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Neuro-ophthalmology

Blurring of Vision: A Practical Approach to Common Case Scenarios 13 February 2016

Sangtam Tiakumzuk MBBS, MMed (Ophth), FRCSEd (Ophth) Registrar, Ophthalmology & Visual Sciences

http://www.deviatingeye.com/graphics/textbook-illustrations/visual-pathway-from-the.html

History

• Laterality of the vision loss

• Time course of the vision loss

• Associated symptoms

Laterality of the vision loss

• Unilateral Versus Bilateral

• Ask specifically if they have checked each eye individually

• Binocular involvement may not be appreciated until the patient is examined

Laterality of the vision loss

Unilateral

• Lesion anterior to the chiasm

Bilateral

• Bilateral ocular, chiasmal, or retrochiasmal process

Time Course of Vision Loss

• Sudden onset to Rapid vision loss

• Days to weeks

• Over months

• Months or years

• (Caveat: Various etiologies exhibit considerable overlap

among the different time courses)

Time course Sudden to Rapid

Ischemic event or origin

Days to Weeks

Inflammation

(Caveat: Various etiologies exhibit considerable overlap among the different time courses)

Over months

• Typical of toxic lesions

Months to Years

• Most consistent with compressive causes

(Caveat: Various etiologies exhibit considerable overlap among the different time courses)

Associated Symptoms

• Globe tenderness or ipsilateral periorbital pain

• Increases with eye movement

• Optic neuritis

Associated Symptoms

• Diplopia, oscillopsia, hemiparesis, and hemisensory changes

• Demyelinating disease

Associated Symptoms

• Nonspecific pain, facial numbness, or diplopia

• Orbital or cavernous sinus lesions

Associated Symptoms

• Headache, jaw claudication, scalp tenderness,

• Systemic symptoms (weight loss, night sweats, malaise, myalgia)

• Giant Cell Arteritis

Examination

• Best-Corrected Visual Acuity (BCVA)

• Color Vision Testing

• Pupillary Testing

• Fundus Examination

• Visual Field Evaluation

BCVA

• BCVA obtained with refraction

• Improvement with pinhole: refractive component

• Worsening with pinhole: retinal or lenticular contribution

Color Vision Testing

• Complements VA

• Optic nerve disease: affect color vision>>VA

• Macular disease: VA=color vision decline correspondingly

Pupillary Testing

• Dim ambient lighting

• Fixate at distance

• Use a well-charged, bright, steady light source

Pupillary Testing

• Stimulate 1 eye for 2–3 seconds

• Quickly move across the bridge of the nose to stimulate the other eye for 2–3 seconds

• Do not rely on a single observation

Pupillary Testing

• If 1 pupil does not react (eg, iris trauma, synechiae, pharmacologic mydriasis or miosis)

Source: AAO-Neuro-Ophthalmology 2015-16

Fundus Examination

• 3CCC: Color, Contour, Cupping

• 3SSS: Shape, Size, Structures ±

Source: AAO-Neuro-Ophthalmology 2015-16

Fundus Examination

Structures

Source: AAO-Neuro-Ophthalmology 2015-16

Visual Field Evaluation • Examiner sits 1m from the patient @ same

level

• Patient covers 1 eye and fixates on the examiner’s nose

• Examiner asks the patient if specific portions of his face cannot be seen

• Ask to identify a target of 1, 2, or 5 fingers presented at the midpoint of each of the 4 Qs

• If cannot identify fingers, present progressively stronger stimuli such as HM, LP in each quadrant

https://www.youtube.com/watch?v=2-9FVywV2j4

Optic Neuritis

• Retrobulbar neuritis

• Papillitis

• Neuroretinitis

Source: AAO-Neuro-Ophthalmology 2015-16

http://www.slideshare.net/aravin8292/optic-neuritis-43175548

http://slideplayer.com/slide/4522455/

Clinical features of demyelinating optic neuritis

• Symptoms

Subacute monocular visual impairment

Usual age range 20–50 years (mean around 30)

Some patients experience tiny white or colored flashes or sparkles (phosphenes)

