- •Contents
- •Foreword
- •Preface
- •Acknowledgements
- •1 When ocular disease is mistaken for neurologic disease
- •Double images
- •What important piece of historical information is still missing in this case?
- •Diagnosis: Monocular diplopia due to cataract
- •Headache and bilateral disc edema
- •What test was done and what was the diagnosis?
- •Diagnosis: Malignant hypertension
- •Chronic optic neuropathy
- •Diagnosis: Glaucomatous optic neuropathy
- •Painful mydriasis
- •What clues suggest an alternative diagnosis?
- •Diagnosis: Acute angle closure glaucoma
- •Invisible retinal disease
- •Twinkling scotoma
- •What aspect of this patient’s positive visual phenomenon is highly atypical for migraine?
- •Diagnosis: Acute idiopathic blindspot enlargement
- •Sudden monocular visual loss with normal fundus
- •Hazy night vision
- •Diagnosis: Hypovitaminosis A
- •Swirling vision
- •Diagnosis: Cancer-associated retinopathy
- •Episodic monocular blur
- •FURTHER READING
- •Monocular diplopia
- •Hypertensive retinopathy
- •Twinkling scotoma
- •Central retinal artery occlusion
- •Hypovitaminosis A
- •Cancer-associated retinopathy
- •Corneal decompensation
- •Glaucoma
- •2 When orbital disease is mistaken for neurologic disease
- •Painless vertical diplopia
- •Diagnosis: Euthyroid Graves’ disease
- •Fatigable ptosis
- •How is lid fatigability objectively demonstrated?
- •Diagnosis: Levator dehiscence
- •Painful ptosis and diplopia
- •The investigation thus far has revealed no intracranial pathology. How would you proceed?
- •Painful optic neuropathy
- •Is this patient’s clinical course consistent with a diagnosis of optic neuritis?
- •Diagnosis: Idiopathic optic perineuritis
- •FURTHER READING
- •Orbital examination and restrictive orbitopathy
- •Levator dehiscence
- •Painful ptosis and diplopia
- •Optic perineuritis
- •3 Mistaking congenital anomalies for acquired disease
- •Headaches and elevated discs
- •Are there clues to the correct diagnosis in this case?
- •Diagnosis: Superior segmental hypoplasia
- •Diagnosis: Type I Duane’s syndrome
- •Intermittent vertical diplopia
- •What other causes of fourth nerve palsy should be considered?
- •How would you pursue a diagnosis of congenital fourth nerve palsy in this patient?
- •Diagnosis: Congenital fourth nerve palsy
- •FURTHER READING
- •Pseudopapilledema
- •Superior segmental hypoplasia
- •Duane’s syndrome
- •Congenital superior oblique palsy
- •4 Radiographic errors
- •Ordering the wrong scan
- •Progressive optic neuropathy
- •Is there a problem with the diagnosis of “chronic optic neuritis”?
- •What clinical features in this case suggest the likely mechanism of her chronic optic neuropathy?
- •What additional radiographic evaluation should be obtained?
- •Headache and papilledema
- •Diagnosis: Cerebral venous sinus thrombosis
- •Idiopathic ptosis and miosis
- •Why is the current study incomplete?
- •Diagnosis: Postganglionic Horner syndrome
- •Diagnosis: Internal carotid artery dissection
- •Headache and bilateral third nerve palsy
- •Diagnosis: Pituitary apoplexy
- •Progressive sixth nerve palsy
- •What aspect of this patient’s presentation provides the most compelling diagnostic clue?
- •Diagnosis: Petrous ridge meningioma
- •Midline and bilateral abnormalities
- •Bilateral idiopathic sixth nerve palsy
- •Is a diagnosis of vasculopathic sixth nerve palsy still tenable here?
- •Diagnosis: Clivus tumor
- •Atypical pseudotumor cerebri syndrome
- •What features of this case are atypical for a diagnosis of IIH? What alternative diagnosis should be considered?
- •Diagnosis: Superior sagittal sinus thrombosis
- •Vertical diplopia
- •Diagnosis: Symmetric Graves’ disease
- •FURTHER READING
- •Neuro-imaging
- •Canalicular meningioma
- •Cerebral venous thrombosis
- •Horner syndrome and carotid dissection
- •Chronic sixth nerve palsy
- •Empty sella
- •Low cerebellar tonsils
- •Sphenoid sinus mucocele
- •Dolichoectatic basilar artery
- •FURTHER READING
- •Pseudotumor cerebri syndrome
- •Chiari malformation
- •Sphenoid sinus mucocele
- •Dolichoectatic basilar artery
- •6 Failure of pattern recognition
- •Painful ophthalmoplegia
- •Where is this patient’s lesion?
