Day 12 (1): The Pupil Flashcards

1
Q

What is the pupil?

A
  • normal: round, centrally-located, symmetric
  • size depends on ambient illumination
    1. constriction: light stimulus; near target
    2. dilation: low-light environment

2 muscles:
1. Iris Sphincter
- contraction –> pupil constriction = MIOSIS
- location: 2 - 3 mm from margin
- innervation: Parasympathetic only via CN3

  1. Iris Dilator
    - contraction –> pupil dilation = MYDRIASIS
    - location: iris root
    - innervation: Sympathetic only via Oculosympathetic system
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2
Q

What is Hippus or Pupillary Unrest?

A
  • NORMAL, rhythmic pupillary oscillation seen with light stimulation
  • balance between opposing innervations by the parasympathetic (sphincter) and sympathetic (dilator) systems
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3
Q

What is the Near Reflex?

A
  • three-component SYNKINETIC reflex that assist in the redirection of gaze from a distant to a nearby object
  • synkinetic: voluntary muscle movement causes the simultaneous involuntary contraction of other muscles

Components:
1. pupillary constriction: iris sphincter
2. lens accommodation: ciliary muscles
3. convergence: medial rectus

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4
Q

Enumerate the pathway of the Pupillary Light and Near Reflexes.

A

Common Afferent Pathway:
1. Retina
2. Optic Nerve
3. Optic Chiasm
4. Distal Optic Tract

Light Pathway
1. Retino-Tectal Tract via the Brachium of the Superior Colliculus
2. Pretectal Nucleus: innervation to both sides
3. EW nucleus

Near Pathway
1. Proximal Optic Tract
2. Lateral Geniculate Nucleus
3. Optic Radiations
4. Striate (Primary Visual) Cortex
5. Parastriate Cortex
6. Thalamomesencephalic Junction
7. EW Nucleus + Main Motor Nucleus of III

Common Efferent Pathway
1. Oculomotor nerve –> MR (near only)
2. Ciliary ganglion
3. Short Ciliary nerve –> Iris Sphincter (light and near), Ciliary Muscles (near only)

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5
Q

What are the components of the AFFERENT pathway of the Pupillary Reflex?

A

Melanopsin system
- light reflex + near reflex: non-image-forming
- vs Rhodopsin system: image-forming (visual)

A. Common Visual and Pupillary Pathway:
1. RGC: intrinsically-photosensitive
2. Optic Nerve
3. Optic Chiasm
4. Anterior/Distal optic tract

*Lesion:
- POOR vision
- POOR light reflex
- GOOD near reflex: synkinetic reflex; converging the eyes also triggers pupillary constriction and lens accommodation even if there is no vision (ask pt to converge eyes towards nose)

*Note: Vision is NOT a prerequisite for accommodation.

B. Divergence of Visual from Pupillary pathway:
4. Posterior/Proximal Optic Tract
–> Tract to the LGN: visual pathway
–> Retino-tectal tract via the Brachium of the Superior Colliculus: pupillary light pathway

*Lesion in the LGN, Optic radiations and Visual cortex:
- POOR vision
- GOOD light reflex
- GOOD near reflex

C. Pupillary Pathway:
5. Brachium –> Superior Colliculus –> Pretectal nucleus
- located in the dorsal midbrain
- supranuclear control center of the light reflex
- part of the pupillary LIGHT pathway BUT NOT the near reflex (Light-Near Dissociation)

*Lesion:
- GOOD vision
- POOR light reflex
- GOOD near reflex

  1. Edinger-Westphal nuclei
    - NEAR reflex: fibers descends directly here from the Thalamomesencephalic Junction which is the supranuclear control center of the near synkinetic reflex

*Lesions:
- GOOD vision
- POOR light reflex
- POOR near reflex

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6
Q

What is the Superior Colliculus?

