Neuro Eye Disease Grand Rounds - afos2020.org

Neuro Eye Disease Grand Rounds

Eric E. Schmidt, OD, FAAOOmni Eye SpecialistsWilmington, NC

The Neuro Eye Exam: History

l Most important part of the examl Vital statisticsl Chief complaint – Clarify, Qualify, Quantifyl Medical historyl Social historyl Medications

The Neuro Eye Exam: Examination“The hunt begins!”

l Stop, look and listenl Visual acuity – Remember the pinhole!l Amsler gridl Red cap testl Color visionl Pupils, pupils, pupils

Examination – “The hunt continues”

l EOM testing– Normal– Forced duction

l Confrontation fieldsl Facial AGl Refractionl Anterior segmentl Optic nerve head evaluationl Retinal Other tests- CT/MRI, lab tests, perimetry

The Neuro Eye Exam

l Diagnosis – “The 90% Solution”

l Management – “Making The Arrest”

The Case Of The Chubby Disk

l 29 y/o WF cc: blurred vision OS, OS seems to be “pulling” for last 3 wks– HA on L side– Feels like pressure (a suction cup) on OS– Seems like a skim over OS

Meds: nonePast hx of “migraines”

Chubby exam

l VA – OD 20/25, OS 20/20 (blurry), poor endpoint on refraction

l PERRL mg (-)l EOM – no restrictionl SLE – wnl OUl IOP- 20mm OD, OSl Fundus – as shown

Chubby disk, Question 1: What is the most correct diagnosis?

l 1. Papilledemal 2. Bilateral disk edemal 3. Pseudotumor cerebril 4. Brain tumorl 5. Optic disk drusen

Disc edema – differential diagnosis

l Intracranial tumorl Elevated ICP – Papilledema, PTCl Vascular/Ischemic – IONl Inflammatory – Optic neuritisl Systemicl Orbital tumors

Papilledema

l Bilateral disk edemal NFL opacification and hemesl Hazy retinal vesselsl Paton’s linesl (-) SVPl Disk hyperemia, exudates and CWS

l Papilledema is always caused by increased ICP!!

Chubby disk, question 2: What is your next move?

l 1. CT Scanl 2. ESR/C-Reactive proteinl 3. Lumbar puncturel 4. MRIl 5. Refer to Jim Thimons!!l 6. Visual field

Given the VF result would you next?

l 1. CT Scanl 2. ESR/ C-Reactive proteinl 3. Lumbar puncturel 4. MRIl 5. Refer to Oh Great One!!!

Chubby disk, question 4:

l MRI showed no mass lesion, but partial empty sella – What are you going to do now?– 1. ESR/C-Reactive protein– 2. Neurology referral– 3. Neuro-eye referral– 4. Lumbar puncture– 5. Prednisone 80mg daily– 6. Diamox 500mg po BID

The Hunt For The Cause

l MRI – Partial empty sellal LP – Opening ICP 402

Chubby question 5: Now what is the diagnosis?

l 1. Meningitisl 2. Benign Idiopathic Intracranial

Hypertensionl 3. Pseudotumor cerebril 4. Viral encephalitis

Chubby question 6: How are you going to treat this?

l Acetazolamide 250mg BIDl Acetazolamide 500mg sequels QDl Prednisone 60mg QDl Serial Lumbar puncturesl Topamax 60mg QDl Optic nerve sheath defenestration

Idiopathic Intracranial Hypertension

l Bilateral disk edema (papilledema)l Due to increased CSFl Tx: Diamox

– Weight loss– Oral steroids– Weight loss– Topamax– Repeat LP– Cerebral shunt– ON defenestration

l WEIGHT LOSS!!, WEIGHT LOSS!!, WEIGHT LOSS!l Lose Weight !*&@*(^^(@!

