Ocular Disease III - Conj gaze + Misc

Ocular Disease III - Conj gaze + Misc

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Cause of INO?

Dz involving connection b/w III and IV nuclei, specifically the MLF

Most common etiology of unilat INO? Other causes?

Ischemic-vascular (most common), neoplasm, trauma

Etiology of bilat INO?

MS (most common in younger pts)

INO is seen mostly in (kids/adults/older), (males/females), and is (chronic/acute).

older, males, acute

DDx of INO vs thyroid dz?

Proptosis in thyroid dz

DDx of orbit dz vs INO?

proptosis in orbit dz

In your pt you see an inability to adduct the RE with a nystagmus during abduction of the LE. Convergence is intact. Dx? Where is the lesion?

Right Posterior INO affecting right MR, at the Pons

"MLF" = "MR Looks Funny"

Anterior INO = convergence is lost

T/F - RINO = RMLF lesion = RMR dysfxn.

True

The CN VI nucleus sends signals to what two muscles for conjugate gaze?

Ipsilateral LR (direct), contralateral MR (MLF connects CN VI nucleus to CN III nucleus)

What causes the nystagmus during INO?

Incr innervation to weak MR results in excessive activity to contralateral LR.

Besides nystagmus and MR dysfxn, what other signs seen in INO?

- Decr saccadic response of affected eye (glissade)
- Decr OKN response (adduction saccade - MR)

Anterior INO is located at what level of the brainstem?

midbrain

Post INO located at what level of brainstem?

Pons = Posterior

Anterior INOs are localized...

between MLF and CN III nucleus

Posterior INOs are localized...

between MLF and CN VI nucleus

Anterior INO is (infra/supra)nuclear?

infra

Posterior INO is (infra/supra)nuclear.

supra

Most INOs are (post/ant).

post (2/3)

ant = 1/3

T/F - Bilat INOs tend to be acute.

False - progressive

T/F - Bilat INOs in younger pts tend to be assoc w/ MS.

True

T/F - If a Bilat INO is complete on both sides, it is most likely MS.

False - Non-MS bilat INOs tend to be complete.

You see a pt with poor adduction in both right and left gaze with no abduction nystagmus. Dx?

Bilat INO

What is WEBINO mostly assoc with?

MS

You see a bilat INO with exotropia. You suspect...

WEBINO

Called "Wall-eyed" b/c as if pt is looking at both walls at each side.

Mortality of unilat INO 18 mos from Dx = __%

42

How do you Tx a pt with INO due to DM?

Tx INO due to vascular etiology with prism; see improvement in 90 days.

A lesion that encompasses the MLF and PPRF on the same side at the level of the pons describes...

One and a half syndrome

Etiology of 1.5 syndrome?

infarction, MS

Your pt on forward gaze has an exo on the RE. On right gaze the RE can abduct but LE appears straight. On left gaze both eyes appear straight. Dx?

left 1.5 syndrome

Think of it this way - eye that appears straight in forward or R/L gaze is the side of the lesion

You see L-N dissoc of the pupils, inability to gaze upward with assoc nystagmus, and lid retraction on downgaze. Dx?

DMS

What specific area of the midbrain is affected in DMS?

Posterior commissure

T/F - It is possible to see a unilateral upward gaze dysfxn in DMS.

False - post commissure lesion affects both eyes for upgaze

Where is the upward gaze center? How does it relate to DMS?

RiMLF. The RiMLF from both sides pass thru the post commissure, which is damaged in DMS

Etiologies of DMS?

Pinealoma, MS, infarction, syphyllis, AV malformations

What conjugate gaze syndrome involves Collier's sign?

DMS - lid retraction on downgaze

Besides L-N dissoc, upward gaze paralysis, and Collier's sign, what other signs of ___ can be present?

DMS; convergence spasm, decr downward saccades, papilledema, skew deviation

(horz/vert) divergence from acquired (supra/infra)nuclear or _____ disruption describes [this conjugate gaze syndrome].

vert, supra, vestibulo-ocular, skew deviation

Your patient's eyes appear to vertically diverge (and he also says he sees vertical diplopia), and you cannot isolate a single EOM that is causing it. You suspect...

Skew deviation

Skew deviation suggests ____ or ____ disease.

brainstem, cerebellar

T/F - Skew deviation typically presents with other neurologic dysfunction(s)

True

T/F - Skew deviations are mostly noncomitant.

False - 90% comitant

Your patient can change her hyper from left to right on different gaze positions. You suspect...

noncomitant skew deviation

*What is the most common cause of spontaneous diplopia in middle-age and early senescent pts?

Graves'

Eye involvement in Graves' dz can occur in pts with...

- Hyperthyroidism
- Secondary hyperthyroidism from Tx of hypothyroidism
- Euthyroid dz (normal T3, T4, TSH with incr thyroid antibodies)

Pathophysiology of ocular involvement of Graves'?

EOMs infiltrated with lymphocytic and plasmacytic material which incr volume of muscle; EOMs become fibrotic and shortened, thus pulls eye in corresponding direction of that muscle

EOM involvement prevalence in Graves'?

