Pupil Lecture

for my pupils

Danah Albreiki
Some slides courtesy of Dr. V Patel

2015
 Outline
• Anatomy of the pupillary pathway
• Afferent limb
• Efferent limb :
• Para-sympathetics
• Sympathetics

• Approach to pupil abnormalities

• RAPD (equal in size)
• Anisocoria:
• Simple anisocoria
• Horner’s syndrome
• Tonic pupil
• 3rd nerve palsy
• Light near dissociation
ANATOMY
 Neuro-anatomy and physiology

• Function of the pupils:

– Vary the quantity of the light
reaching the retina
– Minimize the spherical aberration of
the peripheral cornea and lens
• Iris:
– Two muscles that control the size of
the pupil:
• Dilator m (more peripherally
located encircling the pupil)
• Sphincter m (360 around pupil
margin)
• Ciliary Body : Accommodation
 Afferent limb

Pathology

RAPD
 Efferent
• Parasympathetics (efferent of the light reflex
arc )

• Sympathetics
 Efferent limb - Parasympathetic

Pathology

Pathologic mydriasis
(tonic pupil or third
nerve palsy)
Brain stem
 Brain Stem
Sub-Arachnoid space
 Sub-Arachnoid space

• Pupillary fibers
concentrated
superonasally

• PCOM superonasal
to third nerve
Cavernous sinus
 Superior Orbital fissure

• Along the inferior

branch of the
third nerve
 Ciliary Ganglion
• Synapse in ciliary ganglion

• Ciliary ganglion lies in

posterior part of the orbit
b/w ON and LR m

• CG issues post-ganglionic
cholinergic short ciliary
nerves

• With inferior branch of IO,

then b/w sclera and
choroid to :
– CB
– Iris sphincter
Ratio 30:1

• Adie’s pupil : pathology in

CG
Ciliary Ganglion
Efferent limb - Sympathetics
 Sympathetics
• Start at the poster-lateral
hypothalamus and ends at the
iris dilator and eyelids

Pathology

Horner syndrome
Sympathetics pathway

Lower Brachial plexus

Lung Apex
Cavernous sinus
Superior Orbital Fissure
Ciliary Ganglion
Now that you know your anatomy…
 Approach to pupil abnormalities

• RAPD ( equal in size )

• Anisocoria:
– Simple anisocoria
– Horner’s syndrome
– Tonic pupil
– 3rd nerve palsy
• Light near dissociation
 Relative afferent pupillary defect

• Asymmetric optic neuropathy

• Asymmetric chiasmal disease
• Disease of the optic tract
• Pathology in and around the optic tectum
Can you have an isolated RAPD with no previous or
current vision loss?
 Approach to pupil abnormalities

• RAPD ( equal in size )

• Anisocoria:
– Simple anisocoria
– Horner’s syndrome
– Tonic pupil
– 3rd nerve palsy
• Light near dissociation
 Anisocoria

Which is the pathological pupil?

 Case

• 36 female : neck pain and

anisocoria

• Visit to hair dresser

 Asymmetry Greater in the dark- Anisocoria Algorithm
Pathologic SMALL pupil

Dilation lag and ptosis

Pharmacologic testing

1% apraclonidine (reversal of anisocria)

Or 10% cocaine (normal dilates, abnormal no response)

Negative- PHYSIOLOGIC Positive- HORNER

Wait 24-48 hours

Localize clinically

hydroxyamphetamine
CT lung apex/ MRI neck/ Brain
MRI CS/MRA CTA neck
Anisocoria decreases-
Anisocoria same-
PREGENAGLIONIC or
CENTRAL POSTGANGLIONIC
 Simple Anisocoria
• 20% of normal population

• Anisocoria may increase in darkness but usually decreases in

light

• Pupils react normally to light and near

• Incidence increases with age

• 20% of patients with a unilateral ptosis will have an anisocoria

 Asymmetry Greater in the dark- Anisocoria Algorithm
Pathologic SMALL pupil

Dilation lag and ptosis

Pharmacologic testing

1% apraclonidine (reversal of anisocria)

Or 10% cocaine (normal dilates, abnormal no response)

