N-O Emergencies Pearls:Important Diagnosis · 2011. 3. 6. · NEURO-OPHTHALMIC PEARLS ROSA ANA...

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NEURO-OPHTHALMIC PEARLS ROSA ANA TANG, MD,MPH,MBA MS EYE CARE-UHCO- 2011 N-O Emergencies Pearls:Important Diagnosis Severe consequences : Irreversible damage to the patient *Potentially treatable * Early Dx & management critical Medico legal implications * Early intervention saves lives. 1) Acute diplopia Acute painful ophthalmoplegia [more anxious if pupil abnormal] 2) Acute visual loss [especially if disc is normal]-check that pupil for RAPD Is it optic neuritis or CRAO?? 3) Visual field defects [BTH and acute] 4) Painful anisocoria [dissection] 5) Numbness [with or without pain] N-O Emergencies O Emergencies High Anxiety Level High Anxiety Level Neuro Neuro- ophthalmic Emergencies ophthalmic Emergencies Excessive delay in diagnosis Excessive delay in diagnosis Pituitary apoplexy Pituitary apoplexy: permanently blind if : permanently blind if no intervention no intervention Compressive optic neuropathies Compressive optic neuropathies: mostly : mostly if due to pituitary tumors as reversibility is if due to pituitary tumors as reversibility is tied to chronicity tied to chronicity Myasthenia / thyroid disorders Myasthenia / thyroid disorders: due to their systemic associations and due to their systemic associations and health related issues [thyroid storm and health related issues [thyroid storm and myasthenic crisis] myasthenic crisis] Neuro “Pearls” to keep you out of trouble Beware of the silent Neuro-ophthalmic patient : patients with brain tumors can be sometimes hidden behind a diagnosis of glaucoma –sp. low tension glaucoma. 1) Acute diplopia Acute painful ophthalmoplegia [more anxious if pupil abnormal] 2) Acute visual loss [especially if disc is normal]-check that pupil for RAPD Is it optic neuritis or CRAO?? 3) Visual field defects [BTH and acute] 4) Painful anisocoria [dissection] 5) Numbness [with or without pain] Pearl:TOP TEN DIAGNOSES Pearl:TOP TEN DIAGNOSES YOU DON YOU DON’T WANT TO MISS T WANT TO MISS

Transcript of N-O Emergencies Pearls:Important Diagnosis · 2011. 3. 6. · NEURO-OPHTHALMIC PEARLS ROSA ANA...

Page 1: N-O Emergencies Pearls:Important Diagnosis · 2011. 3. 6. · NEURO-OPHTHALMIC PEARLS ROSA ANA TANG, MD,MPH,MBA MS EYE CARE-UHCO- 2011 N-O Emergencies Pearls:Important Diagnosis Severe

NEURO-OPHTHALMICPEARLS

ROSA ANA TANG, MD,MPH,MBAMS EYE CARE-UHCO- 2011

N-O EmergenciesPearls:Important DiagnosisSevere consequences :• Irreversible damage to the patient

*Potentially treatable* Early Dx & management critical

Medico legal implications* Early intervention saves lives.

1) Acute diplopiaAcute painful ophthalmoplegia[more anxious if pupil abnormal]

2) Acute visual loss [especially if disc isnormal]-check that pupil for RAPD

Is it optic neuritis or CRAO??3) Visual field defects [BTH and acute]4) Painful anisocoria [dissection]5) Numbness [with or without pain]

NN--O EmergenciesO EmergenciesHigh Anxiety LevelHigh Anxiety Level

NeuroNeuro--ophthalmic Emergenciesophthalmic EmergenciesExcessive delay in diagnosisExcessive delay in diagnosis

Pituitary apoplexyPituitary apoplexy: permanently blind if: permanently blind ifno interventionno interventionCompressive optic neuropathiesCompressive optic neuropathies: mostly: mostlyif due to pituitary tumors as reversibility isif due to pituitary tumors as reversibility istied to chronicitytied to chronicityMyasthenia / thyroid disordersMyasthenia / thyroid disorders::due to their systemic associations anddue to their systemic associations andhealth related issues [thyroid storm andhealth related issues [thyroid storm andmyasthenic crisis]myasthenic crisis]

Neuro “Pearls” to keepyou out of trouble

Beware of the silent Neuro-ophthalmicpatient :patients with brain tumors can besometimes hidden behind a diagnosis ofglaucoma –sp. low tension glaucoma.

