- •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
186 Chapter 11: Over-ordering tests
A B
Figure 11.3 (A) Patient who suffered an acute left middle cerebral artery stroke. He had been followed for a chronic left sixth nerve palsy for the preceding 10 years but without neuro-imaging. In addition to his esotropia, notice his left Horner syndrome and right central facial weakness. (B) Axial post-contrast CT shows a giant, partially thrombosed, left intracavernous internal carotid artery aneurysm.
a vasculopathic palsy is a marker for atherosclerotic disease, daily aspirin is appropriate as prophylaxis for other ischemic events such as stroke and heart attack. Some patients experience a subsequent similar episode involving the same cranial nerve, another ocular motor nerve or a facial palsy. Even in the case of such recurrence, the prognosis for ultimate recovery is excellent.
Diagnosis: Vasculopathic cranial mononeuropathy
Tip: The diagnosis of a vasculopathic cranial nerve palsy is a clinical one. Ancillary testing should be reserved for patients with an atypical clinical course.
Episodic scintillating scotoma
Case: A 55-year-old teacher had experienced several episodes of transient binocular visual loss over
the past two years. She described “shimmering” vision that began close to fixation and spread out to the periphery over 20 minutes. Most of these episodes occurred in the right hemifield, but occasional attacks were on the left. There were no other focal deficits, altered consciousness or headache associated with the visual loss. She was generally in good health and taking no medications. She reported a remote history of “sick headaches” without visual disturbance that were usually associated with menses and remitted in her late twenties after her first pregnancy. Her neurologic examination was normal including visual fields full to confrontational testing.
Does this patient need neuro-imaging? An EEG? Other investigation?
The slow spread of the scintillations across the visual field described by this patient is considered
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pathognomonic of migraine. The fact that her visual disturbance sometimes switches sides further rules out a structural lesion. In this case, no additional testing is needed.
Discussion: Migraine with or without visual aura is extremely common, with a lifetime prevalence of 12 to 33% in women and 4 to 22% in men. Many patients with migraine present to primary care physicians, others to ophthalmologists, neurologists or other healthcare providers. Not all patients with migraine require neuro-imaging or other ancillary testing and it is helpful for the clinician to have a set of guidelines for deciding when such work-up is needed.
The presence or absence of headache is not a useful criterion since migraine visual aura without headache is a common occurrence, termed acephalgic migraine or migraine equivalent in the older literature. Moreover, 20% of vertebrobasilar transient ischemic attacks and even occasional seizures are accompanied by headache. The temporal and spatial characteristics of the visual episode are much more helpful. As noted previously (see Chapter 1, Twinkling scotoma), migrainous visual phenomena are usually positive (i.e. seeing something that is not there rather than not seeing something that is there) and usually include a quality of motion (shimmering, sparkling or vibrating). These migrainous scintillations often start adjacent to fixation and spread to the periphery on one side over 20 minutes (Figure 11.4). This slow spread or “march” corresponds to the speed of the spreading cortical depression that underlies an attack and is generally considered to be pathognomonic for migraine. There are rare cases in which this characteristic pattern of scintillations has been associated with a structural lesion (specifically, an occipital arteriovenous malformation or tumor), and it is believed that in these individuals a mechanical stimulus has provoked an episode of migraine. A helpful tip-off in such rare cases is the fact that attacks occur exclusively in the same hemifield. About 20% of patients with migraine report their attacks as always being on the same side (termed
Figure 11.4 ]Migraine visual aura. Typical scintillating scotoma is shown at varying time intervals after onset. The “X” in each instance indicates the visual fixation point and the numbers represent minutes. (From K. S. Lashley, Arch Neurol Psychiatr, 46 (1941), 331–9, with permission.)
“side-locked” migraine), so this feature alone does not guarantee the presence of a structural lesion but the converse is very helpful: attacks that switch sides are not due to an underlying mass lesion.
Migraine attacks are frequently precipitated by an internal or external trigger, and eliciting this history can be helpful in diagnosis as well as treatment. Stress and hormonal change are the most common triggers. The actual migraine episode typically occurs not during the period of stress but in the letdown phase that follows. Hormonal triggers most often involve a decline in estrogen levels such as just prior to menses and during peri-menopause when estrogen secretion tends to be erratic. Other triggers include skipping meals, sleeping late, strong smells, bright lights, caffeine withdrawal and certain foods. In some cases, more than one trigger is required to precipitate an episode; for example, missing breakfast and coffee because one is running late for a morning appointment. In its fullfledged form the headache is severe and associated with nausea, vomiting and a heightened sensitivity to light and sound; however not all migraine attacks have these characteristics. Migraine headaches are usually relieved by sleep and often by triptan medications. In the vast majority of cases, the focal
