Quid Refert, Dummodo non Desinas, Tardius Ire

Neuro-Optha
Opthalmology Dr. Mariano

BASIC NEURO-OPTHALMOLOGY All cranial nerves are at the brainstem or anteriorly at the cavernous
sinus
AFFERENT (light going in to EFFERENT (for ocular movement)
occipital lobe)
Retina CN 3, 4, 6
Optic n. Ocular muscles
Chiasm Brainstem
Tract Pursuit & Saccadic
Cortex pathways

Common Problems Seen

Loss of vision (transient, constant, mono/binocular)
Diplopia
Ptosis
Visual disturbance
pupillary light reflex→ it ends in the pretectal nucleus and it does not go
Pupil irregularities
all the way to the occipital lobe, so you can be occipitally blind but you
Motility disorders
still have pupillary reaction.
Eyelid or facial spasms
Direct - constrict SAME side
Consensual - constrict DIFFERENT side
Considerations
If you shine a light and it doesn't constrict: problem is AFFERENT first,
Where/What is the lesion (read visual fields, interpret, and be able
maybe there's a problem in transmission, the way for you to know is to
to localize the lesion)
check the Consensual, if it doesn’t constrict in consensual then then
o bitemporal hemianopsia → lesion at optic chiasm, nearest to
problem really is AFFERENT
the pituitary so consider a sellar mass
Torch test (swinging penlight test): do it one eye at a time, look for your
When did it start
direct and consensual, what we look at is how brisk, how fast it constricts
How did it present
and re-dilate
o Patient may come to you that her vision is frosted or under
water

Urgent/Emergent

PUPILLARY LIGHT REFLEX PATHWAY

Light shined on one eye

↓
Retina
↓ Sudden & Acute → Vascular
Optic nerve Gradual, progressive → tumor (something that’s compressing)
↓ Long duration + associated pain → Inflammatory
Optic chiasm
↓ RELATIVE AFFERENT PUPILLARY DEFECT (RAPD)
Optic tract
↓ to occipital lobe Normal response
Lateral geniculate nucleus There’s consensual light reflex but there’s NO DIRECT light reflex on the
↓ affected eye
Pretectal nucleus Can be caused by
↓ Optic neuritis or
Edinger-Westphal nucleus on both sides Tumor anterior to the chiasm
↓ To test, ask the Px to look at a distance and do it fast (Marcus Gunn
Constriction of both pupils test/Swing light test)
Normal reaction → when light is shined on one eye → both eye
If a pupil doesn't constrict: Problem in EFFERENT (CN 3) constricts
While shining a light, don’t put it straight away, start underneath
because when you put it straight → the eye converges