Chubby’s sequelae

The Case of 2 Eggs, 4 Pancakes and 8 Strips of Bacon!

l 70 y/o WM sat down for breakfast when he suddenly experienced horizontal double vision

l Felt very “woozy.” “like I’ve lost my depth perception.”

l Denies paresthesia or weaknessl Meds - ASA

Examination

l VA OD 20/30 ph NI, OS 20/30 ph NIl EOM – OD no restriction

OS no adductionCT – 50pd LXT (OS down and out)

10 pd RH

PERRL mg(-)

Examination

l Slit – wnl (-)RIl Fundus – D,M,V,P wnl OU

– (-) HR,DR,disc swellingl BP – 150/70l (-) Bruitl Neurologic survey – neg other than EOM

palsyl ?Diagnosis?

2 Breakfasts. Question 1:

l What is his most accurate diagnosis?– 1. Complete CN3 palsy– 2. Partial CN 3 paresis– 3. Diabetic neuropathy– 4. CN4 palsy– 5. CN6 paresis – 6. Left adduction deficit

Some Diplopia Rules

l Crossed diplopia – exo deviation (MR)l Uncrossed diplopia – eso deviation (LR)l Worse at distance – lateral musclesl Worse at distance – rectus musclel Worse at near – medial musclel Worse at near – oblique muscle

The 4 Questions of Diplopia

l 1). Monocular or binocular?

l 2. Horizontal or vertical?

l 3.Is it worse in any direction of gaze?

l 4.Diplopia greater at distance or near?

l Identifies CN 3,4, or 6

The Rule Of The Pupil

l In all cases of diplopia or ptosis – check the pupil!!!!

– Pupil spared – diabetes– Pupil blown – aneurysm– Pupil miotic - inflammatory

2 Breakfasts, question 2;

l What is the most likely etiology?– 1. Aneurysm– 2. Diabetes– 3. CVA– 4. Intracranial Tumor– 5. Trauma

2 Breakfasts, question 3:

l What should you do next?– 1. Carotid ultrasound– 2. Blood work (CBC, FBS, ESR)– 3. MRA– 4. MRI– 5. CT scan– 6. VF– 7. ER referral

CN 3 Neuropathy

l Horizontal diplopia

l Worsens on contralateral gaze

l Check the lids and pupils

l Can there possibly be a vertical component?

CN 3 Neuropathy Causes

l Adults– 20% Aneurysm– 20% Vascular– 15% Trauma– 45% Other

l Children– 45% Congenital– 20% Traumatic– 10% Neoplasm– 7% Aneurysm

CN 3 Management

l <40 y/o– CT Scan– Angiogram– MRA

–

l Remember the rule of the pupil

l >40 y/o– BP, CBC, FBS– RTO daily x 1 week– RTO weekly x 1 mth– RTO monthly x 3 mths

l CT scan if worsening or no improvement

HOWEVER!!!!

CN 4 Neuropathy

l Vertical diplopia

l Worsens upon contralateral gaze

l Diplopia worse at near

l Head tilt likely

CN 4 Neuropathy Causes

l 40% Trauma

l 20% Vascular

l 10% Neoplasm

l 10% Aneurysm

l 20% Unknown

CN 6 Neuropathy

l Horizontal Diplopia

l Diplopia worsens on ipsilateral gaze

l Diplopia worse at distance

l Most common ocular palsy

CN 6 Neuropathy Causes

l Vascular (esp if unilateral)l Neoplasml MSl Subarachnoid hemorrhagel Meningitisl Traumal 30% Idiopathicl Bilateral 6th is never from infarction

CN 6 Neuropathy Management

l Adults– Blood work– CT scan if progressive– Evaluate for increased ICP– Pain as a prognostic sign

l Children – 33% tumors– All kids with acute 6th need MRI

2 Breakfasts - resolution

l BP – normal

l BS – 117

l CBC – normal ESR – 27mm/hr

l CT scan – massive sinusitis

So Tell Me Oh Great One, How Did Your Patient Fare?

l CT Scan – Massive sinusitisl Oral Antibiotics x 3 weeksl Refused to cook anymore!

