- •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
and ranges from mild to profound. The non-organic nature of the visual loss is most easily demonstrated when one eye is severely affected, typically by using techniques that “fog” the fellow eye, as in the case under discussion. In the crossed cylinder method, minus and plus cylinders of equal magnitudes are aligned in a trial frame over the “good” eye so that they cancel out optically. The patient is asked to read the Snellen chart with both eyes open, starting from the largest letters and reading down to the smallest. As the patient proceeds, the examiner turns one of the cylinders until the good eye is sufficiently blurred that the patient could only be reading with the eye that has the unexplained visual loss. Similar fogging can be achieved with increasing plus lenses in the phoropter, but with that technique the examiner loses the opportunity to observe the patient during the test and, in addition, the movement of the lenses is less subtle than the motion of the crossed cylinders. Red-green duochrome lenses can provide similar information, but in most cases the best acuity one can obtain with this method is around 20/40. Measurement of stereo-acuity can be a helpful adjunct for determining intact near vision. In cases involving both eyes or in those with visual loss in an only good eye, fogging techniques are not applicable. In such cases it is often possible to demonstrate better vision by working from the smallest letters on the Snellen chart up, using encouragement and the power of suggestion.
In cases of non-organic visual loss, the ideal test is one that demonstrates normal vision, but sometimes we must settle instead for showing gross inconsistencies. Examples of this include the ability to navigate about the room or reach for objects despite claimed blindness, intact central visual field in the face of severe loss of acuity, and apparent total monocular blindness with an intact pupillary response.
Diagnosis: Non-organic visual loss
Tip: In most cases of non-organic visual loss, specific examination techniques can disclose the
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nature of the disorder and thereby obviate the need for extensive and costly evaluation.
FURTHER READING
Adie’s tonic pupil
D. M. Jacobson, Pupillary responses to dilute pilocarpine in preganglionic 3rd nerve disorders. Neurology, 40 (1990), 804–8.
R.H. Kardon, J. J. Corbett, H. S. Thompson, Segmental denervation and reinnervation of the iris sphincter as shown by infrared videographic transillumination. Ophthamo-
logy, 105 (1998), 313–21.
A. Kawasaki, Disorders of pupillary function, accommodation and lacrimation. In N. R. Miller, N. J. Newman, V. Biousse, J. B. Kerrison, eds., Walsh and Hoyt’s Clinical Neuro-Ophthalmology, 6th edn. Philadelphia: Lippincott Williams and Wilkins, 2005, Vol. 1, Chapter 16, pp. 739– 805.
Non-arteritic anterior ischemic optic neuropathy
A.C. Arnold, Pathogenesis of nonarteritic anterior ischemic optic neuropathy. Ophthalmol Clin N Am, 14 (2001),
83–98.
S.Lessell, Nonarteritic anterior ischemic optic neuropathy: enigma variations. Arch Ophthalmol, 117 (1999), 386–8.
V. Purvin, Ischemic optic neuropathy. Semin Cerebrovasc Dis Stroke, 4 (2004), 2–17.
Vasculopathic cranial mononeuropathy
K.L. Chou, S. L. Galetta, G. T. Liu et al., Acute ocular motor mononeuropathies: prospective study of the roles of
neuroimaging and clinical assessment. J Neurol Sci, 219 (2004), 35–9.
D. M. Jacobson, Progressive ophthalmoplegia with acute ischemic abducens nerve palsies. Am J Ophthalmol, 122 (1996), 278–9.
D. M. Jacobson, T. D. McCanna, P. M. Layde, Risk factors for ischemic ocular motor palsies. Arch Ophthalmol, 112 (1994), 961–6.
J. T. Kissel, R. M. Burde, T. G. Klingele, H. E. Zeiger, Pupil-sparing oculomotor palsies with internal carotidposterior communicating artery aneurysms. Ann Neurol, 13 (1983), 149–54.
190 Chapter 11: Over-ordering tests
S.K. Sanders, A. Kawasaki, V. Purvin, Long-term prognosis in patients with vasculopathic sixth nerve palsy. Am
J Ophthalmol, 134 (2002), 81–4.
J.D. Trobe, Third nerve palsy and the pupil: footnotes to the rule. Arch Ophthalmol, 106 (1988), 601–2.
Migraine
R.A. Davidoff. Migraine. Manifestations, Pathogenesis, and Management, 2nd edn. Oxford: Oxford University Press, 2002.
S.L. Hupp, L. B. Kline, J. J. Corbett, Visual disturbances of migraine. Surv Ophthalmol, 33 (1989), 221–36.
Non-organic visual loss
K. K. Kramer, F. G. La Piana, B. Appleton, Ocular malingering and hysteria: diagnosis and management. Surv Ophthalmol, 24 (1979), 89–96.
B. W. Miller, A review of practical tests for ocular malingering and hysteria. Surv Ophthamol, 17 (1973), 241–6.
H. S. Thompson, Functional visual loss. Am J Ophthalmol, 100 (1985), 209–13.