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 When the eyes look at a near object, three responses occur –
accommodation, convergence, and constriction ofthe pupil –
bringing a sharp image into focus on corresponding retinal points.
(Video showing R. Eye with direct response and L. Without direct
response) → RAPD
If px complains of blurring of vision, and patient reads 20/400 and other
eye is 20/20 and you check the pupil but didnt see an RAPD -- px thinks
its an optic neuropathy (optic nerve problem) → Isometropic amblyopia
(lazy eye) problem is in the connection - the brain is trying not to use one
eye
If you dont see RAPD → there's NO problem in the visual pathway
If with RAPD - problem in the VISUAL Pathway
If you see a problem with the swinging light test, and it patient saw a
very red color but other eye saw a faded red and you see an RAPD →it is
really a problem with the optic nerve
In examining pupils, you can have anisocoria- unequal sized pupils; few
things to consider
1. Is this an emergency
2. If an emergency, you can see a neuro-ophthalmologist from 1-2wks
pupillary emergencies: if one pupil is bigger than the other and
accompanied by a motility problem (if pupil is dilated and is not
moving) also if there’s a pupil abnormality together with ptosis→
immediate imaging & referral to neuro-opthalmologist
EFFERENT SYSTEM DISEASES
Diplopia
CN palsy
Multiple CN palsy
Abnormal eye movement
Myasthenia gravis
DIPLOPIA
Key question:
Is it only in one eye?
Does it go away when you close either eye?
If a Px come to you with a complaint of double vision and
disappears after covering one eye → investigate.
If double vision still persist → can be an Error of Refraction
Mononuclear diplopia is always refractive in origin
Cataract & astigmatism
Examine lids and pupils in addition to eye movement
Examine all CN
Check V1 (corneal sensation), consider cavernous sinus problem
CN 3, 4, 6 (for ocular motility)
Can be a vasculopathic cranian nerve neuropathy
Demyelinating disease
Compressive
Trauma
↑ ICP
CN 4 & 6 = will always complain of DIPLOPIA
Complete CN 3 palsy = will complain of diplopia because he has ptosis
(elevate that lid and look if dilated)
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OCCULOMOTOR NERVE (CN 3) PALSY
eye is looking down and out + ptosis “No one loves you when you’re
down and out”
check for pupillary light reflex → will tell you the urgency of the case
if pupils are dilated → think of an aneurysm
patient may have aneurysm ..and the first sign of aneurysm is a CN3
problem + pupillary involvement and the most common site of aneurysm
is at the junction of the PCOMM. (posterior communicating artery
aneurysm)
Ruptured aneurysm 70% mortality
ABDUCENS NERVE (CN 6) PALSY
Anatomical facts:
Origin of CN 6 w/c innervates lateral rectus but also gives fibers
w/c eventually end at the contralateral medial rectus
Most common nerve affected or sensitive by raised pressure (swollen
optic nerve)
Diplopia at distance → face turn toward same side will relieve double
vision
TROCHLEAR NERVE (CN 4) PALSY
Only CN which exits dorsally, “stupid nerve” – it can go straight but
instead goes down and up again then to other side
Thinnest (slender)
Longest intracranial course 75mm
Only CN which crosses opposite side
Oblique diplopia
Patient will complain that one image is HIGHER than the other
To relieve double vision → Patient will tilt their head (if acute, px may
not present with head tilt)
Parks-Bielschowski 3-step test
Commonly associated with brain tumors that’s NOT associated with
raised pressure
MULTIPLE CRANIAL NERVE NEUROPATHIES (3, 4, 6)
Important here is AGE of the patient
<50yo – NOT hypertensive, NOT diabetic → Immediate imaging
>50yo – known hypertensive & diabetic, smokes, drinks, if only 1
CN affected you can only observe
Ischemic CN neuropathies are almost always isolated
If multiple simultaneous CN, suspect lesion in posterior orbit/cavernous
sinus region
Usually d/t mass lesion
Cranial nerves are all bunched up in the brainstem and the cavernous
sinus, so any lesion affecting these areas will also affect cranial nerves
Differentiate if lesion is from the brainstem or cavernous sinus
Cerebellum is beside brainstem → px w/ brainstem problem will
also present with cerebella problem
If NO cerebellar problems → think of a cavernous sinus problem
(think of a cavernous sinus THROMBOSIS)
MYASTHENIA GRAVIS
great mimicker → can mimic any cranial nerve problem
hallmark: variability and fatigability
mechanism: autoantibodies directed against Ach
receptors are produced and destroy and block a lot of receptors
Ocular myasthenia gravis
Myasthenic signs restricted to ocular muscles
Fatiguable diplopia & ptosis
First sign of MG >70% are with eye sign
ICE TEST or REST TEST in the clinic demonstrate improvement (Ice
test in the → (+) if Px can open their eyes after application of ice)
Ach receptor antibodies (+) in 50% only
Single fiber EMG
TENSILON TEST (Give atropine bec. one S/E is cardiac arrest) – done
rarely, costs 40k, double vision disappears, effect is around 30s
Miki
 Natural pattern
¼ present w/ diplopia or ptosis
80% may start to develop systemic weakness within 2yrs
Optical Treatment
Prisms
Occlusion
Medical
Pyridostigmine (Mestinon) – anticholinesterase
Prednisone – suppresses autoimmune process
Steroid-sparing drugs
Surgical
Thymectomy
Ptosis & Strab – must be stable, longer, better & must be informed
properly

CLUES in DIAGNOSING DIPLOPIA

Variability & Fatigability → MG Macular sparing → OCCIPITAL

Distance → CN 6, IIH, ↑ICP Meyers Loop - while it goes to the optic radiation. Some fibers go up
Near → CN3, INO to parietal lobe and some fibers go down to temporal lobe then
Down and out → CN3
meet at the occipital cortex
Monocular → Error of Refraction
Occipital lobe function is purely visual – Px would come to you
Tilting → CN 4
with Hx of bumping onto objects
Multiple CN → MG, CST
Temporal & Parietal are integrators- they let their vision come
from the optic nerve to the cortex, it gives visual cues: the WHAT &
VISUAL FIELD DEFECTS WHERE
There’s one px, wherein she couldn’t read the Snellen but when
Only the NASAL optic nerve traverses to the other side whereas the shown a single letter, vision was 20/20 → Simultagnosia (MRI
TEMPORAL doesn't, that's why posteriorly (beyond the chiasm), a lesion showed affecting the parietal, px presents w/ diffulties in
will manifest on both parts of the visual field will be affected comprehensionalso)
Any lesion posterior to the chiasm → always HOMONYMOUS
Anterior to the chiasm → only ONE eye is affected

