DIPLOPIA

TO SWIM OR TO SINK

By
AHMED MAHMOUD AMIN
MD,FRCS(Glasgow)
Orbital fellow , AMC , Amsterdam , Netherlands
Lecturer of Ophthalmology, Al Azhar University, Cairo
AKNOWLEDGEMENT
 OUTLINE

INTRODUCTION
HISTORY
EXAMINATION:
- Individual tests
- Sequence of examination
INVESTIGATIONS
 POSSIBLE SCENARIOS
THE 1-2-3 OF NEURO-OPHTHALMIC
EXAMINATION
1- The afferent visual pathways:
 Visual acuity
 Visual fields
 Color vision
 Ocular fundus examination.
2- The efferent pathways of ocular motility:
 Cover–uncover testing
 Saccade
 Smooth pursuit
 Vergence
 Vestibulo-ocular and optokinetic testing
 Red glass or even better the Maddox rod to characterize diplopia.
3- The afferent and efferent limbs of the pupil
Sensory
Motor
Adaptive

A PAIR OF LIVING MOBILE VIDEO CAMERAS

 ‫‪STRABISMUS‬‬

‫البرئ و المىحرف و مه عىدي القابلية‬

 STRABISMUS

PSEUDO
TRUE
= APPARENT

LATENT MANIFEST

CONCOMITANT INCOMITANT

RESTRICTIVE PARALYTIC
Static (Alignment)

Tendency to deviate

Kinetic (Motility)
 AN
APPROACH
TO
EXAMINING
OCULAR
MOTILITY
■ Measure VA and record refraction.
■ Observe any abnormal head posture (AHP).
■ Using a pen torch at 33 cm, observe the corneal reflections(CRs).
Look for symmetry or asymmetry.
■ Perform cover test (CT) at near (33 cm) to a light and then accommodative target (e.g.
reduced Snellen letter at VA of worst eye, or pictures/toys for children).
CT involves two manoeuvres.
1. Cover-uncover test: to detect manifest strabismus. Cover one eye and look at the non covered
eye. Movement inward to take up fixation indicates an exotropia. Movement outward indicates
an esotropia. If the eye is slow to fix the target, this may indicate poor VA or amblyopia. Note
any manifest or manifest latent nystagmus (MLN) – horizontal jerky, often very fine nystagmus
with fast phase towards the uncovered eye. Note any movement of the covered eye, particularly
dissociated vertical deviation (DVD) associated with infantile esotropia.
2. Alternating cover test: to detect latent strabismus.
Alternately occlude left and right eye for 1–2 seconds each. Do not allow both eyes to view in
between covering as fusion may then take place. A latent deviation sometimes becomes
temporarily manifest after fusion is disrupted by testing. Note how fast the eyes resume
binocular single vision (BSV) once testing is complete (rapid or slow recovery) indicating how
well the deviation is compensated.
■ Move the target occasionally to check fixation.
■ Perform CT at distance to a suitably sized chart letter or picture/toy.
■ Repeat CT with/without glasses, and then with/without AHP.
Eye movements (cranial nerves III, IV, VI)
■ If suggested by the history, check for monocular diplopia by occluding each eye separately.
Note VA. If not already known, clarify if diplopia is horizontal, vertical, or tilted.
■ Observe any head tilt, proptosis , ptosis, or lid retraction.
■ Ask the patient to follow a light held at 50 cm and report if they see diplopia.
Go from primary position and back again in the other 8 cardinal positions of gaze. Ensure the
CRs are always visible; the patient or examiner may need to lift the lids, particularly in down
gaze. Check for lid-lag on downward smooth pursuit and any narrowing (Duane’s syndrome) or
widening (aberrant 3rd nerve regeneration or Brown’s syndrome) of the palpebral aperture on
adduction; observe any pupillary changes, e.g. constriction of the pupil in adduction or other
gaze position may occur with aberrant 3rd nerve regeneration.
■ If diplopia occurs in any position, check which image disappears when an eye is covered; the
more peripheral image comes from the eye with the paretic muscle(s).
■ If CT shows a hypertropia/hyperphoria in the primary position (step 1) then do a CT in right
and left gaze to see where the height is greatest (step 2). If greater in right gaze then repeat
the CT up and down to the right. If the deviation is greater down to the right, a left 4th nerve
palsy is suspected. Finally, compare CT (eyes in primary position, fixing at 3 meters) with the
head tilted right and left, to see if the height differs (step 3). If the deviation is greater on head
tilt left, a left 4th nerve palsy is likely. However, the head tilt test is not always reliable and other
causes of hypertropia should not be ignored, e.g. thyroid eye disease. Park’s three-step test
refers to steps 1, 2, and 3 combined. Step 3 is also called the Bielschowsky headtilt test.
However, the diagnostic importance of testing in all 9 positions cannot be overemphasized.
Vertical deviations can be associated with bilateral (often asymmetrical) muscle under- or
overactions.
■ Examine saccades in suspected supranuclear lesions to help differentiate newly acquired
palsies from mechanical strabismus (normal in the latter). Position a target to the right and left
of the patient’s eyes, within the visual field; instruct the patient to look from one target to the
other as quickly as possible, without moving the head. Repeat the test in the vertical plane.
Compare the vertical and horizontal velocity of the excursion, as well as the velocity in each eye;
e.g. a reduced excursion of the adducting eye on horizontal saccades may indicate an inter
nuclear ophthalmoplegia (same side).
Cerebellar disease and MS may produce hypermetric saccades (eyes overshoot the target).
Myasthenia gravis and Parkinson’s disease may produce hypometric saccades (eyes undershoot
the target).
■ Test convergence to a detailed target, and observe normal pupillary constriction.
■ If required perform Doll’s head maneuver to differentiate supranuclear from nonsupranuclear
lesions (e.g. Steele Richardson Olszewski Syndrome). Ask the patient to fixate a target in the
distance. Inform the patient that you are going to gently move the head right, left, up and down.
Observe the extent of ocular rotations. Doll’s head movements may be absent in supranuclear
lesions.
■ Test optokinetic nystagmus with an OKN drum rotated slowly in front of the patient, both
horizontally and vertically, and in both directions. Horizontal asymmetry may indicate a parietal
lesion. Convergence retraction nystagmus with a downward moving drum (producing upward
refixation saccades) suggests Parinaud’s syndrome.
 AS ANY MUSCLE
Posture
Tone (passive)
Power (active)
Coordination