A
  • paired multilayered structure located in the dorsal midbrain
  • non-mammals: optic tectum/lobe

Layer groups:
1. Superficial Layers
- receive direct input from the retina
- respond ONLY to visual stimuli

  1. Deeper Layers
    - respond to other stimuli such as auditory and somatosensory inputs
    - connected to many sensorimotor areas
    - contain a population of motor-related neurons, capable of activating eye movements

Function:
- direct behavioral responses toward specific points in body-centered space
- each of the two colliculi contains a 2D topographic map representing half of the visual field in retinotopic coordinates
- activation of neurons at a particular point in the map evokes a response directed toward the corresponding point in space
- receives a strong input directly from the retina BUT is largely under the control of the cerebral cortex

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7
Q

What is Light-Near Dissociation?

A
  • differential response of the pupils to light stimulus and near object
    + light reflex: IMPAIRED
    + near reflex: INTACT
  • more commonly affects the afferent pathway
  • may sometimes affect the efferent pathway in chronic cases due to aberrant regeneration

Causes:
1. Afferent Pathway: lesions anterior to LGN
- most common cause
- near reflex is synkinetic: converging the eyes (by ask pt to converge eyes towards nose) also triggers pupillary constriction and lens accommodation even if there is no vision
+ Unilateral: RAPD
+ Bilateral, asymmetric: RAPD
+ Bilateral, symmetric: BAPD

  1. Dorsal Midbrain:
    - disruption of light reflex fibers which pass through the pretectal nucleus with sparing of the near reflex fibers which descend directly to the EW nucleus from the Thalamomesencephalic Junction
    + Dorsal Midbrain Syndrome
    + Argyll-Robertson Pupil
    + Pineal Tumor
  2. Efferent Pathway
    - especially at the level of the ciliary ganglion which normally innervates the ciliary muscles and the iris sphincter in a 30:1 nerve fiber ration
    - due to aberrant regeneration, the fibers originally intended for the ciliary muscles innervate the pupils instead
    - when the near synkinetic reflex is triggered, the stronger innervation goes instead to the iris sphincter causing prominent constriction
    + EWN, CN3: BOTH poor; (+) RRAPD
    + CG, SCN (acute): BOTH poor; (+) RRAPD
    + CG, SCN (chronic): (+) LND
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8
Q

What are the components of the PARASYMPATHETIC EFFERENT pathway of the Pupillary Reflex?

A

Parasympathetic pathway:
- pupillary constriction or MIOSIS

  1. Edinger-Westphal nuclei
  2. Oculomotor nerve (CN 3)
  3. Ciliary ganglion: within orbital muscle cone between the LR and the ON
  4. Short Ciliary nerve
  5. Ciliary Muscles and Iris Sphincter (30:1)
    - for every 1 fiber to the iris sphincter, the ciliary muscles receive 30 fibers
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9
Q

What are the components of the SYMPATHETIC EFFERENT pathway of the Pupillary Reflex?

A

Sympathetic pathway:
- pupillary dilation or MYDRIASIS
- 3-neuron pathway

First-Order Neuron (Pre-ganglionic)
- Hypothalamus –> Midbrain –> Pons –> Medulla –> Ciliospinal Center of Budge (level of C8 - T2)
- at midbrain: close to the Trochlear nucleus

Second-Order Neuron (Pre-ganglionic)
- Ciliospinal Center of Budge –> exits sympathetic trunk –> Lung apex –> loop around Subclavian artery in the Brachial plexus –> Cervical Plexus –> Superior Cervical Ganglion (near the mandibular angle at the bifurcation of the common carotid artery)

Third-Order Neuron (Post-ganglionic)
- Superior Cervical Ganglion –> along with ICA (in its adventitia) –> Skull base –> Cavernous Sinus –> along with Abducens nerve –> transfers to Ophthalmic division of Trigeminal nerve –> SOF via the Nasociliary nerve –>

  1. Long Ciliary nerve:
    - Iris Dilator
    - Muller’s muscle: superior lid
    - Inferior tarsal muscle: inferior lid
  2. Sudomotor (facial sweating) and Vasomotor (vasodilation) fibers branch off at the Superior Cervical Ganglion

Clinical Correlation:
1. Lesions in the 1st and 2nd order neurons upto the Superior Cervical Ganglion:
(+) facial anhidrosis

  1. Lesions in the 3rd order neurons from the ICA to the orbit: (+) facial sweating
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10
Q

How is the pupil examined?

