic 138

micropsia 1

middle cerebral artery stroke 186

migraine 186–8

acephalgic (equivalent) 187

ancillary testing 186–8

aura 15–16, 186–8

complicated 168

vs acute idiopathic blind spot enlargement 17

miosis

during esotropia, convergence spasm 144–5

and ptosis, idiopathic 66–9

monocular visual loss

acute, with disc edema 181–4

chronic 8

large central scotoma 103–7

painful ophthalmoplegia and 94–6

progressive, painful optic neuropathy 40–1

sudden, with normal fundus 11, 17–19

transient

See transient monocular visual loss

MRA

See magnetic resonance angiography

MRI

See magnetic resonance imaging

mucocele, sphenoid sinus 87–8

mucormycosis 88

Müller's muscle 35

multiple evanescent white dot syndrome (MEWDS) 11, 17

multiple sclerosis

optic perineuritis and 43

symptomatic treatment 203–5

See also demyelinating disease, optic neuritis, demyelinating

myasthenia gravis

diagnostic testing 160–1

fatigable ptosis 34

ocular (OMG) 201–3

over-reliance on pyridostigmine (Mestinon) 200–3

pseudo-internuclear ophthalmoplegia (INO) 113–14

saccadic velocity 31, 113–14

mydriasis

isolated unilateral 179–81

painful 10–13

pharmacologic, vs acute tonic pupil 139–42, 180

symptomatic treatment 205

myeloma, multiple 79

myopia, pseudo- 145–6

NAION

See non-arteritic anterior ischemic optic neuropathy

nasopharyngeal biopsy 94, 172–3

nasopharyngeal carcinoma 172–3

near reflex 145

pupillary 143–4

spasm of 144–6

neck extension, Chiari I malformation 112

neck imaging

Horner syndrome 67–9

increased intracranial pressure 69

neck trauma, carotid artery dissection 70

neglect, left-sided 169

nerve stimulation studies 160

neuroimaging

See radiography

neuromyelitis optica 191

neuromyotonia, ocular 205

neuroretinitis 104–7

cat scratch disease 104–7

etiologies 104–5

idiopathic recurrent 106

vs malignant hypertension 6

neurosyphilis 142–3

night blindness (nyctalopia)

