Ocular Disease III - Diplopia

Ocular Disease III - Diplopia

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If your patient has an inferior oblique problem, what kind of diplopia is expected? How about superior oblique?

Vertical (both IO and SO)

Which muscle(s) is/are suspected with horz diplopia worse at far?

LR

Which muscle(s) is/are suspected with horz diplopia worse at near?

MR

Which muscle(s) is/are suspected with vert diplopia worse at far?

IR or SR

Which muscle(s) is/are suspected with vert diplopia worse at near?

IO or SO

What motor nerve is assoc w/ the iris sphincter?

CN III

What motor nerve is assoc w/ the levator?

CN III

What motor nerve is assoc w/ the orbicularis?

CN VII (facial)

What motor nerve is assoc w/ the dilator of the iris?

oculosympathetic branches

What motor nerve is assoc w/ Muller's muscle?

oculosympathetic branches

Your pt complains of monoc diplopia. What test would you use to R/O a cortical problem?

Pinhole test

Place in order of prevalence of palsies (most to least): CN III, IV, VI.

1. CN VI (abducens) = 3x
2. CN IV (trochlear) = 2x
3. CN III (oculomotor) = 1x
[4. Mixed = 1x]

Mnemonic = "ATOM"

What is the only CN nucleus with subnuclei?

CN III

Origin of CN III?

Oculomotor nuclei and Edinger Westphal nuclei

CN III superior branch innervates which muscles? Inferior branch? Parasympathetic fibers?

SR, levator = superior
MR, IR, IO = inferior
Iris sphincter, ciliary muscle = parasymp

T/F - 3rd nerve palsies involve a partial ptosis.

False - full ptosis

Your pt presents with full ptosis, mydriasis, and an eye that goes down and out underneath the ptosis. He also has eye pain. You suspect...

CN III palsy

3rd nerve palsies involve a (miotic/mydriatic) pupil. Why?

Mydriatic b/c CN III innervates sphincter of iris. If sphincter not working, then unable to close pupil, most noticeably in bright light.

Your left 3rd nerve palsy pt also has amblyopia OD. How will this patient present?

Since pt prefers fixation with OS, the left lid will be elevated and will raise OS, thus OD will have lid retraction and hyper.

T/F - 3rd nerve palsy does not involve ocular pain.

False - retro- or peri-orbital pain

Your pt has a 3rd nerve palsy, but you're having a hard time isolating CN IV fxn with motility testing. What can you do to check CN IV fxn?

Look at a vessel on the sclera and see if it rotates to check SO fxn (intorsion).

Your patient presents with a full ptosis OS with the eye below going down and out. Pupils appear normal, both in dark and light. You suspect...

CN III palsy, vascular etiology e.g. DM

List causes of CN III in adults, from most prevalent to least.

1. Undetermined
2. Ischemic-vascular
3. Aneurysm
4. Head trauma
5. Neoplasm

What areas in the brain are suspected to cause CN III palsies?

- Exit from brainstem
- Herniation against tentorial region
- Sup orbital fissure

List causes of CN III in kids, from most prevalent to least.

1. Congenital
2. Trauma
3. Inflammation
4. Neoplasm

Congenital CN III palsies are associated with what conditions (according to the slide)?

Amblyopia, aberrant regeneration

A central lesion at the nucleus of CN III will result in...

Bilateral ptosis (or no ptosis), contralateral SR palsy; ipsilateral III palsy; can also have isolated muscle paresis

A central caudal CN III subnucleus lesion will affect...

both levators (bilat)

A lesion at the lateral CN III subnuclei will affect...

Ipsilateral MR, IR, IO

A lesion at the medial CN III subnuclei will affect...

contralateral SR

Causes of CN III palsy originating from the nucleus?

- Infarction e.g. thrombus, emboli (most common)
- Demyelination e.g. MS
- Tumor, neoplasm
- Thiamine Deficiency (Wernicke's)

T/F - A nuclear CN III palsy can involve a unilateral dilated pupil.

False - E-W subnuclei are fused so both sides are affected

T/F - A nuclear CN III palsy can involve a unilateral ptosis.

False - Central caudal subnucleus responsible for both levators

T/F - A nuclear CN III palsy can involve an isolated IR palsy?

True

T/F - A nuclear CN III palsy can involve an isolated MR palsy?

True

T/F - A nuclear CN III palsy can involve a bilateral III palsy without ptosis.

True

T/F - A nuclear CN III palsy can involve an isolated SR palsy?