A

Set-up:
- dark room
- bright pen light
- fixating at distance

Parameters:
1. Size (mm)
2. Shape
3. Centration
4. Symmetry: anisocoria (unequal pupil size)
5. Light response: direct and consensual
6. Dilation in the dark
7. Constriction at near

Slit-Lamp Examination
- done if with abnormal findings
1. posterior synechiae
2. signs of uveitis
3. iris tears
4. shape irregularities
5. iris segmental contractions
6. iris transillumination defects
7. pigment dispersion
8. iris tumors
9. lens subluxation

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11
Q

How is the light response of the pupil examined?

A
  • shining light in one eye should cause BOTH pupils to constrict EQUALLY
  1. Direct Response
    - pupillary response in the ILLUMINATED eye
  2. Consensual Response
    - pupillary response in the FELLOW eye
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12
Q

What is a Relative Afferent Pupillary Defect?

A

Marcus-Gunn Pupil
- condition demonstrating a difference in pupillary light reaction between two eyes
- requires two eyes but only one working pupil
- due to defect in the afferent pupillary pathway
- DIRECT RESPONSE: dilation or pupillary escape in the AFFECTED eye “relative” to the normal eye
- tested by the Swinging Flashlight Test
- detected only in:
1. unilateral disease
2. bilateral but asymmetric disease
+ RAPD manifests in the eye with the worse disease
+ if bilateral AND symmetric = Bilateral APD

Causes:
- occurs in lesions affecting the visual pathway in FRONT of the lateral geniculate nucleus

  1. Optic neuropathy: most common
    - most objective clinical sign of optic neuropathy
    - if suspect but has (-) RAPD:
    + optic neuropathy RULED OUT
    + optic neuropathy bilateral and symmetric
  2. SEVERE retinal disease: most common
  3. Maculopathy: trace RAPD only when VA worse than 20/200
  4. Amblyopia: trace RAPD only when VA worse than 20/200
  5. Post-chiasmal lesions: contralateral RAPD
    - remember that most fibers in the ON decussate to the contralateral tract
    - optic tract lesions: (+) vision loss
    - tectal RAPD (lesions in the brachium, superior colliculus, pretectal nucleus): (-) vision loss

When is an RAPD not observed?
1. D: no defect
2. P: (+) defect BUT NOT in pupillary pathway
3. A: (+) defect in the pupillary pathway BUT NOT in the afferent side
4. R: (+) defect but no comparison relative to the other eye (bilaterally symmetric disease)

Note:
1. Any problem in FRONT of the retina (cataract, corneal disease, refractive errors, tear film dysfunction) DO NOT produce an RAPD
- ANTERIOR to the pupillary pathway
- there may be vision loss due to poor light transmission but the pupillary light reflex is still intact because the melanopsin system in the retina is still activated by the incoming, albeit disordered, light rays

  1. Any problem in the LGN and beyond also DO NOT produce an RAPD
    - POSTERIOR to the pupillary pathway
    - because the pretectal neurons in the retino-tectal tract have already entered the Brachium prior to reaching the LGN
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13
Q

What is the Reverse RAPD?

A

RAPD by Reverse Testing
- employed when affected eye also has an EFFERENT pupillary defect
+ posterior synechiae
+ trauma
+ oculomotor palsy
+ pharmacological mydriasis
- also utilizes the Swinging Flashlight Test
- CONSENSUAL RESPONSE: dilation or pupillary escape in the NORMAL eye

REMEMBER:
The reverse RAPD is present every time because both pupils dilate when the light swings back to the abnormal pupil but:
1. RAPD: clinician observes the AFFECTED eye for dilation (DIRECT)
2. Reverse RAPD: clinician observed the NORMAL eye for dilation (CONSENSUAL)

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14
Q

What is Anisocoria?