IR = 60-70%
MR = 25%
SR = 10%

"IMS" = "I MuscleS"

Your Graves' dz pt shows up with a right hypo (and restricted upgaze). You suspect what muscle is involved?

IR since Graves' shortens the muscle thus pulls the eye down.

T/F - Graves' dz with ocular involvement is assoc w/ IOP increase in restricted field of gaze.

True - 6mmHg or more

Pathophysiology of ocular myasthenia?

Acquired autoimmunity; destroyed ACh receptors at NMJ (70-89% decr in receptors), thus weakness of with repetitive/sustained activity

Avg lifespan of ACh in normals? In myasthenia?

7-11 days normal
1 day in myasthenia

T/F - Ocular Myasthenia is assoc w/ thymus hypoplasia.

False - hyper

What autoimmune dz mentioned is related to thymus problems?

ocular myasthenia (thymus hyperplasia, thymoma, dysthyroid dz)

Thymoma in myasthenia is more likely in younger, middle, or older aged pts?

Older

T/F - About half of myasthenia pts will show eye signs initially.

True (40-50%)

T/F - Most ocular myasthenia pts will develop generalized dz within 2-3 yrs.

True (50-94%)

What are the most common symptoms of ocular myasthenia? Most commonly affected muscle?

Ptosis and diplopia (90%)
MR most commonly affected

T/F - There is often pupil involvement in ocular myasthenia.

False - little or none

T/F - MG onsets in women earlier than men

True

Why did Dr. S have a picture of Hugh Hefner and some playboy bunnies???

MG occurs in young women (under 40) and older men (over 40)!


Dx? Side of lesion?

R INO

What is the most common CN palsy?

VI (Abducens)

Remember "ATOM" mnemonic - Abducens, Trochlear, Oculomotor, Mixed

What is the Tensilon test used for?

For DDx MG vs neural cause of palsy - if the pt is able to recover after tensilon, then reason is MG

What is Peek Sign?

incomplete eye closure assoc w/ MG

What is Cogan's Lid Twitch Sign?

For MG - have pt look down then up. Lid twitch on upgaze is positive

What is the icepack test? What is its sensitivity?

For MG pts - place ice on eye for 2 mins; ptosis should disappear. Works by decr activity of cholinesterase in NMJ. 90% sensitivity

How do you admin the Tensilon test?

Inject edrophonium chloride into the arm or hand vein of the MG pt; re-eval deviation 3-4 mins post-injection.

T/F - Tensilon test works better for ptosis vs ophthalmoparesis.

True

Sensitivity of Tensilon test? Specificity?

Sens = 86%
Spec = 80%

Sensitivity of Acetylcholine Receptor Antibody Titer? Specificity?

Sens = 64%
Spec = 99%

What is a ptosis crutch?

For ocular myasthenia pts - holds up ptosis with wire behind glasses

What drug mentioned is used to Tx ocular myasthenia? Dose? How does it work?

Pyridostigmine (Mestinon) 60mg TID - an anticholinesterase agent

Mestinon has a __ min onset, duration __ hrs.

30 min, 4 hrs

T/F - Mestinon works better for diplopia vs ptosis.

False - just like tensilon, better for ptosis

ADEs of Mestinon?

GI upset, colitis

Your pt's eyes looks like they "freeze" during EOM testing - there is limited motility in all directions. There is also bilateral ptosis, but the pupils are normal. Dx?

CPEO

T/F - CPEO is worse later in the day.

False - no diurnal variation (vs ocular myasthenia)

T/F - CPEO can be associated with limb and/or facial muscle weakness.

True

T/F - Kearns-Sayre syndrome onsets after age 20.

False - before 20 yrs old

What is the Kearns-Sayre syndrome triad? What other signs?

Triad =Pigmentary retinopathy, Progressive ophthalmoplegia, Heart block

Also incr CSF protein, short stature, delayed sexual maturity

T/F - CPEO is progressive and asymmetrical.

False - it is progressive but symmetrical

Tx Kearns-Sayre syndrome?

No known Tx, just Tx exposure keratopathy

Initial Sx of PSP?

Loss of balance, falls

What disease involves supranuclear ophthalmoplegia with nuchal rigidity?

PSP - also has loss of balance, behavioral changes

Areas of brain affected in PSP?

Basal ganglia, brainstem, frontal lobe, cerebellum, spinal cord

Eye signs of PSP?

Loss of vertical sacc, vert gaze, horz eye movement, bell's phenomenon; square wave jerks

T/F - PSP involves loss of vertical saccades

True

Your patient has no vertical saccades, no vertical gaze, and an inability to do horz eye movements. You also see square wave jerks and no Bell's phenomenon. Dx?

PSP

DMS is aka...

Parinaud's

A pinealoma can cause this conjugate gaze syndrome

DMS (Parinaud's)

T/F - 1.5 syndrome can be caused by neoplasm.

False (the slides did not mention neoplasms as a cause, just infarctions, MS)


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