Remember that you are working in the orbit

FORCED DUCTION TEST
PARALYTIC VERSUS
RESTRICTIVE

- Forced duction test

- Active force generation test
- Differential IOP test
 INVESTIGATIONS
(ANATOMICAL & FUNCTIONAL)

OCULAR SYSTEMIC
-Diplopia chart -Laboratorial
-Hess screen -Radiological
-EMG
-Instrumental
DIPLOPIA CHART
HESS SCREEN
DIFFERENTIAL DIAGNOSIS OF ENLARGED EOMs
COMMON CAUSES OF EOM ENLARGEMENT
 EOMs IN TED
Smooth
Spindle shaped
Sparing the tendons
Solitary (? IR)
Special Sequence of involvement
Symmetrical if bilateral
 I’M Slow or (I’M So Late)
(Inferior, Medial, Superior, & Lateral rectus)

Lateral rectus is the Last to be involved in TRO

 WHERE IS THE LESION?
The motility examination evaluates the integrity of
the following:
Extraocular muscles
Neuromuscular junction
Ocular motor nerves (third, fourth, and sixth)
Ocular motor nuclei
Internuclear pathways
Supranuclear pathways

‫الالعبين والجهاز الفنى والملعب‬

 FLOW DIAGRAM
FROM SYMPTOM TO SITE TO CAUSE
 WHERE IS THE LESION?

Single EOM
Group of EOMs → Single cranial nerve (3rd)
Multiple cranial nerves → Syndrome
( 3 motor cranial nerves & 3 syndromes)
If not → think of mimickers

(LR6 SO4)3
The ocular motility examination consists of the following:
● Observation in primary gaze
● Ductions (monocular eye movements)
● Vergence (binocular dysconjugate movements)
● Versions (binocular conjugate eye movements)
○ Saccades
○ Pursuit
○ Oculocephalic responses and vestibulo-ocular reflex
(VOR)
○ Optokinetic nystagmus
● Detection and measurement (with prisms) of ocular
misalignment (strabismus)
● Detection of nystagmus
 Saccade is a fast eye movement used to acquire a
target by placing the image of the target on the fovea.
 Smooth pursuit is a slow eye movement used to track a
target as it moves by keeping the target on the fovea.
 The vestibular ocular movement is used to keep the
eyes on a target during brief head movements.
 The optokinetic eye movement is a combination of
saccadic and slow eye movements that keeps a full-field
image stable on the retina during sustained head
rotation.
Each of these movements is a conjugate eye
movement, that is, movements of both eyes together
driven by a common neural source.
Vergence movement is a non-conjugate eye movement
allowing the eyes to track targets as they come closer or
farther away.

