Clinical Case Challenges In Neuro-Optometry I

Thomas J. Landgraf, O.D., F.A.A.O.

“Clinical Case Challenges inNeuro-Optometry”

Thomas Landgraf, O.D., F.A.A.O.Clinical Associate Professor, UMSL College of Optometry

My Background

Graduate of ICO…Chicago

Residency at PCO…Philadelphia

SCO x 15 years…Memphis

Now at UMSL College of Optometry

In terms of Neuro-Eye… Dr. Lawrence Gray at ICO & PCO

My Background

At SCO…Chief of Ocular Disease Goals for this lecture Not an expert Share patient care

experiences Share “optometric

legal consultant” experiences

Resources

Journals and Internet Review of Optometry Review of Ophthalmology Handbook of Ocular Disease

Management Clinical Guide To Ophthalmic Drugs

Neuro-Optometry

Why spend 3 hours on it?

Conditions are both: Vision threatening Life threatening

“True” ocular emergencies

Case #1: ONH Edema?

Always A Tough DDx (Differential Diagnosis)S: 52 yo Caucasian

male referred to me

Tentative diagnosis of CRVO OS

Case #1: ONH Edema?

Always a Tough DDx

S:Painless vision loss OS x 2 weeksProsthetic OD due to traumaNo significant medical or ocular conditons

Low daily dosage of methadoneNicotine patch

Case #1: ONH Edema?

Always a Tough DDxO:BVA OS: 20/400OS pupil round and reactive to lightNormal SLXTonometry 17 mm HgBP: 280/170 RAS: not done at previous

visit

Case #1: ONH Edema?

Always a Tough DDx

O:DFE OS:

Optic nerve head edemaAccompanied by flame

hemes, exudates, cotton wool spots, and macular edema

Normal peripheral retina

Case #1: ONH Edema?

Always a Tough DDx

A: Malignant Hypertension and Resultant Retinopathy OS

P: Immediate referral to medical center For lowering of BP Referral to retinal specialist

Level Of Comfort Confirmation

Case #1: ONH Edema?

Always a Tough DDx

Follow-up 4 months laterCurrent meds: minoxidil, norvasc,

coumadinHTN and its complicationsNoted improved vision

But some glare, distortion, “wavy lines” in central vision

Case #1: ONH Edema?

Always a Tough DDxFollow-up 4 months laterBVA OS: 20/20BP: 160/85DFE OS: exudative

macular star, healthy ONH (.2/.2), normal peripheral retina

Case #1: ONH Edema?

Always a Tough DDxFollow-up 4 months later Resolving

Malignant Hypertensive Retinopathy

Improved Blood Pressure

Educated on compliance

Case #1: ONH Edema?

Always a Tough DDx

Bottom LinesPrimary Care OD’s need to take BP’s

Especially on those with retinal vascular disease

Consider typically bilateral retinal conditions

In monocular patients

Case #1: ONH Edema?

Always a Tough DDxTimely diagnosis for malignant HTN

Can significantly reduce morbidity and mortalityLike Neuro-Eye Disease: sight and life

threatening

Pseudotumor Cerebrii (PTC)

Background “false brain tumor” Increased intracranial pressure

without an intracranial mass Major diagnosis of exclusion: a

true intracranial tumor All patients with papilledema must

have neuro-imaging studies

PTC: Why?

Poor CSF absorption By meninges surrounding brain

and spinal cord Increased intra-abdominal

pressure From obesity elevated intrathoracic pressure decreased venous drainage from

the brain

PTC: Diagnosis

Who? Obese women of childbearing

age Secondary

Obstruction to venous drainage: cerebral venous thrombosis

Exongenous agents: tetracycline, vitamin A, corticosteroids, BCP’s

Medical conditons: lupus, sarcoidosis, anemias, blood dyscrasias

PTC: Diagnosis

Symptoms Bad HA’s: frontal, around the

eyes, pressure-like, throbbing Transient visual loss Intracranial noises: heartbeat or

whooshing sound in ears, tinnitus Vision loss: blur, temporal VF

defect

PTC: Diagnosis

Signs Optic disc edema

Unilateral, bilateral, asymmetric

VA, pupils, EOM’S usually normal

VF: blind spot enlargement, inferonasal loss, generalized constriction

PTC: Differential Diagnosis

Intracranial mass

Meningitis: abrupt onset, fever and chills, stiff neck

Bilateral inflammatory optic neuropathy: early and central vision loss, pain on eye movement, retrobulbar