abnormal field and 117–20, 126

photopsias and field defects 124, 134–5

slowly progressive 15, 19–21

non-arteritic anterior ischemic optic neuropathy (NAION) 181–4

overzealous treatment of blood pressure 197–9

progressive 198–9

vs arteritic 195–6

nutritional deficiency 157

nyctalopia

See night blindness

nystagmus 110

Chiari I malformation 112

downbeat 111

causes 111–12

Chiari I malformation 111, 113

symptomatic treatment 205

gaze-dependent 110–11

low oscillatory amplitude 110–11

periodic alternating 111, 205

rebound 111

retraction-convergence 174

symptomatic treatment 205

upbeat

causes 148

Wernicke's encephalopathy 147, 148

obesity

bariatric surgery 147–8, 193

idiopathic intracranial hypertension (IIH) 64, 191

See also weight reduction

obstructive sleep apnea syndrome 198

occipital lobes 127

occipital seizures 16

occipital stroke

bilateral, with macular sparing 122, 131

shunt malfunction 175, 176

twinkling after embolic 158–60

occipital tip stroke

bilateral 127–8, 135

unilateral 123–5

ocular disease 1–13

ocular flutter 112

ocular ischemic syndrome (OIS) 24

ocular media, disorders of

monocular diplopia 3

pinhole test 2–3

vs neurologic disease 1

ocular motility disorders

carotid-cavernous sinus fistula 108

Chiari I malformation 112–13

incidental elevation deficit 27–32

painless vertical diplopia 32–4

saccadic velocity 30, 32

See also ophthalmoplegia, specific disorders

oculomotor nerve

See third nerve

oculosympathetic palsy

See Horner syndrome

one-and-a-half syndrome 165–7

ophthalmic artery embolism 183

ophthalmoplegia

acute unilateral painful 91–4

chronic progressive external

See chronic progressive external ophthalmoplegia

internuclear

See internuclear ophthalmoplegia

and ipsilateral optic neuropathy 94–6

painful, and visual loss 94–6

progressive supranuclear palsy 149–51

See also ocular motility disorders

optic ataxia 170

optic atrophy, dominant (Kjer's) 133

optic chiasm lesions

visual field defects 117–18, 128, 134

vs pseudo-bitemporal defects 128–35

optic cup

size, pseudopapilledema 49

See also cup/disc (C/D) ratio, optic disc cupping

optic disc

at-risk 183

drusen

See drusen, optic disc

elevation, and headaches 45–50

glaucomatous changes 8–10, 20

hemorrhages, pseudopapilledema 49

hypoplasia

See optic nerve hypoplasia

Leber's hereditary optic neuropathy 137–9

lesions, visual field defects 117

opacification of nerve fiber layer 47–8

sharpness of margins 48

tilted 121, 130

optic disc cupping (excavation) 10

differential diagnosis 10, 20

glaucoma 8–10, 19, 20

See also cup/disc (C/D) ratio

optic disc edema

acute unilateral visual loss with 181–4

elevated intracranial pressure

See papilledema

headache and bilateral 8

large monocular central scotoma 103

malignant hypertension 6

neuroretinitis 105–6

painful optic neuropathy 40

pallid, giant cell arteritis 194, 196

pseudo- 138–9

retinal abnormalities with bilateral 4–5

optic disc pallor

abnormal fields and temporal 122, 132–3

progressive optic neuropathy 60–1

optic nerve hypoplasia 51–3

associated abnormalities 51–3

double-ring (halo) sign 51–2

partial 50–3

risk factors 53

superior segmental 52–3

optic nerve sheath fenestration (ONSF)