A
  • UNequal pupil size

RAPD vs Anisocoria
- RAPD: AFFERENT pathway problem
- Anisocoria: EFFERENT pathway problem

Differentials:
1. Check pupillary reaction to light
2. Measure pupil size in both bright and dim illumination

A. EQUALLY reactive to light OR anisocoria greater in the DARK
- problem in the SYMPATHETIC (dilation) system
- light: both constricts due to intact parasympathetic system
- dark: normal eye dilates while affected eye remains constricted –> anisocoria
1. Physiologic Anisocoria
2. Horner’s Syndrome
3. Intermittent Pupillary Mydriasis (overactive)

B. NOT equally reactive OR anisocoria greater in LIGHT
- problem in the PARASYMPATHETIC (constriction) system
- dark: both dilates due to intact sympathetic system
- light: normal eye constricts while affected eye remains dilated –> anisocoria
1. Traumatic iris damage
2. Oculomotor nerve palsy
3. Adie’s Tonic Pupil
4. Pharmacologic Mydriasis
5. Intermittent Pupillary Mydriasis (underactive)
6. Argyll-Robertson Pupil

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15
Q

What is Physiologic Anisocoria?

A

Essential Anisocoria/Normal Benign Anisocoria
- equally reactive (constricts) to light
- anisocoria in light = anisocoria in dark
- in 20% of population
- size difference: < 0.5 mm
- may come and go and may switch sides

Dx:
- confirmed with old photos: establish chronicity
- eye exam normal (VA, motility, lids)

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16
Q

What is Horner Syndrome?

A

Oculosympathetic Paresis
- interruption of oculosympathetic pathway
- equally constricts to light
- (+) dilation lag: slow redilation in the dark OR anisocoria exacerbated by dark

Findings:
1. Pseudoenophthalmos due to smaller palpebral fissure
- upper lid ptosis: Muller’s muscle
- lower lid elevation/reverse ptosis: Inferior Tarsal muscle
2. Miosis: predominant parasympathetic signal
3. Iris heterochromia: Congenital Horner Syndrome
4. Brainstem and spinal cord s/sx: 1st order
5. Arm pain, hand weakness, neck pain, neck trauma, history of neck surgery: 2nd order
6. Anhidrosis or blanching of ipsilateral face: PREganglionic (1st or 2nd order)
- if facial sweating and flushing intact: POSTganglionic (3rd order)
7. Ipsilateral facial pain: 3rd order (due to connection with CN5A)

Localization:
1st order: hypothalamus, brainstem, cervicothoracic spine
2nd order: cervicothoracic spine, pulmonary apical lesions, brachial plexus injury, subclavian aneurysm, iatrogenic cervical injury
3rd order: iatrogenic cervical injury, ICA disease, skull base lesions, cavernous sinus disease

Clues:
1. Ipsilateral Horner’s + Contralateral CN4 palsy = BRAINSTEM (near the ipsilateral trochlear nucleus)
2. Ipsilateral Horner’s + Ipsilateral CN6 palsy = CAVERNOUS SINUS
3. Ipsilateral Horner’s + Harlequin Sign = image entire sympathetic axis
4. Ipsilateral Horner’s + acute + painful =
ICA until proven otherwise [EMERGENCY]
5. Ipsilateral Horner’s + child = NEUROBLASTOMA
- may present with cervical or abdominal mass
- increased production of catecholamines and metabolites in the urine and blood
+ Vanillylmandelic acid (VMA)
+ Homovanillic acid (HVA)
- Dx:
+ MRI with contrast of head, neck, chest and abdomen
+ Urine VMA and HVA

17
Q

How is Horner Syndrome diagnosed?

A

A. Clinical
1. Ptosis
2. Reverse ptosis
3. Dilation lag
4. Anhidrosis + blanching: if preganglionic

B. Pharmacologic confirmation
1. COCAINE: confirmatory test for Horner’s
- MOA: blocks reuptake of NE from synaptic cleft
- place 1 drop then observe after 1 hr
- normal pupil with intact sympathetic innervation will dilate
- INCREASES degree of anisocoria
+ Normal pupil: dilates
+ Miotic pupil: NO change