PTC: Differential Diagnosis

Pseudopapilledema: optic disc drusen or tilted discs, ultrasound may aid

Neuroretinitis: macular exudate, early central vision loss

Bilateral ION: older, vascular risk factors, painless, early vision loss

PTC: Ancillary Tests

Optometric In-Office:VF B scan ultrasoundPhotos or optic nerve

imaging

PTC: Ancillary Tests

Neurologist or neuro-eye doc referralNeuroimaging before lumbar puncture

Standard MRI of the brainCT scan with contrast if patient markedly obese

Neuroimaging

Major Scans Used To Evaluate Neuro-Eye DiseaseCT (Computerized tomography)MRI (Magnetic Resonance Imaging)

Neuroimaging

CTGood to view bony abnormalities,

calcifications, acute hemorrhagesValuable to diagnosis of orbital processesTest of choice for thyroid eye disease

Neuroimaging

MRIFar better at characterizing soft tissuesPreferable for most intracranial processesNot subject to bone artifactContrast media and special studies can

sharpenGadolinium is a contrast material that can

increase signal intensity

PTC: Ancillary Tests

Lumbar Puncture Required for the diagnosis of

PTC Neurologist, radiologist or ER

physician Usually > 200 mm

Lumbar PunctureProcedure Patient positioned on side in fetal position with

back fully flexed 18 g needle inserted at L4-L5 interspace Opening pressure measured when needle

penetrates subarachnoid space HA is most common complication

Lumbar Puncture

Opening pressureNormal: 60-80 mm of H20Borderline elevated: 180-210 mm of H20

Lumbar Puncture

CSF evaluationColor

Clear and colorless is normalCloudy: infectionXanthochromic (yellow): subarachnoid

hemorrhageCell count and differential, cytology,

chemical analysis, serologic analysis, microscopy, culture

PTC: Management

“Comanage” with neurologist Initial LP improved signs and sxsVF, DFE, photos or optic nerve imaging

every month x 3 monthsEvery 2-3 months thereafter for about a

year Individual case variability

PTC: Management

“Comanage” with neurologistOther options for some persistent signs

and sxsCAI’s : acetazolamideOther diureticsWeight lossHA management

PTC: Management

Diamox Not just for angle closure Decreased CSF production up to

50% 1-3 grams qd

500 mg bid, tid, qid

Side effects: taste alteration, nausea, fatigue, diarrhea, tingling

Not with sulfa allergies, kidney disease

PTC: Management

Headache management Topamax (topiramate)

Migraine prophylaxis and epilepsy

PTC: HA relief and mild inhibition of carbonic anhydrase, also causes weight loss

Recently: development of angle closure glaucoma from choroidal expansion

PTC: Management

For signs and symptoms unresponsive to LP, severe vision lossCorticosteroidsSurgery

Optic nerve sheath fenestrationCSF diversion (shunt)

PTC: My Clinical Experience

Relatively rare condition? Not at SCO

“Comanagement” turns into MANAGEMENT Optometrists take the time Need to be familiar with ancillary

diagnostic tests and treatment options

Case #2: Monocular Acute Vision Loss In A Golden

GirlS:85 yo Caucasian femaleCx: acute vision loss OD2 weeks earlier

EaracheSore temporal veinsJaw claudication

Past medical hx: non-contributory

Case #2: Golden Girl

O:BVA

LP OD, 20/30 OS+APD ODBP: 150/100No carotid bruitsSLX: NS consistent with 20/30 VA