False

A nuclear CN III palsy can involve OD with a normal OS.

False (b/c central caudal nucleus controls both levators?)

What are the syndromes involved with the CN III fascicle lesions?

Benedikt's, Weber's, Nothangel's, and Claude's

Benedikt's involves damage to what structures and what Sx?

Ipsilateral CN III paresis + ipsilateral cerebellar ataxia (damaged red nucleus and CN III fascicle)

Weber's involves damage to what structures and what Sx?

Ipsilateral CN III paresis, contralateral hemiparesis, possible upper VII motor neuron involvement (damaged corticospinal tract and CN III fascicle)

Your pt presents with the whole right side of the body paralyzed and a ptosis OS with the eye under the lid down and out. You suspect...

Weber's; ipsilateral CN III paresis + contralateral hemiparesis, due to damaged CN III fascicle + corticospinal tract

Nothangel's involves damage to what structures and what Sx?

Ipsilateral CN III paresis, contralateral hemitremor (damaged CN III fascicle + brachium conjunctivum)

Claude's involves damage to what structures and what Sx?

Ipsilateral CN III paresis, ipsilateral cerebellar ataxia, contralateral hemitremor (damaged CN III fascicle, red nucleus, and brachium conjunctivum)

Basically Benedict's + Nothangel's

Your pt presents with a full ptosis + down and out OS, uncoordinated limb movements of the left side, and tremors on the right side. You suspect...

Claude's

Ipsilateral CN III paresis, ipsilateral cerebellar ataxia, contralateral hemitremor (damaged CN III fascicle, red nucleus, and brachium conjunctivum)

T/F - Thiamine deficiency can cause CN III fascicle damage.

False - causes nuclear CN III damage

T/F - Demyelination can cause CN III fascicle damage

True

Sx of uncal herniation? Why?

Pupil enlarges and unreactive to light (= Hutchinson's pupil), pt becomes comatose

Due to CN III palsy caused by supratentorial mass pushing uncus of temporal lobe against CN III

What are the causes of CN III problems in the Sub-arachnoid space?

Posterior communicating artery (PCA) aneurysm, meningitis, tumors, trauma

You must R/O ____ if you see a full ptosis with a dilated pupil because it may be fatal.

PCA aneurysm

A PCA aneurysm can cause a (dilated/miotic) pupil. Why?

Pushes dorsomedial aspect of CN III, where pupillary fibers lie.

In a PCA aneurysm, the pupil takes up to ___ days to become dilated.

3-5

Most common cause of isolated, non-traumatic CN III palsy with pupil involvement...

PCA aneurysm

T/F - PCA aneurysms present w/ pain.

True - almost always (retro-orbital or peri-orbital)

T/F - PCA aneurysms rarely have isolated pupil involvement.

True

PCA aneurysms involve EOMs within ___ to ___ (what time frame?).

hours to days

T/F - Cavernous sinus dz can involve proptosis.

True

Your pt has a full ptosis OS with the eye under the lid down and out, pupils normal. You also see engorged, corkscrew-like conjunctival vessels and proptosis. You suspect ____. Why?

Cavernous sinus dz causing CN III palsy

Causes of orbital-related CN III palsies?

Tumor, inflammation, trauma

Your pt has a Hx of assault (he was punched in the left eye), and has pain, proptosis, and conj chemosis. On EOM testing, all but the the MR, IO, and IR work fine. You suspect...

Selective trauma to inferior branch of CN III.

EOM fibers are in the (outer/inner) part of CN III.

inner

T/F - The pupil is typically not spared in ischemic-vascular CN III palsies.

False - typically spared (62-83%)

T/F - Pupil-sparing CN III palsies can involve pain.

True

Pupil sparing CN III palsies resolve after ___ months (with/without) aberrant regeneration.

3-4, without

T/F - Pupil sparing CN III palsies typically resolve on their own.

True

Describe the mechanism of vasculoplastic palsy.

Thickening/hyalinization of nutrient vessels or decr perfusion at vascular border of nerve, ischemic demyelinization of segment of nerve, eventual remyelinization and recovery

T/F - Vasculopathic CN III palsies that spare the pupil show diffuse pattern of EOM paresis.

True

T/F - Aneurysm-related CN III palsies that spare the pupil show diffuse pattern EOM paresis.

False - show focal (superior branch) pattern of EOM paresis

Pupil fibers are in the inferior branch

T/F - Ophthalmoplegic migraines first appear in adults 20-30 yrs old.