  1. APRACLONIDINE: alternative to cocaine but not available locally
    - MOA: WEAK alpha-agonist
    - place 1 drop then observe after 30-45 mins
    - affected pupil will dilate because of denervation sensitivity seen after 5 - 8 days
    - Reversal: DECREASES degree of anisocoria
    + Normal pupil: NO change
    + Miotic pupil: dilates
    + Note: false-NEGATIVE in ACUTE cases because alpha-1 receptors NOT yet upregulated in acute cases
    - if NO reversal: Physiologic Anisocoria
  2. HYDROXYAMPHETAMINE: differentiates pre- from postganglionic Horner’s
    - MOA: releases NE from POSTganglionic axons
    - place 1 drop then observe after 1 hr
    - result:
    + PRE: affected pupil dilates
    + POST: affected pupil NO change
    + Note: 24-48 hrs interval with cocaine test because cocaine can inhibit uptake of HA into the presynaptic vesicles, decreasing HA test accuracy
  3. PHENYLEPHRINE: confirms post-ganglionic Horner’s
    - MOA: STRONG alpha-1 agonist
    - dilute 10% solution in 0.9 mL preservative free natural tears (= 1%) and place 1 drop and observe after 1 hr
    - affected pupil will dilate because of denervation sensitivity seen after 5 - 8 days
    - result:
    + PRE: affected pupil NO change
    + POST: affected pupil dilates (REVERSAL)

C. Determine the etiology
- any lesion in the sympathetic pathway
1. CT/MRI with contrast of the head and neck
2. CTA of the head and neck

18
Q

What is the Law of Denervation Sensitivity?

A
  • an organ depleted of its normal innervation becomes more sensitive to the chemical transmitters normally released from those nerves
19
Q

What is the Harlequin Sign/Syndrome?

A
  • pathogenesis unclear but likely due to autonomic dysfunction
  • brought on by heat stress, exercise, or sudden emotion in the patient

Signs:
A. PREganglionic: 1st- or 2nd-order neuron
1. IPSILATERAL anhidrosis & blanching in the face, neck and face
2. CONTRALATERAL hyperhidrosis & flushing
3. IPSILATERAL Horner syndrome (ptosis, reverse ptosis, miosis)

B. POSTganglionic: 3rd-order neuron
(-) Horner syndrome

Treatment: based on diagnosed etiology
1. Idiopathic: no treatment needed; avoid aggravating factors
2. Severe: sympathectomy

20
Q

What is Intermittent Pupillary Mydriasis

A

2 Forms:
1. Sympathetic OVERactivity
- unilateral mydriatic pupil
- EQUALLY reactive to light and near target
- transient and intermittent (15 mins - 1 hour)
- asymptomatic if isolated
- may be associated with migraine
- normal eye exam (EOMs, lids)
- Tadpole Pupil: peaked pupil due to segmental spasm of dilator muscle causing one segment of the iris to be pulled to a peak

  1. Parasympathetic UNDERactivity
    - unilateral mydriatic pupil that is NOT or POORLY reactive to light and near target
    - transient and intermittent (15 mins - 1 hour)
    - asymptomatic if isolated
    - may be associated with migraine
    - normal eye exam (EOMs, lids)
    + vs CN3 Palsy: EOM dysfunction + ptosis
21
Q

What happens in iris sphincter damage?

A
  • irregularly-dilated pupil that’s POORLY reactive to light and near target
  • anisocoria exacerbated by light
  • associated with blunt trauma, iatrogenic injury or uveitic damage (iritis)
  • Dx: Slit-lamp Examination
    1. iris sphincter tear
    2. posterior synechiae
22
Q

What happens in oculomotor nerve palsy?

A
  • mid-dilated pupil that’s POORLY reactive to light and near target
  • anisocoria exacerbated by light
  • ALWAYS associated with EOM deficits:
    1. Adducted: MR
    2. Elevated: IO + SR > IR
    3. Extorted: IO + IR > SR
    2. Ptosis: LP

Remember:
ISOLATED oculomotor palsy + headache:
(-) RRAPD: lesion in the orbital apex or pituitary apoplexy
(+) RAPD: possible aneurysm of the posterior communicating artery [EMERGENCY]

Dx: Cranial CT scan with contrast

23
Q

What is Tonic Pupil?