NPC
A: METHOD FOR EXAMINING AND RECORDING OCULAR MOTILITY.
B: RECORD OF SIXTH NERVE PALSY OF RIGHT EYE.
C: RECORD OF RIGHT ORBITAL BLOW-OUT FRACTURE AND LIMITED
UPGAZE.
The direction of the caloric response is named after the FA ST phase

(Remember : COWS for Cold—Opposite, Warm—Same)

PARKS–BIELSCHOWSKY 3-STEP TEST REVEALING A RIGHT SUPERIOR
OBLIQUE (RSO) PALSY.
CIRCLE THE PAIR OF MUSCLES AT EACH STEP AND THE PARETIC
MUSCLE WILL HAVE THREE CIRCLES AROUND IT
RED GLASS TEST
MADDOX ROD
eXo→crossed
INO
INO & WEBINO
HORIZONTAL GAZE PALSY
ONE & HALF
 EIGHT & HALF

A lesion producing the one-and-a-half

syndrome but also involving the intra-axial
portion of the facial nerve is termed the eight
and-a-half syndrome (7 + 1.5 = 8.5)
FIFTEEN & HALF
SKEW DEVIATION
What is the lesion?
T - mnemonic

Trauma
Thyroid
Tensilon ( Myasthenia)
Thiamine deficiency
Toxins
Turkey
 PROGRESSIVE
OPHTHALMOPLEGIA
Rapidly progressive ophthalmoplegia should
suggest myasthenia, the Miller Fisher variant
of Guillain-Barre´ disease, or botulism; prognosis
in these disorders depends on respiratory
involvement, and early symptoms of
dyspnea should be carefully sought.
 MYASTHENIA GRAVIS
(Look for an eyelid that won’t stay open or closed)

Diurnal variation
Descending march course
- Drooping of upper lid
- Diplopia
- Dysphagia
- Dysarthria
- Dyspnea
Decremental response on EMG
Dysthyroid ophthalmopathy ( 5–10%).
 CASCADE OF EVENTS IN
MYASTHENIA GRAVIS
1- Thymus
 Thymus imaging
 Thymectomy if indicated
2- Antibodies
 Acetyl choline receptor antibodies assay
 Plasmapheresis to wash these Antibodies
3- Antigen / Antibody reaction
 Steroids
 Cytotoxic drugs
4- Acetyl choline as if depleted
 Tensilone test (diagnostic)
 Anticholine estrases as Mestinon (therapeutic)
5- Muscle dysfunction
 EMG & preferably single muscle fiber EMG
 AAO
ATAXIA
AREFLEXIA
OPHTHALMOPLEGIA
 Third nerve palsies may reveal a neurosurgical emergency such as
intracranial aneurysm or pituitary apoplexy, especially when the
pupil is involved.
 Be particularly aware of the possibility of aneurysm when the third
nerve palsy is partial, even when the pupil is spared .
 The ‘‘rule of the pupil’’ applies only if the patient has a complete
third nerve palsy (i.e., all muscles innervated by the third nerve are
involved, with minimal remaining function).
 A patient with pupil sparing complete third nerve palsy must be
seen in follow-up to verify that the pupil remains normal, because
mydriasis may be delayed for a few days in patients with
compressive third nerve palsy.
3rd NERVE PALSY
FOUR CLINICAL FEATURES FOUR COMMON LESIONS
WHERE IS THE LESION?
 WHAT IS THE LESION?

PUPIL INVOLVEMENT?