Singin’ The Blues

l 84 y/o WF referred in for “papilledema”l She had no complaints, no ocular symptoms

and had not noticed any change in visionl CVA 3 yrs priorl Meds: Celebrex, Tylenol

Vida’s Exam

l VA – OD LP, OS -20/200 ph 20/80l Pupils – OD 6mm, oval, sluggish (+)APD

OS 4mm sluggish responsel SLE OD 1+ stromal haze, PCL

OS PCL, limbal pannus, 3+ inf endo pigment

l Fundus – as shown

Which is the acutely involved eye?

l 1. Right

l 2. Left

l 3. Both

What’s Vida’s Diagnosis?

l 1.Optic atrophy OD, papilledema OSl 2.Papilledemal 3.Optic atrophy OD, ION OSl 4.Optic atrophy OD, Optic neuritis OSl 5. CRVO OSl 6.Foster-Kennedy Sxl 7. Brain tumor

What is the most appropriate next step?

l 1.Disk photos, recheck 1 weekl 2. MRIl 3. FBSl 4. Intravenous Fluorescein angiographyl 5. TA Biopsyl 6. ESR/C-RPl 7. Prednisone 80mg po

ESR = 89mm/hr , C-RP elevated Now What?

l 1. TA biopsyl 2. Medrol dose pakl 3. Refer to internistl 4. Prednisone 80mgl 5. Refer to neurologistl 6. MRIl 7. IV methylprednisolonel 8. LP

Now what is the diagnosis?

l 1. Foster-Kennedy Syndromel 2. Old ION OD, Acute ION OSl 3. Old ION OD, CRVO OSl 4. Giant cell arteritisl 5. Optic nerve hypoplasia OD, ION OS

Headache awareness day

l 47 y/oWF l CC: Episodic visual fluctuation

Severe HA on top of head x 4 mthsNumbness on L side of face

l Saw neurologist 3 mths prior– Normal CT– Dx: Migraine syndrome– Symptoms no better since migraine tx

My Exam

l VA OD 20/25 OS 20/25l Improved to 20/20 with more (+)l SLE: Normall IOP - 14 OD, 16 OSl Disks – as shown

What Are You Going To Do Next?

l 1. Repeat CT scanl 2. Refer to (a different) neurologistl 3. MRIl 4. Fundus photos and follow-up 1 monthl 5. OCTl 6. VF

Name That Visual Field!

l 1. Bitemporal hemianopsial 2. Left homonymous hemianopsial 3. Right homonymous hemianopsial 4. Double arcuate scotoma OUl 5. Nasal step

This patient’s VF

l Left homonymous hemianopsia, denser above than below

l What is the most likely etiology?l What do you do now?

Where is the lesion most likely located?

l 1. Left optic nervel 2. Optic chiasml 3. Parietal lobel 4. Temporal lobel 5. Occipital lobe

Visual Fields – Rules of the Road

l Defect in 1 eye – retina or ipsilateral ON or tractl Bitemporal – optic chiasm lesionl Homonymous defects – posterior fossa lesions on

ipsilateral side– Denser above – temporal lobe– Denser below – parietal lobe– The more congruous, the more posterior the lesion

l Congruous lesion w/ macular sparing – occipital lobe lesion

What do you want to do next for this patient?

l 1. CT scanl 2. Lumbar puncturel 3. MRAl 4. MRIl 5. Neurology referrall 6. Neuro-surgery referral

1 Final Question

l Why was the first CT normal?

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A Tale of Transient Visual Loss

l 79 y/o WM called saying that he “Goes blind OD”l Happened 2 times yesterday, “blindness” lasts only 5

minutes, only ODl Describes it as a curtain that rising which gets

blacker over that timel Complete blackness occursl Gradually clears upl Been occurring for 2 mths

Transient Loss Continues

l Med hx:– 3 CVA– Aneurysm– ASA, verapamil

l Oc hx:– SRNVM w/subsequent macular scar OS– Bilateral cataract extraction

The Exam

l VA OD 20/25, OS 20/100l Pupils - 4mm round and reactive ODl 6mm oval and reactive OSl SLE – OD PCL

OS PCL w/ 2+ PCO, nasal subconj heme

l DFE – OD as shownOS – chorioretinal macular scar

What is his diagnosis?

l 1. Amaurosis fugaxl 2. Branch retinal vein occlusionl 3. Ischemic optic neuropathyl 4. Retinal embolusl 5. Transient Ischemic attack (TIA)

What is your next move?