A
  • parasympathetic denervation leading to isolated iris sphincter or ciliary muscle dysfunction
  • parasympathetic fibers: EW nuclei –> travel with CN3 –> Ciliary ganglion –> Short Ciliary nerves –> ciliary muscles (30x) and iris (1x)

Cause: damage to the Ciliary Ganglion or the Short Ciliary nerves
1. Trauma
2. Autonomic disorders (DM, Sjogren)
3. Iatrogenic injury (PRP, Orbital surgery)
4. Ischemia (Giant Cell Arteritis)
5. Infection (VZV, HSV)
6. Idiopathic

Findings:
1. Mydriasis
- sympathetic innervation predominates in the affected eye

  1. Anisocoria exacerbated by light
    - normal eye: constricts
    - affected eye: remains dilated
  2. Light-Near Dissociation
    - Poorly reactive to light: segmental palsy only or show vermiform movements
    - Reactive to near targets

Denervation Sensitivity
- postsynaptic receptors UPregulated to facilitate reinnervation –> increased sensitivity of iris sphincter to acetylcholine
- CHRONIC cases: aberrant regeneration –> redirection of the fibers for the CB to the iris –> when accommodating, signals meant for the CB goes instead to the iris causing miosis
- effect: TONIC iris constriction with slow redilation when focusing back at distance

  1. Other eye examinations normal (lids, EOMs)
    - rules out Oculomotor palsy

Symptoms:
1. Anisocoria: affected eye mydriatic
2. Photophobia: inability to constrict to light
3. Difficulty adapting to dark: tonic constriction
4. Blurring of vision at near: impaired accommodation because signals meant for the ciliary muscles goes to the iris instead

Diagnosis: Pilocarpine 0.1%
- 0.1 mL of 1% Pilocarpine + 0.9 mL saline
- muscarinic agonist
- based on principle of denervation sensitivity
- present only in CHRONIC cases; if acute –> false-negative
- place 1 drop and check after 30 - 45 minutes
- REVERSAL of anisocoria
+ normal pupil: NO change
+ affected pupil: constricts

Others:
1. CBC, ESR, CRP: r/o GCA
2. FBS, HbA1c: r/o DM
3. Syphilis, HIV: r/o infectious causes
4. Orbital CT: r/o tumor

Holmes-Adie Syndrome
- tonic pupil in women aged 20 - 40
- idiopathic
- unilateral
- associated with decreased or absent DTR
- Dx: NONE

24
Q

What happens in Pharmacologic Pupil?

A
  • causes fixed mydriasis or miosis depending on the etiologic agent
  • etiologic agent causes stimulation or inhibition of either the parasympathetic or sympathetic system
    + Topical: unilateral (–> anisocoria)
    + Systemic: bilateral

Mydriasis: largest pupil (7 - 8 mm)
1. Alkaloids: cholinergic antagonist
- Pesticides: Gardener’s Pupil
2. Parasympatholytic cycloplegics: strong muscarinic antagonists that can paralyze the ciliary muscles and disrupt accommodation
- Atropine
- Cyclopentolate
- Tropicamide
- Scopolamine
- Ipratropium, Tiotropium
3. Sympathomimetics
- Apraclonidine: alpha-agonist
- Phenylephrine: alpha-agonist
- Salbutamol, Formoterol: beta-2 agonists

Note: Both parasympatholytic and sympathomimetic drugs cause pupil dilation BUT ONLY parasympatholytic can cause cycloplegia because ciliary muscles DON’T have adrenergic receptors

Miosis: smallest pupil (1 - 2 mm)
1. Pilocarpine: muscarinic agonist
2. Organophosphates: acetylcholinesterase inhibitor

PE:
1. Anisocoria (if unilateral involvement from topical pharmacologic agent)
2. Fixed pupils: NO reaction to light or accommodation
3. Normal lids, EOMs and neurologic exam

Diagnosis
1. Pilocarpine 1 - 2% drops
- test for pharmacologic MYDRIASIS
- instill 2 drops and check after 30 - 45 minutes
- normal eye: constricts
- affected eye: NO change

  1. Tropicamide 1%
    - test for pharmacologic MIOSIS
    - instill 2 drops and check after 30 - 45 minutes
    - normal eye: dilates
    - affected eye: NO change
25
Q

What is Argyll-Robertson Pupil?