1) Acute diplopiaAcute painful ophthalmoplegia[more anxious if pupil abnormal]

2) Acute visual loss [especially if disc isnormal]-check that pupil for RAPDIs it optic neuritis or CRAO??

3) Visual field defects [BTH and acute]4) Painful anisocoria [dissection]5) Numbness [with or without pain]

Pearl:TOP TEN DIAGNOSESPearl:TOP TEN DIAGNOSESYOU DONYOU DON’’T WANT TO MISST WANT TO MISS

Page 2: N-O Emergencies Pearls:Important Diagnosis · 2011. 3. 6. · NEURO-OPHTHALMIC PEARLS ROSA ANA TANG, MD,MPH,MBA MS EYE CARE-UHCO- 2011 N-O Emergencies Pearls:Important Diagnosis Severe

Pearl: TOP TEN DIAGNOSESYOU DON’T WANT TO MISS

6)Pituitary apoplexy: permanently blind if nointervention7)Progressive visual loss: Compressive opticneuropathies: mostly if due to pituitary tumorsas reversibility is tied to chronicity8)Myasthenia :due to their systemic associations and healthrelated issues [ myasthenic crisis]

Pearl:TOP TEN DIAGNOSESYOU DON’T WANT TO MISS

9. GCA10. PAPILLEDEMA

Neuro symptoms thatNeuro symptoms thatmake us PANICmake us PANIC

1. Acute diplopia: can be a killer[ie: aneurysm/myasthenia] - always make

sure what the underlying cause is

2.Papilledema: the cause can kill thepatient

Three sx that may causeus to PANIC too!!!

3) Visual field defects [BTH and acute]4) Painful anisocoria [dissection]5) Numbness [with or without pain]

SIGN: PUPILABORMALITY :Anisocoria

P =pupil abnormality

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Anisocoria

Normal light reaction Abnormal lightreaction

• physiologic

• Horner’s• Adie’s tonic

• 3rd nerve palsy

• pharmacologic

• sphincterdamage

In a patient with aUnilateral fixed dilated

pupil :LOOK FOR :

ANY HINT OF III CN PARESIS ORPTOSIS.LIGHT-NEAR DISSOCIATION OFPUPIL : TONIC PUPILEVIDENCE OF ANT. SEGMENTTRAUMA IN SLIT LAMP EXAM.

Question :what if there is greateranisocoria in dark

P= pupil abnormality

Dilation lag:

Anisocoria w/ dilation lag

In Dark after 15-20 seconds

In dark in 1st 5 seconds

In light

How to localize thelesion in Horner’s

syndrome

Historical : based on associatedsymptoms.Clinical: Based on associated signs.Based on pharmacological testing.

Localization of the lesionaccording to the symptoms in

Horner’s SyndromeFirst-order neuron

lesionsSecond-order neuron

lesionsThird-order neuron

lesions

Hemisensory loss

Dysarthria

Dysphagia

Ataxia

Vertigo

Nystagmus

Preceded by trauma

Facial, neck, axillary, shoulder or

arm pain

Cough

Hemoptysis

History of thoracic or neck surgery

History of chest tube or central

venous catheter placement

Neck swelling.

Include diplopia from sixth

nerve palsy

Numbness in the distribution

of the first or second division of

the trigeminal nerve

Pain.