l 1. Auscultate for bruitl 2. Carotid doppler ultrasoundl 3. ESR/ C-RPl 4. OCTl 5. Temporal artery biopsyl 6. VF

Transient Tale Part III

l Bruit R Carotidl ESR – 35mm/hrl Doppler ultrasound – Clinically significant

stenosis R Common carotid

l Immediate referral to vascular surgeon –endarterectomy

l 3 yrs later – VA 20/25 OD, no more sxs

The Case Of The Friendly Preacher’s Wife

l 38 y/o WF complaining of a “lazy” RUL x 1 mth.

l Lid droop seems worse in AMl HA over OD for past 3 days, otherwise she

feels normall Px denies redness, d/c, trauma. VA to her

seems normal.

Examination

l VA OD 20/20 OS 20/20 w/CLl Externals as shownl Pupils OD OS

– Light 3mm 3.5mm– Dark 3.5mm 5.5mm

– (-) APD

5 Step Pupil Evaluation

l 1. Anisocoria greater in dim or bright light?l 2.What are the lid positions?l 3. Direct responsel 4. Afferent responsel 5. Near vs direct response

l PERRLA or PERRL mg(-) ?

What Is The Preacher’s Wife’s Diagnosis?

l 1. Adie’s tonic pupill 2. Argyll-Robertson Pupill 3. Aneurysml 4. Horner’s Syndromel 5. Benign essential blepharospasml 6. Myasthenia gravisl 7. Pharmacologically induced anisocorial 8. She has the hots for her eye doctor!

Anisocoria greater in bright or dim light?

l Dim light – sympathetic

l Bright light – parasympathetic

l If 1 pupil is bigger in bright light but smaller in dim light – Tonic Pupil

Horner’s Syndrome-Oculosympathetic paresis

l Most common cause of a miotic pupil– Miosis– Ptosis– Anhydrosis– (-) APD– Kearne’s lower lid sign

Horner’s syndrome

l Etiology– 0 – 20 Trauma– 30 -50 Neoplasm– 50+ Malignancy

l Definitive diagnosis made byl 1.pupillary dilation lag testl 2. The “C” test

Practical Use For Cocaine in Your Office

l Instill 1 drop of 5% Cocaine– Normal eye dilates– Horner’s pupil will not dilate

– Positive diagnosis of Horner’s syndrome

Paredrine test

l Localizes lesionl Helps us figure out the etiology

l 1% hydroxyamphetamine– If pupil dilates- pre-SCG lesion– If pupil does not dilate – post-SCG lesion

l Use pain as a prognosticator

Horner’s Syndrome Causes

l 1st & 2nd order neuron lesions– Trauma– Intra-thoracic lesion– Tumors

l Pancoast’sl Thyroid neoplasml Malignancies

l Usually quieterl Get CT scan

Horner’s Syndrome Causes

l 3rd order neuron lesion– Intracranial vascular or inflammatory condition

(Vascular HA, Aneurysm, Sinusitis, Cavernous Sinus Sx,Idiopathic)

– Get Head CT, MRI, MRA

Friendly Preacher’s Wife

l Her OD pupil did not dilate with either cocaine or paredrine.

l What is her diagnosis?l Where does the lesion lie?

– 1. Head– 2. Neck– 3. Chest

Preacher’s Wife cont.

l What is the most appropriate test to order?1) CT scan of head2) MRI of head3) MRA4) ESR5) Benign neglect

The Friendly Preacher’s Wife

How did it all end up?

As Many Disease As She Pleases

l 77 WFl Macular hole repair OS 8 yrs priorl Subsequent SRNVM w/ large macular scarl VA OD 20/20, OS HM@6’ – stable for 5 yrsl Recently complained of HA “alot” over OSl Says her vision OS is worsening, “it will go

black at times!”

Exam

l VA OD 20/25- OS – LPl SLE – OD no change, OS – 2+ PCOl DFE –OD - D,M,V,P wnl OS small macular

bleed adjacent to macular scarl ONH - .1/.1 OD pink, .15/.15 OS large area

of PPAl What now?l Did we forget something?