A
  • bilateral irregular miotic pupils
  • (+) bilateral light-near dissociation
  • Prostitute’s Pupil: accommodates but does not react

Pathology: damage to dorsal midbrain
1. damage to the pretectal nucleus at the region of the superior colliculus interrupts the pupillary light reflex
2. ventrally-located near reflex fibers from the higher cortical centers descending directly to the EW nucleus are spared
3. supranuclear adrenergic fibers from the higher cortical centers exert an INHIBITORY action on the basal firing rate of the parasympathetic neurons in the EW nucleus
- damage to the inhibitory fibers causes unopposed firing of the EW nucleus causing sustained miosis
4. lesions can develop chronically, asymmetrically and unevenly –> slow to loss or light reflex

Etiology:
1. Neurosyphilis: Prostitute’s Pupil
2. Diabetes mellitus
3. Neurosarcoidosis
4. Encephalitis
5. Demyelination from chronic alcoholism
6. Multiple sclerosis
7. Herpes zoster

PE:
1. Bilateral irregular, miotic pupils: < 2 mm
- do NOT dilate fully to light due to unopposed parasympathetic innervation
- NO changes with pharmacologic mydriasis
2. Light-near dissociation
- sluggish or absent light reflex
- intact near reflex with brisk redilation
- near reflex may also be impaired once lesion enlarges causing a FIXED MIOTIC pupil
3. (+/-) Iris atrophy and transillumination defects

Ddx: Tonic Pupil
- NO tonic constriction to near
- NO blurring of vision and able to accommodate at near
- BRISK redilation to distance

Tx: determine and treat primary cause

26
Q

What is the Dorsal Midbrain Syndrome?

A

Parinaud Syndrome/Pretectal Syndrome/Sylvian Aqueduct Syndrome/ Koerber-Salus-Elschnig Syndrome

Cause: lesions affecting the dorsal midbrain
1. Young: pineal region tumors or mass effect
- pineocytoma, pinealoma, pineal cyst
- germinoma
- obstructive hydrocephalus: 4th ventricle
2. Elderly:
- brainstem hemorrhage
- ischemic infarction

Hallmarks:
1. Triad: complete in only 65% of cases
- Upward gaze palsy
- Convergence-Retraction Nystagmus
- Light-Near Dissociation
2. Collier’s Sign: only in 20 - 40% of patients
- Bilateral lid retraction

Diagnosis:
1. Complete eye exam: lids, pupils, EOMs
2. CT/MRI of the brain
3. CSF analysis
4. Blood tests: r/o infectious causes

27
Q

Discuss the pathology involved in the 4 hallmarks of Parinaud Syndrome.

A

Symptom 1: Upgaze palsy
- limitation of upward eye movements
- most common initial presenting symptom
- due to damage to the Vertical Gaze Centers or to the Posterior Commissure where fibers cross contralaterally
- downgaze is preserved due to bilateral innervation unless:
1. late stage disease
2. large SOL
3. bilateral lesion
- Setting-Sun Sign: preference for downgaze in primary position

Vertical Gaze Centers:
- in close proximity to the Sylvian aqueduct and unilaterally innervated thus prone to unilateral SOL and increased ICP
1. Rostral Interstitial Nucleus of the Medial Longitudinal Fasciculus (riMLF)
- vertical saccades
2. Interstitial Nucleus of Cajal (INC)
- all other vertical eye movements: vertical gaze holding and skew deviation

Symptom 2: Convergence-Retraction Nystagmus
- due to damage to the midbrain supranuclear fibers
+ exert inhibitory effect on the basal excitatory firing of the oculomotor nucleus
+ loss of inhibition leads to constant stimulation of the ff:
1. MR: convergence
2. SR, IR: globe retraction
3. LPS: lid retraction
+ irregular oscillatory or “nystagmoid” eye movement
+ more pronounced with upgaze

Symptom 3: Collier’s Sign
- bilateral lid retraction
- due to damage to the midbrain supranuclear fibers
- loss of inhibition of the EW nucleus causing constant excitatory innervation to the LPS

Symptom 4: Light-Near Dissociation
- bilateral light reflex impaired while near reflex intact
- due to damage to the light reflex fibers that pass through the Brachium of the Superior Colliculus to the Pretectal nucleus to the posterior commissure on its way to the EW nucleus
- near reflex fibers descend from the higher cortical centers more ventrally directly to the EW nucleus
- seen in > 2/3 of patients