- •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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Figure 8.8 Follow-up macular visual field testing (central 10 degrees) one year later shows near-complete resolution of this patient’s previous homonymous defect.
missing information in the scotoma is compensated by information obtained from the rest of the visual field.
Due to the very small size and central location of the homonymous scotoma, it can be overlooked on standard automated threshold tests, dismissed as a non-specific parafoveal depression or misinterpreted as a test artifact from unstable fixation. Amsler grid testing is particularly useful in detecting such subtle visual field defects, provided that visual acuity is around 20/40 or better. The most commonly used Amsler grid consists of a series of parallel black lines on a white background, forming a grid of 400 squares covering a 10 cm × 10 cm area. When the patient views the fixation dot in the center of the grid with one eye at a distance of 30 cm, each square represents 1 degree of visual angle and the test assesses the central 10 degrees of the visual field. The patient is asked to fixate on the center of the grid and mark with a pencil any regions where the lines are blurred, missing, or distorted. In this way, the patient can map out his/her own paracentral scotoma. As with other psychophysical tests, the reliability of the Amsler grid test depends on patient comprehension and
cooperation. Other effective methods for assessing the central field are Goldmann perimetry (using the smallest visible stimulus), central automated fields like the Humphrey 20–10 or Octopus M2 and the tangent screen.
Diagnosis: Small homonymous scotoma due to occipital stroke
Tip: A small homonymous hemianopic scotoma can often be suspected from the history. The Amsler grid is a quick and effective method for assessing the central visual field in such cases.
Post-cardiac bypass visual loss
Case: A 63-year-old machinist experienced bilateral visual loss upon awakening after coronary artery bypass surgery. Visual acuity was 20/200 OU with markedly decreased color vision. By confrontation he appeared to have bilateral central scotomas, stating that he could see the examiner’s hair, chin and ears but not eyes, nose and mouth. Ischemic optic neuropathy was suspected but the pupillary responses and optic disc appearance were normal.
126 Chapter 8: Misinterpretation of visual fields
A
B
Figure 8.9 Goldmann perimetry in the above patient with bilateral visual loss following cardiac surgery. (A) His initial visual field was interpreted as showing bilateral central scotomas. (B) On closer inspection, these defects are actually bilateral homonymous hemianopic scotomas with a small mismatch along the vertical meridian.
Is there another possible explanation for this patient’s visual loss, and how would you investigate this alternative mechanism?
Infarction of the retrobulbar segment of the optic nerves, called posterior ischemic optic neuropathy, would be consistent with a normal fundus appearance acutely. However, the presence of brisk pupil reflexes is not. The combination of marked bilateral central visual loss and normal pupillary responses suggests cortical visual loss, specifically due to a lesion involving the occipital tips. Initial examination of the field with Goldmann perimetry suggested bilateral central scotomas with an odd vertically ovoid shape (Figure 8.9A). Based on this appearance and the other clinical features, the central portion of the field was tested again, this time including careful exploration of the vertical meridian to either side of fixation. With this technique it was found that what appeared to be bilateral central scotomas were indeed matched, bilateral, homonymous hemianopic scotomas in the central field with a small vertical step between the two sides (Figure 8.9B). As expected based on the clinical findings, a CT scan revealed bilateral infarcts at the occipital tips (Figure 8.10). Based on the clinical context and radiographic appearance, the mechanism was presumed to be embolic.
Discussion: The topographic representation of information carried in the afferent visual pathways is displayed within the occipital cortex in a very precise arrangement. The central (macular) visual field is transmitted to the posterior aspect of the occipital lobes, also termed the “occipital tip”. Information from the peripheral field is displayed more anteriorly, adjacent to the genu of the corpus callosum. The occipital lobes are separated by the interhemispheric fissure, and each receives its own blood supply. While damage to the occipital tips can produce bilateral homonymous defects, it is unlikely that the right and left defects would be perfectly symmetric. The resultant scotomas, therefore, show a mismatch along the vertical meridian, as in the above case. Because the posterior cerebral
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Figure 8.10 Axial post-contrast CT of the head shows areas of low density at the occipital tip bilaterally with adjacent gyriform enhancement on the right side.
arteries that supply the occipital lobes arise from a common trunk, the basilar artery, it is not uncommon for emboli traveling in this arterial system to arrive in both occipital lobes.
Although a unilateral post-geniculate lesion never affects visual acuity (still normal in the intact hemifield), a bilateral lesion involving macular fibers does produce loss of acuity of varying degree that is always symmetric in the two eyes. The resultant bilateral central visual loss often suggests bilateral optic neuropathy or maculopathy as the cause, and the finding of bilateral central scotomas furthers this impression. In such cases, careful inspection of the contours of the central scotomas, with particular attention to each side of the vertical meridian, should provide the correct localization. Goldmann perimetry is particularly well suited for such exploration but an automated field that concentrates on the central field, such as the Humphrey 10–2 program, should also provide comparable information.