HISTORICAL DIAGNOSIS OF HORNER’S

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Horner’s

Etiology50% idiopathic50% secondary

Syndromes:Lateral medullary or

Wallenberg: centralCavernous sinus:

post-ganglionic+VI CN ipsilateral

Pharmacological :How to confirmHorner’s syndrome-Cocaine

testingCocaine blocks the re-uptake of theneurotransmitter Norepinephrine in pre-synaptic terminal causing dilation of thenormal pupilIn Horner’s: no dilation of the tested pupil isseen with cocaine. This is due to lack of Nor-epinephrine molecules on the pre-synapticvesicle : there is nothing to block .Tells us THERE IS A Horner’s but not thelevel of the lesion.

Apraclonidine (0.5% or 1%)Iopidine

Apraclonidine is an ocular hypotensiveagent.

It is a weak, direct-acting alpha-1 receptoragonist.

Apraclonidine has little to no effect on a normalpupil size.

APRACLONIDINE 0.5 %

Should eliminate or reverse theanisocoria [small Horner’s pupilbecomes larger and normal stays samesize].Reverses the ptosis.Read after 30 minutes of instillation.

Where is the Horner’s lesion- -Paredrine (Hydroxyamphetamine) Test

1. Hydroxyamphetamine releasesnorepinephrine from the stores inthe post-ganglionic neuron causingdilation of the pupil.

Where is the Horner’s lesion- -Paredrine (Hydroxyamphetamine) Test

3. First and second order neurondysfunction-no effect of Paredrineas this substance only works at thelevel of the third order(post-ganglionic neuron) hence pupilDILATES with paredrine as thenormal pupil does.

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Congenital Horner’sSyndrome

Heterochromia with LIGHTER IRIS in Horner’seye most distinct feature

Normal pigmentation of iris depends on the sympatheticinnervation and occurs before age 2

So usually in Horner’s before age 2 you see heterochromia Think of birth trauma to pre-ganglionic neuron (forceps).

Question: acute painfulHorner’s

P=pupil abnormality

Etiology of Post ganglionicHorner’s

1. Internal carotid A. dissection - AcuteUnilateral headache or facial pain.2. Cluster headache- Transient post-ganglionic Horner’s syndrome withepisodes of excruciating hemicranialheadaches.3. Trauma- base of skull fractures4. Cavernous sinus Lesion

SIGN: PUPIL

MOST IMPORTANT OBJECTIVEPUPIL SIGN IN UNILATERAL VISUALLOSS CASES

SIGN : cornealanesthesia

In the absence of corneal diseaseWhat does this means?

SIGN: DISC EDEMA

IF BILATERAL: MUST EXCLUDEPAPILLEDEMA

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Swollen optic nerves

Bilateral ON swelling is likely due toincreased ICPFirst investigation should be imagingwith brain MRI to exclude brain tumor

Increased ICPIncreased Intracranial Pressure withabnormal Imaging and/or CSF (otherthan high pressure)MOVIE pneumonic:to be excludedFIRST!!!!!!!!!!!! while monitoringVA/fields M Mass/Meningitis O Obstructive Hydrocephalus V Venous Hypertension I Infectious Causes

(Abscess/Meningitis)

E E dema (Non Infectious meningeal )©NEDS2001

Pearl

The diagnosis of pseudotumor cerebriis a diagnosis of exclusion This is basedon a specific diagnostic criteria asfollows:Hx: no sx other than HA, tinnitus ,dv,tvo.Exam :disc swelling normal BPNormal MRI and CSF except for highpressure

Modified Dandy criteria of IIHRef: Friedman &Jacobson : Neurology :59:1492-1495,2002

Symptoms and signs ofincreased ICPOtherwise normalneurologic examNormal level of alertnessNeurodiagnostic normalexcept elevated ICPNo other cause ofincreased ICP present.

Japanese Pearl!!BILATERAL DISC EDEMA

ALWAYS CHECK THE BLOODPRESSURE AS MALIGNANTHYPERTENSION CAN PRESENTWITH THIS FUNDUS PICTURE

Secondary Pseudotumorsyndromes

All imaging negative, includingMRI, MRA, MRV, Angiogram,CAT scanLooks like primary pseudotumorcerebri but there is somethingelse that may be precipitating it.