idiopathic intracranial hypertension 192–3

NAION 198–9

optic nerve sheath meningiomas 61–3

optic neuritis

“chronic” 59–60

demyelinating (idiopathic) 199

prednisone therapy 199–200

vs acute idiopathic blind spot enlargement 17

vs Leber's hereditary optic neuropathy 137–9

vs neuroretinitis 103–4, 106–7

vs optic nerve sheath meningioma 61–2

vs optic perineuritis 40–3

time course 59–60

Optic Neuritis Treatment Trial (ONTT) 199–200

optic neuropathy

acute monocular 137, 139

chronic glaucomatous 8–10, 18, 19

compressive 61–3

diagnostic tests 2

and ipsilateral ophthalmoplegia 94–6

ischemic

See ischemic optic neuropathy

Leber's hereditary

See Leber's hereditary optic neuropathy

painful 40–3

progressive unilateral 59–63

time course 59–60

visual field defects 117

visual symptoms 1

optic neurotomy, radial 198

optic perineuritis (OPN) 40–3

vs optic neuritis 41–3

optical coherence tomography (OCT) 14

orbicularis oculi, myasthenic weakness 34

orbital apex syndrome 95–6

orbital disease 27–43

radiographic diagnosis 33

restrictive 27–34

orbital floor fracture, with muscle entrapment 29–32

orbital inflammatory disease

etiologies 40

idiopathic (orbital pseudotumor) 38–41

paranasal sinusitis and 88

presumptive diagnosis 163

orbital myositis

conjunctival injection 28

idiopathic 38–40

vs 6th nerve palsy 152–3

vs Graves’ orbitopathy 39

orbital venous congestion 107–9

oscillopsia 110–13

otolaryngology (ENT) evaluation 172–3

pain

chronic pink eye 107–8

on eye movement 27, 38–40, 152

orbital inflammatory disease 38–40

postganglionic Horner syndrome 70

See also eye pain, headache

painful mydriasis 10–13

panhypopituitarism 51–3

papilledema

atypical pseudotumor cerebri syndrome 80

headache and 63–6

idiopathic intracranial hypertension 86, 191–2, 194

shunt malfunction 175

vs malignant hypertension 4, 6–7, 49

vs optic disc drusen 47–50

See also optic disc edema, pseudopapilledema

paragangliomas 67–9

paramedian pontine reticular formation (PPRF) 114, 147, 165–6

paranasal sinus disease 87–88

paraneoplastic syndromes

cancer-associated retinopathy (CAR) 16, 22–3

downbeat nystagmus 111

parietal lobe syndrome, non-dominant, with negative neuroimaging 169–72

Parinaud's syndrome (dorsal midbrain syndrome) 143, 173–4, 177

parkinsonian syndromes, monocular diplopia 3

patent foramen ovale 123

pattern recognition 91–100

perimetry 117

Goldmann

See Goldmann perimetry

limitations of automated 120

retinitis pigmentosa 117, 120

See also visual field testing

periorbital swelling, Graves’ orbitopathy 81, 83

peripapillary microvascular angiopathy 138

pernicious anemia 157–8

petrous ridge meningioma 75–6

phenylephrine 205

photographs, old family 57

photophobia 21–3, 205

photopsias

abnormal field and 124, 134–5

after embolic stroke 158–60

gradual visual loss with 21

localizing features 14–17

persistent 14, 17

preceding acute visual loss 17–18

See also scintillations, visual

photostress test 2

pilocarpine 181, 205

pinhole test 2–3

pink eye

chronic 107–10

See also conjunctival injection

pituitary apoplexy 72–5

pituitary tumor

bitemporal visual field defect 118

hemorrhage into 73

longstanding 72

polyopia 3

cerebral 3–4

positron emission tomography (PET), Alzheimer's disease 169, 171

post-cardiac bypass visual loss 125–8

posterior ciliary artery embolism 183

posterior communicating artery (pCOM) aneurysms

acute third nerve palsy 163–4, 185

isolated unilateral mydriasis 180

ruptured 12

posterior ischemic optic neuropathy (PION) 17–18, 127

posterior reversible encephalopathy syndrome 7

prednisone

acute isolated painful 3rd nerve palsy 163

demyelinating optic neuritis 199–200

giant cell arteritis 195, 197

ocular myasthenia 201, 203

orbital inflammatory disease 38–40

orbital myositis 152

tapering 197

Tolosa Hunt syndrome 92

preeclampsia–eclampsia syndrome 7

pregnancy

acute diplopia 146–7

progressive visual loss 61–2

prion diseases 168–9

progressive supranuclear palsy (PSP)

Doll's head maneuver 150–1

magnetic resonance imaging 151

symptomatic treatment 205

vs chronic progressive external ophthalmoplegia (CPEO) 149–52

proptosis

absence, orbital apex syndrome 96

carotid-cavernous sinus fistula 107–8

detection 27–8

pseudo-bitemporal defects 119, 128–35

pseudo-internuclear ophthalmoplegia (INO) 113–14

pseudo-myopia 145–6

pseudopapilledema

buried optic disc drusen 45–50

vs papilledema 47–50

pseudotumor cerebri

See idiopathic intracranial hypertension

psychic paralysis of gaze 170

ptosis

acute painful ophthalmoplegia 91–2

aponeurotic 34–7

“boxer” 70–2

and diplopia

painful 37–40

painless 160–1, 200–1

fatigable 34–7

Horner syndrome 34, 67–8

and miosis, idiopathic 66–9

myogenic 35–7

neurogenic 35–7

senile 35

pupillary light reflex 143–4

pupillary near reflex 143–4

pupillary sphincter

See iris sphincter

pupils

Argyll Robertson, vs chronic tonic 142–4

dilatation lag 68, 70

oculomotor nerve palsy with aberrant regeneration 96–7

speed of responses 143

symptomatic treatment of disorders 205

tonic

See tonic pupils

unilateral non-reactive 12

See also anisocoria, miosis, mydriasis

pyridostigmine (Mestinon)

ocular myasthenia 200–3

side-effects 201–2

radial optic neurotomy 198

radiation therapy

6th nerve palsy after 173

metastatic breast cancer 95

optic nerve sheath meningioma 61–2

petrous ridge meningioma 75

radiography 59–82

incidental findings 85–90

midline and bilateral abnormalities 76–82

ordering wrong scan 59–69

subtle findings 70–6

true negatives 165–77

see also specific imaging modalities

ragged red fibers 152

RAPD

See relative afferent pupil defect

reading glasses, impaired downgaze 205

reading the paper, sudden difficulty 122–5, 131

recoverin antibodies 22

reinnervation stage, Adie's pupil 181

relative afferent pupil defect (RAPD)