False - children under 10 yrs old

Your 8-yo pt presents w/ nausea, periorbital pain, OS down and out with full ptosis, and severe headache on the left side of his head. Pupils are normal. You suspect...

Ophthalmoplegic migraine

Describe the progression of recovery in ophthalmoplegic migraine.

ptosis first, then pupils, then EOMs

CN palsies of ophthalmoplegic migraine - list from most to least frequent CNs affected.

CN III, VI, IV

"OAT" - Oculomotor, Abducens, Trochlear

T/F - Most cases of Ophthalmoplegic migraine spare the pupil.

True - 2/3rds of cases, suggests vascular etiology (int carotid ischemia?)

T/F - Ophthalmoplegic migraine does not respond to steroids.

False - Ophthalmoplegic migraines do respond to steroids

T/F - Aberrant regeneration is possible in ischemic-vascular related events.

False - aberrant regeneration never in ischemic-vascular events

Etiologies of aberrant CN III regeneration?

- Acute CN III palsy (trauma, aneurysm, tumor)
- Congenital III nerve palsy

Possible mechanisms of aberrant regeneration?

1. Misdirection of sprouting axons from one structure to another
2. Loss of insulation of adj branches
3. Reorganization of CN III nucleus

Describe Pseudo Von Graefe's sign. This is caused by...

Pt looks down, lid goes up; caused by aberrant regeneration of IR fibers going to LPS

Describe Lid synkinesia. This is caused by...

Eye adducts, lid shoots up. When eye goes out, lid goes down. Caused by aberrant regeneration of MR branch redirecting to superior branch

T/F - Tonic pupil is a sign of aberrant regeneration.

True

T/F - Monocular horiz OKN is a sign of aberrant regeneration.

False - vert

Aberrant regeneration with no prior Hx of CN III palsy, caused by slow compression of CN III is called...

primary aberrant regeneration

Aberrant regeneration following acute CN III palsy or congenital CN III palsy is called...

secondary aberrant regeneration

In adults, if you see a non-traumatic, isolated CN III palsy, what is the management?

R/O aneurysm, refer MRI/angiogram STAT (gadolinium contrast, CT or MR angiography)

In adults, if you see a pupil-sparing CN III palsy, what is the management?

Ischemic dz workup: BP, CBC, ESR, oral glucose tolerance test, VDRL/FTA-ABS (syphillis), ANA; STAT CT and/or MRI

Your 45 yo pt has a pupil-sparing, complete CN III palsy and a Hx of long-term DM, what is the management?

- Watch pupil daily for 1 wk to see if it becomes involved (if involvement, refer for MRI)
- Watch monthly for improvement (if not impr after 91 days or aberrant regen, send for arteriogram)

Management of aberrant regen with no Hx of head trauma?

MRI

Management of isolated, acquired, non-traumatic CN III palsy in a kid?

Order MRI thru neurologist, ped ophthalmologist, or pediatrician

With CN III palsy with pain, you should suspect...

aneurysm

CN III palsy in kid, R/O...

aneurysm

CN III palsy with pupil sparing in older pts, think...

vascular dz

CN III palsy with pupil sparing, think...

ischemic-vascular

T/F - Most aneurysms affecting CN III involve the pupil.

True (86-97%)

T/F - Most aneurysms affecting CN III involve pain.

True - almost always

T/F - Most ischemic-vascular problems affecting CN III can involve pain.

True

Your pt has a hyper OD and complains of words going double vertically while reading, and sometimes finds himself reading into the next line of print. You suspect...

CN IV palsy

T/F - CN IV pts tend to hold their reading material down and out to reduce Sx.

True

You expect left SO palsy pts to tilt their head...

To the right

SOTO = Sup Oblique Tilt Opposite

CN IV palsy pts have the inability to (extort/intort).

intort (UA SO)

remember "inferior people extort"

CN IV palsy pts have an overacting ___ muscle.

IO (ipsilateral)

Why is an RSO palsy worse on right head tilt?

The primary depressor on right head tilt is the SO, but since the SO is underacting, will see a more hyper OD (see slide of smiley face)

How would bilat CN IV palsy present if you had them gaze to either side or tilt on either side?

Rhyper in left gaze and right head tilt (R-L-R)

Lhyper in right gaze and left head tilt (L-R-L)

Upon EOM testing you determine your pt has a CN III palsy. What are your Park's 3-step findings?

Trick question - Park's works only if one EOM is involved.

On Park's 3-step, you find a right hyper, worse on left gaze and right head tilt. You suspect...