Negative- PHYSIOLOGIC Positive- HORNER

Wait 24-48 hours

Localize clinically

hydroxyamphetamine
CT lung apex/ MRI neck/ Brain
MRI CS/MRA CTA neck
Anisocoria decreases-
Anisocoria same-
PREGENAGLIONIC or
CENTRAL POSTGANGLIONIC
 Back to case
• + dilation lag

• Apraclonidine test : reversal of

anisocoria

• Carotid doppler : Carotid

dissection
 Painful Horner is an emergency!
It’s a carotid dissection until proven
otherwise
 Dissection of internal carotid artery

• May be spontaneous or
traumatic

• May present with headache,

neck, or facial pain

• Horner’s in 50% of patients

• Stroke due to carotid

occlusion or emboli

• Hx : chiropractor , hair
dresser visit, neck trauma
(whiplash) or manipulation
 Horner’s syndrome

• Miosis – dilator pupillae

• Ptosis – muller’s muscle
• Facial ahidrosis – sudomotor
fibers
• Pseudo-enophthalmos – inferior
lid retractors
 Central Horner’s syndrome
• Lesions of thalamus or
hypothalamus
– Contralateral hemiparesis
– Contralateral hypesthesia

• Midbrain lesion

• Pontine lesion

• Medullary lesion (Wallenberg’s

syndrom )
 Wallenberg’s Syndrome
• Inferior cerebellar peduncle
– Ipsilateral ataxia
• Descending sympathetics
– Ipsilateral Horner’s
• Nucleus ambiguous
– Dysphagia, hoarseness
• Spino-thalamic tract
– Contralateral body decrease
in pain, temp
• Descending tract of V
– Ipsilateral decrease in facial
pain, temp
• Nucleus solitarius
– Dysphagia, taste
 Pre-ganglionic Horner’s syndrome
• Syringomyelia
• Fracture-dislocation of cervical spine
• Traction on the brachial plexus
• Trauma to thorax or neck
• Tumors of mediastinum or pulmonary apex
• Thoracic aneurysm
– Increasing pain in neck, shoulder and
upper chest
– Dry cough, hoarseness
• Carotid dissection
• Atherosclerosis of internal or external carotid
• Neck SOL
• Nasopharyngeal CA
– Ipsilateral paralysis of tongue, anesthesia
of pharynx and dysphagia
 Postganglionic Horner’s

• Cavernous sinus lesions

– Trigeminal neuropathy
– Ocular motor
neuropathy
• Cluster headache
• Middle cranial fossa mass
encroaching on Meckel’s
cave and the internal
carotid artery at the
foramen lacerum
• Basal skull fracture
• Carotid dissection
 Horner’s syndrome – pharmacologic
tests

• Cocaine test
• Paredrine test
• Iopidine test
 Cocaine test

• Cocaine inhibits re-

uptake of
norepinephrine

• Horner’s pupils show

poor dilation regardless
of level

• Anisocoria>0.8 mm
indicative of Horner’s
 Paredrine test

• Paredrine stimulates presynaptic

release of norepinephrine

• Distinguishes presynaptic or
central from postganglionic
lesions

• Postganglionic will NOT dilate

• inaccurate within 24-48 hours of

cocaine test
– Failure of affected pupil to
dilate
 Apraclonidine (iopidine test)

• Alpha receptor agonist

• Dennervation
supersensitivity of alpha
receptors in the iris dilator

• Pupillary dilation in
Horner’s syndrome

• No effect in normal pupils

• Therefore, reversal of
anisocoria
 Approach to disorders of the pupil

• Equal in size
– Relative afferent pupillary defect
• Anisocoria
– Simple anisocoria
– Horner’s syndrome
– Tonic pupil
– 3rd nerve palsy
• Light – near dissociation
 Case

• 36 female
• Asymmetric pupils
Which Is the pathologic pupil ?
 Greater in the light- Anisocoria Algorithm
Pathologic DILATED pupil

Normal EOM Abnormal EOM

SLE TNP

Abnormal iris structure

Segmental constriction
IRIS DAMAGE

Pilocarpine (0.125%)

Pupil constricts No constriction

Pilocarpine 1%
TONIC or
POSTGANGLIONIC LESION
Pupil constricts- No constriction –
TNP PHARAMCOLOGIC BLOCKADE
 Ciliary Ganglion

• Only 3% of cells in the ciliary

ganglion give rise to axons
that supply the iris sphincter

• THEREFORE, the vast

majority of the midbrain
parasympathetic outflow is
concerned with
accommodation and not with
pupillary constriction
 Tonic pupil

• Therefore, aberrant
regeneration will favour re-
innervation by an
accomodative axon rather
than by an axon driven by
the direct light reflex.