TRAUMA & TUMOURS

MOST COMMON CAUSES OF ACQUIRED FOURTH-NERVE LESIONS BY
ANATOMICAL LOCATION
Sixth nerve palsies may be associated with
raised intracranial pressure.
Finding bilateral disc edema on funduscopic
examination suggests this diagnosis.
Supra nuclear motility disorders are often
emergent because they are associated with
lesions affecting the brain stem.
 ‫الزمان و المكان‬

‫التاريخ و الجغرافيا‬
PAINFUL DIPLOPIA
 RED FLAGS
Except for infectious orbital cellulitis and some
forms of orbital pseudotumor, diseases
involving the extraocular muscles rarely
represent an emergency.
Ocular myasthenia gravis is not an emergency
unless the patient has systemic symptoms
such as respiratory distress or dysphagia.
Isolated fourth nerve palsy is rarely an
emergency.
 STRUCTURES PASSING
THROUGH THE SUPERIOR
ORBITAL FISSURE

LIVE FREE TO SEE NO INSULT AT ALL

SUPERIOR ORBITAL FISSURE SYNDROME
TOLOSA-HUNT SYNDROME
CAVERNOUS SIUS (SUEZ CANAL)
6 TRIBUTARIES (WATER)
1-BRAIN ( via middle cerebral veins)
2- FACE ( dangerous triangle via…)
3- EYE (via ophthalmic veins)
4- OTHER CAVERNOUS SINUS (via …)
5- EAR ( via mastoid emmissary vein)
6- PHARYNX ( via…)
 CAVERNOUS SIUS (SUEZ CANAL)

7 PASSENGERS (SHIPS)
1- 3rd
2- 4th
3- 6th
4- 1st division of 5th
5- 2nd division of 5th
6- Sympathetic fibers
7- ICA
 CCF

Direct or indirect
 High flow or low flow
Spontaneous or traumatic
 CCF
Enlargement of :
Superior ophthalmic vein
EOMs
Cavernous sinus
BARROW CLASSIFICATION
COMPARISON OF DIRECT & INDIRECT CCF
CCF
BLOOD IS SHIFTED AWAY
FROM THE ARTERY RESULTING
IN ISCHAEMIA

THE VEIN RECEIVES ARTERIAL BLOOD UNDER PRESSURE

RESULTING IN :
1- CONGESTION
2- ARTERIALIZATION OF THE VENOUS WALLS
3 - PULSATIONS
 CCF

Arterial blood Aqueous

Venous
is stolen with drainage is
drainage is
resultant impeded due
impeded
ischaemia to ↑↑EVP
TREATMENT OF PARALYTIC STRABISMUS

Identify & treat the cause

‫سادة أم باالضافات ضغط و سكر و أشعات‬
Wait for 6months
‫ كيف نتغلب على اإلزدواجية؟‬: ‫وفى هذه اآلونة‬
Patching or Prism
After 6months:
If improved → ok
If Paresis → Recess Ressect
If Paralysis → Tendon transplantation
OCULAR OSCILLATION
 DIRECTION : horizontal/vertical/rotational
 WAVEFORM : pendular/jerk (direction of fast phase)
 AMPLITUDE : large/small (effect of gaze position on amplitude)
 REST : primary position (at rest/gaze evoked)
 FREQUENCY : fast/slow
THE EXAMINER CHECKS FOR NYSTAGMUS BY VIEWING THE EYE
WITH A DIRECT OPHTHALMOSCOPE AND COVERING THE OTHER EYE
WITH HIS HAND TO ABOLISH FIXATION
 JERK NYSTAGMUS: THE EYE REPETITIVELY
SLOWLY DRIFTS IN ONE DIRECTION (SLOW PHASE)
AND THEN RAPIDLY RETURNS TO ITS ORIGINAL
POSITION (FAST PHASE).

 PENDULAR NYSTAGMUS: DRIFT OCCURS IN TWO

PHASES OF EQUAL SPEED, GIVING A SMOOTH
BACK AND FORTH SLOW MOVEMENT OF THE EYE.
 NULL POINT: A POSITION OF GAZE WHERE THE
NYSTAGMUS IS MINIMIZED.
 COMPLETE THE PUZZLE

Cause & predisposing factors

Condition (true versus pseudo)
Complications
Associations
Treatment (effects & side effects)

NULL POINT
OCULAR
NYSTAGMUS VERSUS NYSTAGMUS BLOCKAGE PRISM
VESTIBULAR
NYSTAGMOID SYNDROME SURGERY
CNS
TORTICOLLIS
EARLY ONSET NYSTAGMUS
 LATENT NYSTAGMUS
(A) WITH BOTH EYES OPEN ,NO ABNORMAL EYE MOVEMENTS MAY BE
EVIDENT.
(B) WHEN ONE EYE IS COVERED, BOTH EYES DEVELOP A JERK
NYSTAGMUS WITH A FAST PHASE TOWARDS THE UNCOVERED EYE.