large monocular central scotoma 103

Leber's hereditary optic neuropathy 137

neuroretinitis 104–5

progressive optic neuropathy 60–2

renal cell carcinoma 79

rest test, myasthenia gravis 160–1

restrictive orbitopathy 27–34

retinal artery occlusion 18–19

branch 13, 18

central

See central retinal artery occlusion

retinal detachment

central serous 104–5

malignant hypertension 6

retinal disease

bilateral optic disc edema with 4

invisible (occult) 13–24

ischemic 23–4

peripapillary 9, 14, 17

photopsias 16–17

visual symptoms 1

retinal edema, retinal artery occlusion 13, 18–19

retinal embolism 23–24, 70–2

retinal hemorrhages

increased intracranial pressure 4, 7

malignant hypertension 4, 6–7

retinal vasculature

optic disc drusen 48–9

situs inversus 121, 130

retinal veins

occlusion of central 109

tortuous 49, 107

retinitis pigmentosa 118–20, 127, 128

retinol

See vitamin A

retinopathy

acute zonal occult outer (AZOOR) 16

cancer-associated (CAR) 16, 22–3

hypertensive 4–6, 8

rod-cone dystrophy 125, 134–5

“ruling out” process 155

saccadic velocity

internuclear ophthalmoplegia 114

myasthenia gravis 31, 113–14

ocular motility disorders 30, 32

orbital myositis vs 6th nerve palsy 152

progressive supranuclear palsy 149, 151

sarcoidosis 155

Schilling test 157

scintillations, visual

episodic 186–8

persistent 14, 17

See also photopsias

scleral depression 2

scotomas

arcuate, painful optic neuropathy 40–1

bilateral ceco-central

dominant optic atrophy 123, 132–3

optic neuropathies causing 156

vitamin B-12 deficiency 156

ceco-central monocular 137

episodic scintillating 186–8

homonymous hemianopic

See homonymous hemianopia

junctional 91

large central monocular 103–7

monocular 14

orbital apex syndrome 95

right homonymous superior quadrantopic 158–9

ring 118, 127

twinkling 9, 14–17

See also visual field defects

scrapie 168

sectoral sphincter palsy 140–2

sectoranopia

homonymous horizontal 98–100

quadruple 98, 100

seizures, occipital 16

sella turcica, enlarged

See empty sella

septo-optic dysplasia 51

shunting

idiopathic intracranial hypertension 193

See also ventriculoperitoneal shunt

simultanagnosia 170

sinus disease 87–8

situs inversus, retinal vessels 121, 130

sixth (abducens) nerve

imaging of full course 76

nucleus, lesions 165–6

sixth nerve palsy

acute isolated 184–6

after radiation therapy 173

aneurysmal 186

bilateral idiopathic 76–80

causes of bilateral 77

chronic isolated 185–6

congenital 53–4

convergence spasm vs bilateral 144–6

restrictive orbitopathy simulating 30

slowly progressive 75–6

sphenoid sinus mucocele 87

vasculopathic (ischemic) 77, 184–5

vertebrobasilar dolichoectasia 88–9

vs orbital myositis 152–3

skull base

imaging 77–9

tumors, with negative scans 172–3

sleep apnea, obstructive 198

sleep test, myasthenia gravis 160–1

slit ventricle syndrome 174

small vessel disease 98–9

smell, decreased sense of 85

spasm of near reflex 144–6

spatial organization deficits 169, 171

spheno-cavernous syndrome

See cavernous sinus/superior orbital fissure syndrome

sphenoid sinus mucocele 87–8

sphenoid wing meningioma 63

splinter hemorrhages

on disc margin (Drance hemorrhage) 10, 20

malignant hypertension 5–6

square wave jerks, macro 151

Stargardt's disease 14

stellate maculopathy 104

steroids

See corticosteroids

strabismus surgery 203–4

stroke

chronic 6th nerve palsy and 186

downbeat nystagmus 111

internal carotid artery dissection 72

lateral geniculate body 98–100

occipital

See occipital stroke

occipital tip

See occipital tip stroke

twinkling after embolic 158–60

Wernicke's encephalopathy vs brainstem 146–9

subarachnoid hemorrhage 110

subtle clinical findings 103–14

sudden difficulty reading the paper 122–5, 131

superior colliculus syndrome

See Parinaud's syndrome

superior oblique palsy, congenital 54–7

superior ophthalmic vein

dilation 107–9

shunting of blood through 108

thrombosis 110

superior orbital fissure lesions 91, 95

See also cavernous sinus/superior orbital fissure syndrome

superior sagittal sinus thrombosis 64–5, 81–2

sympathetic pathway, to eye 66–7

symptomatic treatment, failure to provide 203–5

syphilis, tertiary 142–3

tearing, excessive 3

temporal artery biopsy, false-negative 155, 195

Tensilon test 161

positive 113, 114, 160–161

reluctance to perform 160–1

tests

false negative results 155–77

over-ordering 179–89

therapeutic nihilism 191

thiamine

deficiency 147–9

trial of therapy 148

third (oculomotor) nerve, divisions 37

third (oculomotor) nerve palsy

with aberrant regeneration (oculomotor synkinesis) 96–8

acute bilateral painful 72–5

acute isolated painful

causes 162–3

with negative neuroimaging 162–5

acute painful, with mydriasis 12

aneurysmal 163–5, 185

isolated partial 31

with isolated unilateral mydriasis 180

progressive isolated painful 172–3

with pupil sparing 185

superior division 37–8

vasculopathic (ischemic) 185

vs restrictive orbitopathy 29, 31

three-step test, Bielschowski 55–6

thrombocytosis, essential 81

thrombolytic therapy 72, 74

thymectomy 203

thyroid eye disease (Graves’ orbitopathy)