• This is the basis of the light

– near dissociation seen in
the tonic pupil
Tonic pupil right eye
 Adie’s Tonic Pupil

• Affected pupil starts off larger but becomes smaller

with time. At onset unilateral but may become
bilateral.

• Benign lesion of ciliary ganglion resulting in

neuronal loss and aberrant regeneration. Affects
mostly women in their 20’s and 30’s.

• Often decreased deep tendon reflexes (achilles most

common) – Holmes Adie’s syndrome
 Adie’s Tonic Pupil

• Light reaction poor or absent. Accommodation is

slow and tonic. Can mimic A-R pupils when
bilateral

• Poor re-dilation after accommodation and

vermiform movements are common

• Light / near dissociation possible after some

regeneration has occurred (8 weeks)
 Adie’s Syndrome

• Accommodation paresis recovers

• Pupillary light reaction does not recover and may

worsen

• DTR tend to become increasingly hyporeflexic

• Affected pupil becomes smaller

• Tendency for bilaterality with increasing age

 Argyll Robertson Pupils

• Small and dilate poorly in

darkness
• Pupils irregular due to iris
damage
• Light-near dissociation
• Bilateral and symmetric
• Dilate and constrict well
pharmacologically if no iris
atrophy
• Distinguish from bilateral tonic
pupils
 Dorsal Midbrain Syndrome

• Supranuclear vertical gaze palsy

• Accommodation abnormalities
• Convergence retraction nystagmus
• Affected pupils are larger , often light /
near dissociation
• Lid retracation (Collier’s sign)
• Skew deviation

• Can be caused by a pinealoma, stroke,

or MS
• Papilledema frequently with pinealoma
 Approach to disorders of the pupil

• Equal in size
– Relative afferent pupillary defect
• Anisocoria
– Simple anisocoria
– Horner’s syndrome
– Tonic pupil
– 3rd nerve palsy
• Light – near dissociation
3rd nerve palsy and pupillary
involvement
 Approach to disorders of the pupil

• Equal in size
– Relative afferent pupillary defect
• Anisocoria
– Simple anisocoria
– Horner’s syndrome
– Tonic pupil
– 3rd nerve palsy
• Light – near dissociation
 Near reaction

• Convergence,
accommodation, pupillary
constriction

• Only important
diagnostically if near
reaction better than light
reaction
 L-N dissociation
• DDx:
– De-afferentiation
– Tonic pupils
– Adie’s
– Tectal lesions:
Parinaud syndrome
– ARP
– Aberrant regeneration
of the third nerve
– DM
 Case
• 34 female
• H/A
• 32 weeks pregnant
• Incidental Horner
 Case

• 58 male H/A R/O GCA

• CC: severe H/A for last one month. No other GCA Sx, Labs
normal. No trauma

• O/E : ptosis and miosis

• + dilation lag

• Reversal of Anisocoria with Iopidine

Carotid dissection
Thank you
 Defective pupillary light reaction
• Associated with vision loss:
– RAPD
– Amaurotic (deafferented) pupil

• Not associoted with vision loss :

– Pre-tectal pupils
– Argyll Robertson pupils
– Third nerve palsy
– Tonic pupils
– Pharmacologically dilated pupils

• Ocular causes of un-reactive pupils:

– Trauma
– ACG
– Iritis
– Congenital mydriasis
 Abnormally shaped pupils
Will not be discussed in this lecture
• Congenital :
– Aniridia
– Ectopia lentis et pupillae
– Iris coloboma
– AC cleavage abnormalities
– Ectopic pupils
– Persistent pupillary membranes
• Acquired :
– Iritis
– Trauma
– Iris atrophy (DM, herpes)
– Neurologic causes ( tonic, neuro-syphilis, severe MB damage,
tad-pole )
Dilation lag video
 Case

• 60 female
• Ptosis and EOM restriction
• Cavernous ICA aneurysm
• Video