Clinical features of demyelinating optic neuritis

Discomfort/pain in or around the eye (>90%), typically exacerbated by ocular movement

May precede or accompany the visual loss and usually lasts a few days

Frontal headache and tenderness of the globe may also be present

Uhthoff phenomenon in MS (sudden worsening of vision or

other symptoms on exercise or increase in body temperature)

Wilhelm Uhthoff (31.7.1853 – 21.3.1927) German ophthalmologist (Wikipedia)

https://mmcneuro.wordpress.com/category/ms/

Clinical features of demyelinating optic neuritis

• Signs

VA is usually 6/18–6/60, but may rarely be worse

Other signs of optic nerve dysfunction - particularly impaired colour vision and a RAPD

Optic disc is normal in the majority (retrobulbar neuritis); the remainder show papillitis

Jacques Jean Lhermitte 20.1.1877 – 24.1.1959French neurologist, neuropsychiatrist (Wikipedia)

Lhermitte sign in MS

Visual field defects

Transient Visual Loss (TVL)

• Sudden loss of visual function (partial or complete) in 1 or both eyes <24 hours

• MC of monocular TVL is retinal ischemia due to carotid artery disease

• MC cause of binocular TVL is migraine

Detailed history

• Monocular • Lesion anterior to the chiasm

• binocular • Bilateral ocular, chiasmal, or

retrochiasmal process

Age

< 50 years,

• Migraine

• Vasospasm

• (Important exception in pregnant

women is eclampsia - harbinger of more serious and permanent visual loss, usually occurring within days of delivery)

Older patients

• CVA

• GCA

Duration of visual loss

Lasting only seconds

• UL or BL transient obscurations of vision

• Often precipitated by a change in posture

• OD drusen or papilledema

Lasting several minutes (<15)

• Symptomatic ipsilateral ICA stenosis

Pattern of visual loss and recovery

• Descending curtain in 1 eye - classic description of TMVL from retinal emboli

• Altitudinal visual loss strongly suggests carotid artery disease, sometimes vasospasm

• Visual loss precipitated by exercise also suggest vasospasm

Pattern of visual loss and recovery

• Posterior circulation ischemia typically causes: complete BL visual loss (ie, cortical blindness) homonymous hemianopia (often associated with brainstem and

cerebellar symptoms or deficits)

• BL geometric quality-hexagonal “chicken wire” pattern strongly suggests occipital lobe dysfunction

• Whiteout of vision or gradual “closing in” of peripheral vision may also signal occipital lobe ischemia

Associated symptoms and additional signs

• Positive visual phenomena and headache accompanying TVL suggest migraine

• Persistent headaches and intracranial noises are typical for increased intracranial pressure

• Elderly with headaches, wt. loss, fever, malaise, and scalp tenderness strongly suggest GCA

Associated symptoms and additional signs

• LOC, dizziness, diplopia, dysarthria, or focal weakness

• Suggest global perfusion problems, often involving the brainstem or cortex

• Skin or joint changes or Raynaud phenomenon may accompany collagen vascular disease

Transient Monocular Visual Loss (TMVL)

• Amaurosis fugax (“fleeting blindness”) is TMVL due to retinal emboli

• Abrupt, painless, and descends (or ascends) like a curtain over part or all of the visual field in 1 eye

• Usually resolves in 10–15 minutes but can take up to 1 hour in some cases

Transient Monocular Visual Loss (TMVL)

• At resolution, the curtain may either retract or dissolve like a clearing fog

• Amaurosis fugax is not associated with other neurologic deficits

• Ocular examination is often normal

Transient Monocular Visual Loss (TMVL)

• Emboli that cause TMVL usually travel to and lodge in blood vessels that supply the retina

• Ophthalmoscopically appear distinctive, their probable site of origin can often be inferred

• Such an inference may be crucial in directing appropriate patient evaluation

Transient Monocular Visual Loss (TMVL)

• 3 most common types of emboli:

Cholesterol (Hollenhorst plaque)

Platelet-fibrin

Calcium

Other less common: cardiac tumors (myxoma), fat (long-bone fractures,

pancreatitis), sepsis, talc, air, silicone, and depot drugs (corticosteroids)