lid retraction 28, 81

mild radiographic changes in symmetric 81–3

monocular diplopia 3

painless vertical diplopia 32–4

restrictive orbitopathy 30–1

vs carotid-cavernous sinus fistula 109

vs orbital myositis 39

thyroid function tests 33–4

tilted disc syndrome 121, 130

tinnitus, pulsatile 107, 110

Tolosa Hunt syndrome (THS) 91–4

diagnostic criteria 92–3

magnetic resonance imaging 91–4

tonic pupils

acute, vs pharmacologic mydriasis 139–42, 180

causes 181

chronic, vs Argyll Robertson pupils 142–4

idiopathic

See Adie's tonic pupil

sectoral sphincter palsy 140–2

top-of-the-basilar syndrome 148

topiramate 192

topless disc syndrome (superior segmental hypoplasia) 53

transient ischemic attacks (TIA), photopsias 16

transient monocular visual loss (TMVL) 23–4

corneal decompensation 23–4

internal carotid artery dissection 70

retinal vascular disease 18, 23–4

transmissible mink encephalopathy 168

trauma

internal carotid artery dissection 70, 72

restrictive orbitopathy after 29–31

triamcinolone 198

trochlear nerve

See fourth nerve

twinkling after embolic stroke 158–60

Uhthoff's phenomenon, vs Chiari I malformation 112

ultrasonography, optic disc drusen 46, 49–50

uncal herniation, constricted fields after 120–2

upgaze deficit, incidental 27–32

upgaze palsy 173–7

Valsalva maneuver, headache provoked by 112

vascular risk factors 185, 198

vasculopathic cranial mononeuropathy 184–6

ventriculoperitoneal shunt

constricted visual fields 120, 129, 131

idiopathic intracranial hypertension 193

malfunction, without ventriculomegaly 173–7

vertebrobasilar dolichoectasia 88–90

vertical fusional amplitudes 55, 57

vestibulo-ocular reflex (Doll's head maneuver), progressive supranuclear palsy 150–1

vision

blurring

See blurring of vision

bouncing 110–13

degradation in light

See hemeralopia

dimming or darkening 1

double

See diplopia

hazy night 19–21

missing areas 1

swirling 21–3

visual field defects 117–28

bitemporal

See bitemporal visual field defects

cancer-associated retinopathy 16, 21, 23

constricted fields after herniation 120–2, 129

glaucoma 8, 10, 18

incidental 119, 128–30

inferior altitudinal

anterior ischemic optic neuropathy 181, 183

partial optic nerve hypoplasia 50–3

localizing features 117

malignant hypertension 4–5

night blindness and 117–20, 126

non-organic vs organic constriction 121–2

photopsias and 124, 134–5

post-cardiac bypass 125–8

progressive optic neuropathy 60–1

pseudo-bitemporal defects 128–35

right-sided 98–100

severe constriction, causes 121–2

sudden difficulty reading the paper 122–25, 131

temporal disc pallor and 122, 132–3

“tunnel” or “gun-barrel” 121

See also scotomas

visual field testing 117

abnormal fields and temporal disc pallor 132–3

central 123, 125, 127

hypovitaminosis A 15, 21

idiopathic intracranial hypertension 194

non-organic vs organic constricted fields 121–2

twinkling scotoma 9, 14

vertical alignment of temporal field 119, 130

See also perimetry

visual loss

monocular

See monocular visual loss

non-organic 156, 188–9

optic disc drusen 47

pituitary apoplexy 73

progressive 59–63

sequential, with “swirling clouds of smoke” 21–3

time course 59–60

transient, atypical pseudotumor cerebri syndrome 80

unexplained 156–8, 188–9

See also blurring of vision

visual pathways, afferent 117

vitamin A (retinol) 21

deficiency 15, 16, 19–21

serum 21

vitamin B-1

See thiamine

vitamin B-12

deficiency 156–8, 179

dietary intake 157

serum testing 156–8, 179

therapy 157

Wallace mutation 137

Wegener's granulomatosis 155

weight reduction

bariatric surgery 193

idiopathic intracranial hypertension 192

See also obesity

Wernicke's encephalopathy 147–9

risk factors 147–8

vs brainstem stroke 146–9

white-eyed blowout 29

white-eyed shunt 108




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