Case #2: Golden Girl

O: DFE: pallid swelling of the

optic nerve OD Othewise normal retina

and posterior pole OU

Case #2: Golden Girl

A: Provisional Diagnosis: Giant Cell

Arteritis OD

P: FLAN:

increased arterial filling time OD Choroidal nonfilling defect OD

80 mg Prednisone po daily

Case #2: Golden Girl

P: R/O all causes of Anterior Ischemic Optic NeuropathyCBC:

Elevated monocyte and platelet countsESR: 44FTA-ABS and VDRL non-reactive

Case #2: Golden Girl

One week later….. Right temporal artery

biopsy Ear pain and

temporal HA resolved

Case #2: Golden Girl

Two weeks later…..ESR: 4Plan:

Monitor with ESRAnd for prednisone side effects

Case #2: Golden Girl

Eventually…..VA did not improve OD

But remained stable OS

Anterior Ischemic Optic Neuropathy (AION)

ArteriticOr…..

Giant Cell ArteritisNomenclature following vision loss

Temporal Arteritis

AION-artertic

BackgroundGranualomatous vasculitis of medium-

sized arteries “True” ocular emergencyThe Goal: Prevention of contralateral vision

loss

AION-arteritic

BackgroundContralateral vision loss

2/3 if untreatedWithin weeks if untreated

AION-arteritic

Why

Granulomatous vasculitis of temporal artery Occlusion of short posterior ciliary arteries (supply anterior optic nerve) AION-artertic

AION-arteritic

Diagnosis: Who?Rose Nylen on the Golden GirlsAverage age of onset = 70 yearsFemale and ScandanavianLower incidence rates

Tennessee & Israel

AION-arteritic

Diagnosis: symptomsUnilateral decreased VA,

temporal HA, scalp tenderness

VA usually < 20/200Amaurosis fugaxAnorexia, fever, malaise,

depressionOnset is variable

AION-arteritic

Clinical FeaturesVasculitis of coronary arteries: MI, CHF,

angina pectorisNeurologic: peripheral neuropathies,

ischemic brain damagePolymyalgia Rheumatica: pain and

stiffness of the neck, shoulders, hips

AION-arteritic

Diagnosis: signs AION-arteritic

Optic nerve edema, hemes, cotton wool spots

APD VF defects: central, altitudinal, arcuate

AION-arteritic

Diagnosis: signs AION-arteritic

Pallor described as chalky white

CRAO in up to 10% of patients Disc eventually glaucoma-

like with cupping BUT with pallor

AION-arteritic

Differential Diagnosis Vs. non-arteritic AION

Worse VA Worse VF HA and scalp tenderness Constitutional symptoms Older Worse ESR, CRP, CBC

variables

AION-arteritic

Ancillary TestsOptometric In-Office

VFOptic nerve imagingPhotosFLAN considered to check for

choroidal perfusion defects

AION-arteritic

Ancillary TestsReferral

ESR (erythrocyte sedimentation rate) Westergren 15% of GCA patients: normal ESR

CRP (C-reactive protein) Elevated in > 91%

Also elevated WBC, platelet counts; IgG anticardiolipin antibodies

AION-arteritic

Ancillary Tests Referral

Temporal artery biopsy Should be performed on all

suspects > 95% sensitive, 100% specific Can be done shortly after

steroid treatment Inflammatory cells in the

muscular walls of the artery

AION-arteritic

Diagnosis: Summary History and clinical

impression ESR and CRP Confirm with

temporal artery biopsy

AION-arteritic

ManagementReferral: neurologist, internist (PCP),

rheumatologistFor suspects or diagnosed:

Systemic steroids

AION-arteritic

ManagementSystemic steroids

Hospital admission1-2 gm IV methylprednisone x 2-3 days 60-100 mg of oral prednisone: tapered very

slowly

AION-arteritic

Management

Systemic steroidsAnecdotal vision recovery?Poor prognosis?No solid support for anti-steroid medications

(Rheumatrex aka methotrexate)

AION-arteritic

My Clinical Experience This is rare?

Most important from an educational perspective

Presume the worst if suspect Vs. glaucomatous optic

neuropathy Combination of AION + OAG in

my patients