RSO palsy (RLR - think of the first letter as the side affected, hence LRL would be LSO palsy)

In your CN IV palsy pt, you get 6 left hyper, thus vertical fusional amps are expected to be...
A) 10 BU, 15 BD OD
B) 10 BU, 10 BD OS
C) 15 BU, 10 BD OS
D) 10 BU, 15 BD OS

D) 10 BU, 15 BD OS

On the bilateral maddox rod test, you place (BU/BD) prism over the (paretic/nonparetic) eye.

BD, nonparetic

On the bilateral maddox rod test, if the patient sees the line from his RE tilted to the left, you expect his RE to be (extorted/intorted).

extorted (line is intorted)

Etiology of CN IV palsies and their percentages?

Neoplasm/aneurysm (10%)
Ischemic (20%)
Undetermined (30%)
Trauma (40%)

Now I Understand Trochlear palsies

Causes of CN IV palsies in children, from most to least prevalent?

Congenital (67%)
Trauma (28%)

Name the 4 syndromes assoc w/ CN IV.

Nuclear-fascicular
Sub-arachnoid space
Cavernous sinus
Orbit

T/F - Nuclear-fascicular CN IV (Midbrain) lesions usually unilateral.

False - asymmetric, bilat

On your pt you see a hyper LE with a head tilt to the right, and a partial ptosis with miosis on the RE. You also notice that the patient's skin is flushed on the right side. You suspect ____. Why?

Lesion at right brainstem affecting right CN IV nucleus (thus UA LSO) and right sympathetic branches (thus Horner's).

Remember SO is controlled by contralateral CN IV nucleus - right CN IV nucleus innervates left SO.

T/F - A CN IV nuclear-fascicular lesion at the brainstem would affect L-N dissociation.

True

Where do the CN IV fibers decussate?

Anterior medullary vellum, on dorsal surface of brainstem

In sub-arachnoid space, a head injury can result in compression of CN IV where?

at tentorial edge

How can disc edema occur with CN IV palsies?

Incr intracranial pressure at subarachnoid space

A pinealoma in the sub-arachnoid space can cause this CN palsy.

CN IV

CN III palsies are typically (severe/moderate/mild) head trauma, while CN IV palsies are typically (severe/moderate/mild) trauma.

severe = CN III
moderate = CN IV

Contusion of the (dorsal/ventral) midbrain tend to cause CN IV palsy.

dorsal (remember how CN IV exits dorsal midbrain and wraps around to the front)

What is the problem with assessing orbital CN IV palsies?

Hard to assess whether it is really a nerve cause vs tendon, trochlea, or muscle.

Why is CN IV susceptible to injury?

long intracranial course

T/F - Sphenoid bone fractures is a possible cause of CN IV palsy.

True

Peak incidence of CN IV palsies (decades)?

4th-5th decade

T/F - CN IV palsy can be associated w/ vascular dz.

True - HTN, DM, arteriosclerosis, temporal arteritis

T/F - Congenital CN IV palsy tends to be bilat.

False - unilat

T/F - 10 degrees or more on the double maddox rod test indicates bilat involvement.

True

You see an isolated non-traumatic CN IV palsy with large vert amps, confirmed w/ old photos. Dx? Mx?

Congenital CN IV palsy, Mx = no further eval needed

Mx of 25-yo non-traumatic CN IV palsy with normal vertical amps?

R/O ocular myasthenia, send for MRI

Do this for 20-40 yo

Mx of 60-yo non-traumatic CN IV palsy?

Ischemic-vascular workup

Do this for 40-65 yo

Your pt complains of horz double vision when looking at street signs and a right eye that turns in. You notice his head is turned to the right. Dx?

R CN VI palsy

In your right CN VI palsy pt, you expect the (exo/eso) to get worse when he looks to the (right/left).

eso, right

How do you DDx ocular myasthenia gravis vs CN VI palsy?

myasthenia is transient, worse when tired

By looking at the eyes, how you do DDx thyroid eye dz vs CN VI palsy?

thyroid eye dz = see lid retraction

How do you DDx Mobius syndrome vs CN VI palsy?

Mobius = also see CN VII affected thus see reduced facial tone

Etiologies of CN VI palsies in adults?

Undetermined (21-32%)
Misc (20-30%) - migraine, pseudotumor, MS
Ischemic (9-37%)
Trauma (7-17%)
Neoplasm (7-31%)

Most likely cause of CN VI palsies in young adult/middle age?

Mass lesions

Causes and prevalences of CN VI palsy in children?