PIE in the SKY defect (SUPERIOR quadrantinopsia) → lesion in TEMPORAL

Can either be occipital/temporal but NEVER Parietal

PIE in the FLOOR defect (INFERIOR quadrantinopsia) → lesion in

PARIETAL
Always parietal or occipatl but NEVER Temporal
Confrontation Field test
Make sure you cover one eye and eye-level with the examiner
Draw confrontation field (baliktad) on how patient sees it NOT how
you see it
If field defect is on the L. side – look for it at the R. side of brain

More congruous homonymous hemianopsia → lesion in OCCIPITAL

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 AFFERENT SYSTEM DISEASES TYPICAL OPTIC NEURITIS

Optic neuritis Inflammation of the optic nerve, often caused by demyelinating event (in
Ischemic optic neuropathy (arteritic vs. non arteritic) the US), in the Philippines it is purely inflammation or caused by bleeding
Toxic optic neuropathy (e.g. ethambutol toxicity) sudden vision loss with optic disc swelling
other optic neuropathies (compressive, papilledema, inflammatory, CARDINAL sign is decreased vision (especially color), seeing faded color
hereditary) suggests optic nerve problem
chiasmopathies/chiasmal disorders pain with eye movements
stroke that causes defects in the visual field females > males
RAPD present
OPTIC DISC Optic disc normal (2/3)
brown hair, blue eyes → observe for few weeks
Look for disc borders, hemorrhage, color (if pale), cup-disc ration typically found in Caucasian people
(reported as 0.3, 0.5 etc.) age b/w 15-45y/o
Pale optic disc → atophy (20% have tumor) it is associated with MS (Multiple Sclerosis) – sakit ng mga people away
In papilledema, there should be increase ICP, if there is no from the equator
mentioned increase ICP, label it as optic head swelling or disc In the Phil’s, most common cause is inflammatory or infectious
edema (normal ICP = 15-25 mmHg) Unilateral
pallor – vision affected > 6 weeks Pain
o On eye movements
o First symptom to appear & disappear (when pain disappears, vision
would drop)

R’s in Optic Neuritis

Radiology
o MRI is only done to see White matter lesions to confirm MS
(75% over 15yrs)
o More lesions ↑ risk of developing MS
Recover
o IV steroids only hasten recovery but not final VA outcome
Risk factors
(N. optic disc) o 20-40yo, F, white, family Hx of MS
Recurrence
In disc is swollen & with hemorrhage, bilateral already, can be o Higher with those given with oral steroids (standard doses)
hypertensive retinopathy, consider space occupying lesion →
BRAIN TUMOR Most common symptom: PAIN on EYE MOVEMENT
Most common Visual Field Defect: DIFFUSE
Typical pattern: 1-2wks – stable visual acuity, after 2wks – improved
vision
Treatment of choice for typical optic neuritis – A. IV methylprednisolone
250mg 4x/d for 3days with Oral steroids 60 mg for 11 days (eto sagot
palagi due to optic neuritis trial but not because final visual acuity of
receiving steroids vs not is the same) B. Prednisone 60mg 1x/d for 14
days C. IV dexamethasone 20mg 1x/d
Treatment of Choice → OBSERVE (improve spontaneously)
If not improving in 2wks time – then do imaging and IV steroids

Differentiate optic neuritis from papilledema; clinically, make the Px

READ
o Optic nerve swelling but Px can read 20/20 → Papilledema on
ACUTE onset, if >6wks chronic already; difficulty reading
o Subtle findings, good visual acuity with light perception → Optic
neuritis

Atypical optic neuritis: (typical occurs in ages 15-45) you’re supposed to

do imaging

PAPILLEDEMA

Disc edema d/t ↑ ICP (mass, pseudotumor cerebri), optic nerve edema
Headache, transient visual obscurations, diplopia, tinnitus (ringing
sound), complains of changes in vision
Normal visual acuity & visual fields early
Opthalmoscopy & Imaging