Cholesterol (Hollenhorst plaque)

• Yellow-Orange/Copper

• Refractile

• Globular or rectangular

• Usually @ major bifurcation

• Source: CCA or ICA, rarely Aorta/Innominate artery

AAO

Kanski

Cholesterol (Hollenhorst plaque)

• Evaluation

General medical examination Noninvasive studies of

carotid patency Angiography, including

Aortic arch Cardiac assessment (not for

source of embolus but because these emboli increase the risk of cardiac disease and death from cardiac dysfunction)

AAO

Kanski

Platelet-fibrin

• Dull gray-white

• Long smooth shape

• Concave meniscus @ each ends

• Usually mobile

• Lodge along course of vessel

• Source: wall of atherosclerotic vv, heart (esp. valves)

AAO

Kanski

Platelet-fibrin

• Evaluation

General medical examination

Cardiac assessment, including Holter monitor and ECHO

Noninvasive studies of carotid patency

Hematologic studies

AAO

Kanski

Calcium

• Chalky white • Large • Round or ovoid • Lodge in 1st or 2nd

bifurcation • May overlie optic disc • Source: Heart/great vv,

RHD, calcific aortic stenosis, calcification of mitral valve annulus

AAO

Kanski

Calcium

• Evaluation

General medical examination

Cardiac assessment, including ECHO

Angiogram of aortic arch

AAO

Kanski

Medical treatment

• TMVL due to carotid artery stenosis (ie, retinal TIA) begins with aspirin

• Clopidogrel bisulfate is useful for patients who are intolerant of or allergic to aspirin

• Cilostazol, may be more protective than either aspirin alone or clopidogrel

Medical treatment

• Once antiplatelet therapy is maximized, consider adding a statin or increasing the dose of a statin if already taking

• There is some evidence that high doses of statins can reduce plaques and the frequency of stroke

• Finally, ACE inhibitors and ACE receptor blockers may be useful for their favorable effects on endothelial tissue

Transient Binocular Visual Loss

• Migraine

• Occipital mass lesions: tumor, arteriovenous malformation

• Occipital ischemia: embolic, vasculitic, hypoperfusion

• Occipital seizures

Migraine

• MC cause of binocular TVL is the homonymous hemianopic defect caused by migraine

• Migraine with aura (classic migraine-30% of migraine): typical visual aura have a hemianopic distribution

• Migraine aura without headache (acephalgic migraine-5% of migraine)

Source: AAO-Neuro-Ophthalmology 2015-16

“Scintillating scotoma”: blind region surrounded by a margin of sparkling lights “Fortification spectrum”: begins with a small scotoma, gradually expands into the peripheral vision

Fortification spectrum (Teichopsia-Greek word teichos-”town wall”) so-named because of the resemblance of the zig-zag margin to the ground plan of town fortifications in Europe

Source: AAO-Neuro-Ophthalmology 2010-11 http://clements.umich.edu/exhibits/online/geometry_of_war/geoamberina.php

• Aura lasts <60 minutes and is typically followed by a throbbing headache on the contralateral side of the head

• Most experience associated nausea, photophobia, and phonophobia

• When untreated, migraine attacks typically last from 4 hours to 72 hours

• Abnormal excitatory activity followed by a wave of depressed neuronal function

• Some experience “Alice in Wonderland effect” (micropsia/macropsia)

• Heat waves, cracked glass, kaleidoscopic vision, or fragmented vision

Walsh & Hoyt’s Essential Neuro-Ophthalmology 2016

Who need Evaluation?

• Typical history with normal neurologic and ophthalmic exam

• Neuroimaging studies unlikely to show an intracranial abnormality

• Occasionally, mass lesion/large vascular malformation (often residual-visual field defects)

Source: AAO-Neuro-Ophthalmology 2015-16

Who need Evaluation?

• Following findings may suggest additional evaluation of patients presumed to have migraine:

Headache or aura always occurring on the same side

Headache preceding the aura

Neurologic deficit, including visual field defect, persisting after aura resolves

Atypical (>1 aura occurring in a single day, lack of expansion of/change in aura, duration <5 min or >60 min)