- •Diagnosis: Tolosa Hunt syndrome
- •Painful ophthalmoplegia and visual loss
- •Diagnosis: Orbital apex syndrome
- •Painless diplopia
- •Diagnosis: Oculomotor nerve palsy with aberrant regeneration
- •Diagnosis: Lateral geniculate body stroke
- •FURTHER READING
- •Painful ophthalmoplegia
- •Orbital apex syndrome
- •Third nerve misdirection
- •Lateral geniculate body
- •Painless central gray spot in a teenager
- •What is the most likely cause of this patient’s neuroretinitis, and how would you test for it?
- •Diagnosis: Neuroretinitis due to cat scratch disease
- •This patient had an additional non-ocular symptom which she did not volunteer because she didn’t think it was relevant to her eye problem, yet this symptom was an important clue to the correct diagnosis. What question should be asked?
- •Bouncing vision
- •What examination techniques can help in the detection of nystagmus when the oscillatory amplitude is particularly small?
- •Diagnosis: Downbeat nystagmus due to Chiari I malformation
- •Diagnosis: Myasthenic pseudo-INO
- •FURTHER READING
- •Neuroretinitis
- •Downbeat nystagmus
- •Diagnosis: Retinitis pigmentosa
- •Diagnosis: Bilateral occipital stroke with macular sparing
- •What simple “bedside” test could be performed to further investigate this patient’s symptom?
- •Diagnosis: Small homonymous scotoma due to occipital stroke
- •Post-cardiac bypass visual loss
- •Is there another possible explanation for this patient’s visual loss, and how would you investigate this alternative mechanism?
- •Diagnosis: Bilateral homonymous hemianopic scotomas secondary to bilateral occipital tip strokes
- •Pseudo-bitemporal defects
- •What is the next step in this patient’s evaluation?
- •Diagnosis: Tilted disc syndrome
- •Diagnosis: Dominant optic atrophy
- •Diagnosis: Rod-cone dystrophy
- •FURTHER READING
- •Tilted disc syndrome
- •Dominant optic atrophy
- •9 Neuro-ophthalmic look-alikes
- •Does his clinical course change your mind about the diagnosis?
- •Acute tonic pupil vs. pharmacologic mydriasis
- •Chronic tonic pupils vs. Argyll Robertson pupils
- •Convergence spasm vs. bilateral sixth nerve palsies
- •What metabolic abnormality can produce this clinical picture?
- •Chronic progressive external ophthalmoplegia vs. progressive supranuclear palsy
- •This combination of horizontal and vertical gaze limitation with slowed saccades could be due to either supranuclear gaze palsy or ocular myopathy. How can we distinguish these two mechanisms?
- •Orbital myositis vs. sixth nerve palsy
- •FURTHER READING
- •Optic neuritis vs. Leber’s hereditary optic neuropathy
- •Acute unilateral mydriasis
- •Light near dissociation
- •Convergence spasm
- •Wernicke’s encephalopathy
- •Progressive supranuclear palsy
- •Sixth nerve palsy vs. orbital myositis
- •10 Over-reliance on negative test results
- •Unexplained visual loss
- •Diagnosis: Pernicious anemia with normal serum B12 level
- •Twinkling after embolic stroke
- •Diagnosis: Digoxin toxicity with therapeutic levels
- •Painless ptosis and diplopia
- •Headache and third nerve palsy
- •What additional test should be obtained?
- •Diagnosis: Aneurysmal third nerve palsy
- •Truly negative neuro-imaging
- •Brainstem syndrome with negative scan
- •Can you localize this patient’s lesion?
- •Homonymous hemianopia with negative neuro-imaging
- •What disease processes would you consider here?
- •Non-dominant parietal lobe syndrome with negative neuro-imaging
- •Can you localize this patient’s problem?
- •Diagnosis: Visual variant of Alzheimer’s disease
- •Progressive third nerve palsy
- •What other investigations might be helpful?
- •Diagnosis: Third nerve palsy secondary to nasopharyngeal carcinoma
- •Upgaze palsy
- •Diagnosis: Shunt malfunction in the absence of ventriculomegaly
- •FURTHER READING
- •Digoxin toxicity
- •Myasthenia
- •Aneurysmal third nerve palsy
- •One-and-a-half syndrome
- •Cortical visual loss with negative neuro-imaging
- •Skull base tumors with negative imaging
- •Shunt failure with negative neuro-imaging
- •11 Over-ordering tests
- •Isolated unilateral mydriasis
- •If an isolated, enlarged and poorly reactive pupil is not a sign of a pCOM aneurysm, what other causes should be considered?
- •Diagnosis: Adie’s tonic pupil
- •Acute unilateral visual loss with disc edema
- •Diagnosis: Non-arteritic anterior ischemic optic neuropathy (NAION)
- •Acute isolated sixth nerve palsy
- •What is the most likely diagnosis and what evaluation would be appropriate?