Neoplasm 40%
Misc 30%
Trauma 20%
Undetermined 10%

Where is CN VI most vulnerable?

Brainstem (Pons), fascicle, sub-arachnoid space, petrous apex (temporal bone), cavernous sinus, orbit

What nuclei is CN VI close to (at the level of the pons)?

V, VII, VIII

T/F - CN VI is close to the cerebellum.

True

Why is it possible to have both a CN VI and VII palsy in a central lesion at the level of the pons?

VII fibers loop around VI nucleus

Lesion at MLF at the level of the lower pons will cause...

Ipsilateral INO

Lesion at the PPRF at the level of the lower pons will cause...

Ipsilateral horz gaze paresis

Lesion at the oculo-sympathetic nucleus at the level of the lower pons will cause...

ipsilateral horner's

Lesion at the pyramidal tract at the level of the lower pons will cause...

contralateral hemiparesis

Pontine glioma can cause progressive damage of these CNs...

V, VI, VII, VIII

Remember these are CNs within the Pons!

Your pt complains of diplopia at distance, RE turned in, and hemiplegia of the left side. Dx? Why?

Raymond's. Lesion at ventral pons affecting VI fibers + corticospinal tract leading to VI palsy & contralat hemiplegia

Describe Millard-Gubler's syndrome.

Fascicular syndrome at ventral pons causing Raymond's (VI palsy + contralat hemiplegia) and VII palsy

Name the fascicular syndromes of CN VI.

Raymond's, Millard-Gubler, Foville's

Describe Foville's

Anterior inferior cerebellar artery infarct affecting pontine tegmentum (CN VI fascicles + olivary nucleus)

T/F - CN VI palsy involves the lowest degree of head trauma vs III & IV.

True

How does head trauma cause a CN VI palsy?

1) Fracture of petrous bone - lateral
2) Hyperextension of neck - stretch brainstem

How can the sub-arachnoid space be involved in a CN VI palsy?

Incr intracranial pressure causes stretching of CN VI with downward displacement of brainstem. Causes bilat disc edema, incr esodeviation, nausea, HA.

Inflammation of the meninges and tip of the petrous bone describes...

Gradenigo's

Gradenigo's can cause these CN palsies...

V, VI, VII, VIII

Note these are all Pons-level CNs

CN V, VI, VII, and/or VIII palsies after an ear infection in a child makes you think...

Gradenigo's

T/F - Nasopharyngeal carcinomas are more common in caucasians.

False - Asians

Nasopharyngeal carcinomas present with...

decr tearing (V nerve), facial pain, can block eustachian tube and cause "clogged" ear

Cerebellopontine angle tumors involve these CN palsies and what Sx? Locaton?

CN V, VI, VII, VIII (these are all Pons CNs); ataxia, papilledema

At petrous apex

For CN __ palsies, you have to consider Pontine Gliomas, Gradenigo's, and Post-viral infections in children.

VI

T/F - Consider Gradenigo's for CN VI palsies age 15-35.

False - consider MS in that age range

Gradenigo's - considered in children

T/F - Nasopharyngeal carcinomas, meningiomas are considered in CN VI palsies age 35-55.

True

T/F - In pts over 55, consider ischemic vascular, giant cell arteritis as causes of CN VI palsy.

True

You have an 8yo pt coming in with non-traumatic CN VI palsy - Mx?

Aggressive management to R/O glioma. Also R/O post-viral infection. Monitor other cases q2wks for improvement. If neuro signs or no improvement, MRI.

Neuroimaging is indicated for non-traumatic VI if...

- accompanying neuro signs
- severe pain
- Hx cancer
- Palsy worsens or does not resolve in 3-6 mos
- Papilledema

Mx of non-traumatic VI if pt has papilledema?

Neuroimaging

You've followed your non-traumatic CN VI palsy pt for the past month, but the palsy is still present. Mx?

Continue to f/u; if does not resolve in 2-6 mos (total 3-6 mos) then neuroimaging

How often to f/u for vascular-ischemic related CN VI palsies?

every 6 weeks

T/F - Meningeal dz or increased intracranial pressure will most likely cause bilat VI paresis.

True

It is most important to R/O this cause of CN VI in kids...

glioma

It is most important to R/O these causes of CN VI in older pts...

ischemic-vascular dz or carcinoma

What important sign in the eye should you watch for in CN VI palsies?

disc edema

What is Hutchinson's pupil?

Pupil enlarges and unreactive to light, seen in Uncal Herniation.

Ophthalmoplegic migraines respond well to this drug.

Steroids


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