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 Px came with a (-) 300 grade meaning myopic, recently came with a shift PUPILLARY ABNORMALITIES
in refraction with (-) 100, MRI revealed a tumor, the cause of the shift is
↑ICP, optic nerve is bathed in CSF so CSF will go to the optic nerve Anisocoria – unequal pupil size
sheath, push the eyeball. myopic eye is very long, so eyeball was pushed Difference in size is:
to be smaller o more prominent in the DARK = HORNER’s (frequent board
Image below shows ill-defined borders exam question)
Papilledema is NOT a diagnosis, the prerequisite is raised ICP o more prominent in the LIGHT = ADIE’S (anisocoria with deep
tendon abnormalities)
o DARK = LIGHT → Physiologic
Anisocoria with any motility problem = consider Aneurysm
Can be accidental discovery
Physiologic in 40% of patients
It can be isolated or associated with lid or ocular motility abnormalities
Can be iatrogenic or self-induced (Pharmacologic)

HORNER’s SYNDROME

CENTRAL RETINAL ARTERY OCCLUSION (not discussed)

Painless loss of vision

May be preceded by Amaurosis Fugax
Source of emboli usually carotid or cardiac
Less common causes: vasculitis (GCA, Anti-phospholipid syndrome)
Order Carotid Doppler Study A defect in oculosympathetic flow to the eye (pupil does not dilate in
DARK)
Sympathetic innervation is affected, Muller’s muscle is sympathetically
ARTERITIC ISCHEMIC OPTIC NEUROPATHY
innervated only contributes 1mm lid elevation (slightly ptotic only)
TRIAD
patients > 60 y.o. (older age group) headache, malaise, myalgia (calf 1. Ptosis
pain), weight loss, fever, jaw claudications and transient loss of vision 2. Anhydrosis
Labs: ESR, CRP high 3. Miosis (worst in dark)
Temporal artery biopsy Internal carotid artery dissection, neck trauma or surgery, Brainstem
> 3 cm is needed due to skip lesions strokes (Wallerburg Syndrome), apical lung tumors
giant cell arteritis/Temporal cell arteritis Urgent MRI/MRA of the head and neck for ACUTE Horner’s Syndrome
Immediately give high steroids or else the px can go blind or px may die “One horny PAM from the coast”
because there is active inflammation of all blood vessels One = T1 lesion (from the posterior hypothalamus it goes all the
do temporal artery biopsy, get about 3-5cm specimen b/c there are skip way to T1 then up the carotids)
lesions Horny = Horner’s
patient always present with severe pain, headache, malaise, weight loss PAM = triad
(b/c patient has jaw claudication every time the patient tries to Coast = Pancoast Syndrome (tumor of the lungs)
eat→patient has to change his/her diet) Horner’s syndrome, SUDDEN onset + pain → Dissecting aneurysm
normally present with disc pallor (optic nerve should be orange, if pale or OCULOSYMPATHETIC PATHWAY
chalky white →optic nerve ATROPHY)

COMPRESSIVE LESIONS

slowly progressive loss of vision (in optic neuritis, it is sudden)

can be unilateral/bilateral DIAGNOSIS
pituitary tumors, craniopharyngiomas & meningiomas of the skull base Cocaine test → tell if Horner’s or NOT
require MRI for diagnosis Testing topical cocaine in the conjunctival sac differentiates
Horner's syndrome, in which the pupil does not dilate, from
physiologic anisocoria
Testing with hydroxyamphetamine drops → localize the lesion
3rd test: Apraclonidine test – get sudden result

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 Px with carotid dissection – manifested with Horner’s because
oculosympathetic pathway goes down and up with the carotids

ADIE’S/TONIC PUPIL

END
Black – from power point
Blue – trans from lecturer
Red – from book
Green – old trans

SEGMENTAL CONSTRICTION - Pathognomonic

Damage to the CILIARY GANGLION and/or short ciliary nerves
Sluggish or no reaction to light
Tested in a WELL-LIGHTED room
HALLMARK:
Delayed dilation after constriction (slow redilation)
Segmental constriction → BAG of WORMS
Constricts to 0.125% Pilocarpine
DTR’s may be affected → ADIE-HOLMES
Sweating only on one side of body → ADIE-ROSS-HOLMES Syndrome
LIGHT NEAR DISSOCIATION
Initially present with dilated pupil but will later become constricted

CLUES in DIAGNOSING ANISOCORIA

Dark > Bright → HORNER’S

Bright > Dark → ADIE’S
Dark = Bright = accommodation → PHYSIOLOGIC
Dark = Bright < accommodation → LIGHT NEAR DISSOCIATION
Anisocoria +/- CN3 → ANEURYSM

NOT DISCUSSED

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