- •Diagnosis: Vasculopathic cranial mononeuropathy
- •Episodic scintillating scotoma
- •Does this patient need neuro-imaging? An EEG? Other investigation?
- •Diagnosis: Migraine aura
- •Unexplained visual loss
- •What feature in this case suggests nonorganic visual loss? Is additional ancillary testing needed?
- •Diagnosis: Non-organic visual loss
- •FURTHER READING
- •Adie’s tonic pupil
- •Non-arteritic anterior ischemic optic neuropathy
- •Vasculopathic cranial mononeuropathy
- •Migraine
- •Non-organic visual loss
- •12 Management misadventures
- •Management of idiopathic intracranial hypertension
- •Evaluation and treatment of giant cell arteritis
- •Overzealous treatment of blood pressure in NAION
- •Prednisone for demyelinating optic neuritis
- •Over-reliance on pyridostigmine bromide (Mestinon) in ocular myasthenias
- •Failure to provide symptomatic treatment
- •FURTHER READING
- •Idiopathic intracranial hypertension
- •Giant cell arteritis
- •Non-arteritic anterior ischemic optic neuropathy
- •Optic neuritis
- •Ocular myasthenia
- •Nystagmus
- •Index
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Common Neuro-Ophthalmic Pitfalls
Common
Neuro-Ophthalmic
Pitfalls
Case-Based Teaching
Valerie A. Purvin
Aki Kawasaki
CAMBRIDGE UNIVERSITY PRESS
Cambridge, New York, Melbourne, Madrid, Cape Town, Singapore, São Paulo
Cambridge University Press
The Edinburgh Building, Cambridge CB2 8RU, UK
Published in the United States of America by Cambridge University Press, New York
www.cambridge.org
Information on this title: www.cambridge.org/9780521713269
© V. A. Purvin and A. Kawasaki 2009
This publication is in copyright. Subject to statutory exception and to the provision of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of Cambridge University Press.
First published in print format 2009
ISBN-13 978-0-511-47921-2 eBook (EBL)
ISBN-13 978-0-521-71326-9 paperback
Cambridge University Press has no responsibility for the persistence or accuracy of urls for external or third-party internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate.
Every effort has been made in preparing this publication to provide accurate and up-to-date information which is in accord with accepted standards and practice at the time of publication. Although case histories are drawn from actual cases, every effort has been made to disguise the identities of the individuals involved.
Nevertheless, the authors, editors, and publishers can make no warranties that the information contained herein is totally free from error, not least because clinical standards are constantly changing through research and regulation. The authors, editors, and publishers therefore disclaim all liability for direct or consequential damages resulting from the use of material contained in this publication. Readers are strongly advised to pay careful attention to information provided by the manufacturer of any drugs or equipment that they plan to use.
Contents
Foreword |
page vii |
|
Preface |
ix |
|
Acknowledgements |
xi |
|
1 |
When ocular disease is mistaken for |
|
|
neurologic disease |
1 |
2 |
When orbital disease is mistaken for |
|
|
neurologic disease |
27 |
3 |
Mistaking congenital anomalies for |
|
|
acquired disease |
45 |
4 |
Radiographic errors |
59 |
5 |
Incidental findings (seeing but not |
|
|
believing) |
85 |
6 |
Failure of pattern recognition |
91 |
7 |
Clinical findings that are subtle |
103 |
8 |
Misinterpretation of visual fields |
117 |
9 |
Neuro-ophthalmic look-alikes |
137 |
10 |
Over-reliance on negative test results |
155 |
11 |
Over-ordering tests |
179 |
12 |
Management misadventures |
191 |
Index |
207 |
|
v
Foreword
If you have already bought this book, you made the right choice. If you are just browsing through it and trying to decide whether to buy it, you must be tempted. Go ahead – you will not be disappointed.
This is the work of two gifted clinicians. With deep credentials in academic neuro-ophthalmology and frequent performances on the lecture circuit, they are highly respected in their field. What makes them especially distinctive is that they not only understand neuro-ophthalmic disease, they catch its finest nuances and they know how to share them – and teach them.
If you think you need a straight-up textbook to learn this material, think again. Reading a textbook might be like using a guidebook to go through an art museum. Reading this book is like having a personal guide who is both erudite and passionate. It is much more fun.
The subject matter is all cases – real cases that the authors have grappled with. Each illustrates a critical problem in neuro-ophthalmic diagnosis or management. Because we learn best when we make mistakes, the authors have selected cases in which someone stumbled.
The cases are presented as mystery stories. Whether you are a neuro-ophthalmic novice or a sophisticate, an ophthalmologist or a neurologist, a physician in training or a physician in practice, you will enjoy matching your wits against these gurus!
Jonathan D. Trobe, MD
University of Michigan
vii