Fast phase towards the Fixing eye

- Reassurance
- Rule out gliomas of the anterior visual pathway
LATE ONSET NYSTAGMUS
THE PATTERN ALTERNATES IN A PENDULAR OR JERK FASHION, SIMULATING THE MOTION OF A SEE-SAW
LOCALIZING VALUE OF NYSTAGMUS PATTERNS
AND NYSTAGMOID EYE MOVEMENTS
CONDITION FEATURES LESION LOCATION

OCULAR FLUTTER Rapid , conjugate , horizontal Probably abnormalities of

oscillations burst neurons

OPSOCLONUS Combined horizontal , vertical Probably abnormalities of

and/or torsional oscillations burst neurons

OCULAR BOBBING Rapid downward deviation Pontine dysfunction

with a slow drift up to primary
position

INVERSE BOBBING (OCULAR Slow downward movement , Non localizing

DIPPING) fast upward movement

OCULOPALATAL Rhythmic oscillations Interruption of connections in

MYORHYTHMIA associated with simultaneous Mollarete triangle :Inferior
(MYOCLONUS) contraction of non ocular olive , Red nucleus and
muscles(Palate , Tongue) Dentate nucleus
 ‫‪PUPILS‬‬

‫البؤبؤ – إوسان العيه‬

 PUPIL EXAMINATION
(ANATOMICAL & FUNCTIONAL)
 Site (? Corectopia)
 Size
 Shape
 Symmetry (? Anisocoria)
 Reaction to light
- Direct
- Consnsual
- Swinging flash light test (RAPD)
 Reaction to near (Light/Near dissociation)
 Reaction to accomodation
 Reaction to drugs
Where is the lesion?(Anatomical site of the lesion)

What is the lesion ?(Aetiology)

Associations
 PUPIL EXAMINATION
QUALITATIVE & QUANTITATIVE

Pupil size in mm
Grading of RAPD with neutral density filters

Neutral density filter bar

AFFERENT PUPILLARY
DEFECTS

RAPD
ARP
 ‫وطفئ الىور والمية تكدب الغطاس‬

Scotopic conditions aggravate the difference ..Sympathetic lesion..? Smaller pupil is abnormal
EFFERENT PUPILLARY
DEFECTS
 3RD NERVE PALSY
Isolated or as a part of …syndrome
Pupil involving or sparing or delayed
Partial or complete
Signs of aberrant regeneration
DORSAL MIDBRAIN SYNDROME

 Supranuclear Upgaze palsy

Convergence-retraction nystagmus on attempted
upgaze
 Bilateral Lid retraction(Collier’s sign)

Abnormal pupil
Light-near dissociation
Pupils usually large
Slightly unequal
Often oval in shape
ARGYLL ROBERTSON PUPIL
(ARP)
ARP: Accomodation Reflex Present
PRA : Pupillary Reflex Absent
SYPHILIS ??
 ADIE’S

DENERVATION
ABERRANT REINNERVATION
DENERVATION SUPERSENSITIVITY
LIGHT–NEAR DISSOCIATION
?? TONIC
?? SYNDROME
Acute ADIE’S
LITTLE OLD ADIE’S
 Iritis

PUPIL

Trauma Drugs
TRAUMA (the 7 rings)
 IRITS (Posterior synechiae)

One point : kidney shaped pupil upon

dilation, no effect on aqueous dynamics
Multiple points : festooned pupil upon
dilation no effect on aqueous dynamics,
Ring synechiae : iris seclusion(no dilation) &
iris bombe → CAG
Total synechiae : no dilation , no bombe but
2ry CAG?
PHARMACOLOGIC BLOCKADE

NOT ONLY BY DRUGS

RUBBING RATHER THAN INSTILLING
NO REACTION TO LIGHT
NO REACTION TO NEAR
NO REACTION TO 1% PILOCARPINE
PHYSIOLOGIC ANISOCORIA

Small difference
Same in bright or dim light
Sound pupil
LIGHT NEAR DISSOCIATION
HIPPUS