Ancillary Tests

l IVFA – no evidence of new SRNVMl OCT – Plush NFL, no SRNVMl ESR – 20mm/Hrl C-RP – 0.8

l What now? Is she just crazy?l Are you sure we haven’t overlooked

anything?

Explain the VF result

l NOW what would you do?

MRI

l Suprasellar mass with impingement on ONl Probable gliomal Underwent resection

l Craniopharyngioma!

The Case Of Droopy Dora

l 71 y/o BF referred for recent onset of irritation OS.

l Also complains of blurry vision l HBP, arthritis,allergyl No hx of diplopia or eye turnl States that OS feels “weak”

Droopy Dora exam

l BCVA OD 20/50, OS 20/50+2l PERRL mg(-)l CT – 15pd LXT, no EOM restrictionl SLE – OD 1+ NS, OS 1+NS, tr bulb inj,

dellen, (-)NaFl but dry areas OS>ODl IOP – 15OD, 16OSl C/D - .45/.45 OU D,M,V,P wnl

I diagnosed dry eye, Rx’d Restasis OU BID

l Recheck 3 weeks(because of LXT)- and the cataracts must come out!

l CC: OS eyelid drooping x 1wkl VA 20/60 OUl Externals – CT -5LXT

– PA 10mm OD, 5mm OSl Disks flat, BP 186/86

What is Dora’s diagnosis?

l 1. Dermatochalasisl 2. Mechanical ptosisl 3. Bell’s palsyl 4. 3rd nerve palsyl 5. 6th nerve palsyl 6. CVAl 7. Something else

What is causing Dora to droop?

l 1. Hypertensionl 2. Diabetesl 3. Brain tumorl 4. Traumal 5. Aneurysml 6. CVAl 7. Myasthenia gravis

More Dora Data

l ESR, FBS, CBC, carotid doppler all nll VA fluctuatesl At next visit - 20pd int LXT l Lids- ptosis was absent this AM but now

ptosis measures 3mm, “the droop changes”

Now what is Dora’s diagnosis?

l 1.3rd nerve palsy due to aneurysml 2. 6th nerve palsy due to CVAl 3. MGl 4. Thyroid eye diseasel 5. Idiopathic 3rd nerve palsy

What test could you order to confirm this?

l 1.Ach-receptor antibody testl 2. ESRl 3. Tensilon testl 4. MRI of orbits

Ocular myasthenia gravis

l Disease of NMJl Variability and fatigability are keys to diagnosisl Cogan’s lid twitch, orbicularis weaknessl Vision may gradually deterioratel Diagnosis confirmed by tensilon test and Ach

antibody testl Tx – prednisone, pyridostigmine or monitorl Associated w/ thyroid dysfunction and thymoma

Myasthenia gravis

l Autoimmune disorderl Weakness of voluntary musclesl Disease of younger women and older menl Ocular, systemic or bothl Disease of thymus gland, thyroid gland

Ocular Myasthenia

l Alternating asymmetric bilateral ptosisl Worsens in bright lightl Worsens as day progressesl Myriad of EOM anomaliesl Fatigue phenomenon

Suspect myasthenia if:

l Alternating/ variable diplopial Mixed non-localizable neuropathiesl Emotional traumal (+) tensilon test

The Damsel In Distress

l 88y/o WF – Complains of “darkness” OSl Does not change, she woke up this way 3

days agol No pain, no HA, no photopsia or photophobial Med Hx- Synthroid, ASA, Simvastatin, Vit D,

Fel Normal affect to px??

Damsel’s particulars

l VA – OD-20/40 , ph NIl OS -20/125, ph NIl EOM – no restrictionl SLE – normal; no AC rxn, no RIl IOP – 15OD, 18OSl Conf VF – Constricted OS- only sees

temporallyl Before DFE – anything else??

What is your differential diagnosis?

l What tests do you want to do?

Lab Results

l ESR – 86mm/hrl C-RP – 1.01 (elevated)l Elevated white count, l Elevated platelets

l What is the diagnosis?

Now what?

l Refer to Neurol Refer to Retinal Refer for TA Biopsyl Refer to Pizzimenti!!l Begin steroid therapy