- •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
32 Chapter 2: Orbital disease or neurologic disease?
A B
Figure 2.9 A 62-year-old man with chronic painless vertical diplopia. (A) There is markedly limited elevation of the right eye when abducted. Depression is full and the eyes are aligned on downgaze. (The pupils have been pharmacologically dilated.) (B) Axial contrast-enhanced T1-weighted MRI of the head shows no cause for his diplopia.
in primary position and normal saccadic velocity in the direction of paresis should suggest restrictive orbitopathy, even in the absence of typical orbital signs and symptoms.
Painless vertical diplopia
Case: This 62-year-old engineer had a one-year history of painless vertical diplopia that remained undiagnosed despite a variety of neurodiagnostic tests. After some initial progression he felt that his double vision had remained stable. His medical history was unremarkable except for wellcontrolled hypertension. Neuro-ophthalmic examination showed a 30-diopter left hypertropia in primary position with limitation of right eye elevation that was most marked with the eye abducted (Figure 2.9A). Infraduction was full and saccadic velocities were normal, as was the remainder of the
examination. A CBC, ESR, acetylcholine-receptor antibody titers and thyroid function tests were normal, as was a brain MRI (Figure 2.9B).
What clinical features help to localize the source of this patient’s ocular motility disorder?
The normal saccadic velocity in the face of marked limitation of eye movement speaks strongly against supranuclear or cranial nerve dysfunction and points instead to either myasthenia or orbital restrictive disease (see Chapter 7, Farmer with an adduction deficit). The absence of variability over a one-year time period would be unusual for myasthenia. This pattern of motility disturbance (decreased elevation with the eye abducted) is typical of orbital restrictive disease, specifically due to loss of inferior rectus muscle elasticity. This is the most frequently affected extraocular
Chapter 2: Orbital disease or neurologic disease? |
33 |
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A B
Figure 2.10 Orbital CT in the above patient with vertical diplopia. (A) The axial contrast-enhanced image is nearly normal, showing just subtle thickening of the right medial rectus muscle. In retrospect, this abnormality of the medial rectus could also be seen on the MRI. (B) The coronal image is much more helpful, demonstrating enlargement of the right inferior rectus muscle.
muscle in patients with thyroid eye disease, and therefore this pattern of motility disturbance is characteristic.
Based on the examination features described above, a dedicated CT scan of the orbits was obtained. This study showed a slightly plumper right medial rectus muscle compared to the left medial rectus and definite enlargement of the right inferior rectus muscle consistent with thyroid eye disease (Figure 2.10).
Discussion: This case illustrates an important point regarding the radiographic diagnosis of orbital disease. While brain MRI may detect orbital pathology in some patients, in other cases important findings will be missed if dedicated orbit views are not obtained. This is particularly true for abnormalities of the vertical eye muscles, as illustrated by this case. The horizontal rectus muscles are well visualized with axial views, which are often included in standard head scans, but coronal sections are needed to adequately evaluate the vertical muscles.
MRI is often considered preferable to CT because of its higher spatial resolution and superior soft tissue detail. Indeed, MRI is more sensitive than CT for inflammatory orbital disorders such as scleritis, optic neuritis and optic perineuritis (see below, Painful optic neuropathy) and is also superior for the detection of disease within the optic canal. However, CT scanning is a good alternative for demonstrating most orbital pathology and may be preferable in certain settings. CT provides excellent visualization of disorders of the extraocular muscles, such as thyroid eye disease. CT is preferable for all cases of trauma, for evaluating changes in the orbital bones and for demonstrating calcification, as in optic nerve sheath meningiomas and optic disc drusen. Whether MRI or CT is used, adequate orbital imaging includes both axial and coronal views.
This case also illustrates the limitations of thyroid function tests for the determination of Graves’ orbitopathy. Abnormal test results (e.g. elevated T4 levels and suppressed TSH or, occasionally, low thyroid levels and elevated TSH) are helpful when