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Typical Patient:- PTCClinical Associations

Obese female of childbearingageGeneral population:1 : 100,000

Women 20-44 who are 20%greater ideal body weight:19.3 : 100,000

Female : Male8 : 1

ObesityRecent weight gainPregnancy?

SIGN: DISC EDEMA-WHAT ABOUTTHIS PICTURE?

ODEMS type I NEURORETINITISUNI OR BILATERAL

TYPICAL : Associated to infectionsViralSyphilisCat ScratchLyme’sTBToxoplasma /ToxocaraIdiopathic

ODEMS -ATYPICALVascular entities

AION: rarePapilledema-CHRONICMalignant Hypertension: bilateral

Pearls

True ODEMS is idiopathic, often withoptic disc edema as the presentingsign & 2 weeks later the star follows

ODEMS IS NOT SEEN IN MSPATIENTS.

Neuroretinitis with specific etiologiesshould be treated appropriately

Pearl

Every new patient c/o blurry vision andyou cannot correct to 20/20 OR you findelevated discs should have at leastconfrontation VF & pupil check for RAPD

Automated perimetry for those who havelots of Sx and no findings.

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SIGN: PROPTOSIS

MOST COMMON CAUSE UNI ORBILATERAL : THYROIDIN CHILDREN –UNILATERAL ANDACUTE-THINK ORBITAL CELLULITIS

IF PULSATILE TINNITUS/BRUIT –THINK CCF

TRIO TYPE I

Occurs most often in women.Symmetric proptosis.Symmetric eyelid retraction.Minimal orbital inflammation.Minimal or no myopathy, howeverEOMS may be large due to edema notmyositis.Corneal exposure may be considerable.

TRIO TYPE II

EOM enlargement marked /asymmetricwith myositis and restrictive myopathy :IR & MR most common involved : “ can’tlook up “. (IR>MR>SR>LR)With restrictive myopathy the eye ispulled in the direction of the involvedmuscle.

Clinical Presentations-Thyroid

Mild orbitopathy

Moderate orbitopathy

Optic neuropathy

Goals of Treatment-Thyroid

Protection of visual acuityControl of inflammationCorrection of muscle dysfunctionReduction of proptosisImprovement in cosmetic appearance

TRIO-TREATMENT

Localized protective/lubrication.Medical anti-inflammatory: high dose( 80-100 of prednisone QD) for few weeks.Medical : orbital radiation is preferred forpatients over age 55 ( Ref: Martin &Corbett ).Surgical for visual loss :orbitaldecompression if medical treatment fails.Surgical for motility/lid : only whenorbital findings stabilize.Radiotherapy : less and less likely to be ofany help

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Pituitary tumors and theoptometrist

Pituitary tumors in adults present a wide spectrum of symptoms andphysical findings many of which affect vision.Visual symptoms are gradual in onset due to the benign histopathologyof these tumors and its location.

PITUITARY GLAND TUMOR – CLINICALPRESENTATION

Hormone Mass effect

Galactorrhea/Amenorrhea

Acromegaly Cushing’s Hypopituitarism

Neuro-ophthalmologic ( visionloss, diplopia)

Cerebrospinal fluid (CSF) leak

Pituitary tumortreatment

Goals of pituitary tumor treatment :

control of tumor growth

normalize pituitary function

preservation or restoration of visualfunction .

In pituitary disorders

Routine visual field examinations aftertreatment: 1st year every 3 months 2nd to 5th year every year Every 2 years there after

Savino et al

PITUITARY APOPLEXYTRIAD

SEVERE HEADACHE WITH SX OFSAHACUTE DIPLOPIA : III OR VI BUTTOTAL OPHTHALMOPLEGIA MOSTLIKELYVISION LOSS /BTH

Sign: PTOSIS

1.Isolated ptosis with no DV and normalpupils is SELDOM an emergencyOcular myopathy [MG] rarely presentsemergently if there is only ptosis unlesscan’t swallow or breath-then we are introuble.

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Purely OcularMyasthenia

Initial presentation of MG in up to70%Ocular precedes clinically generalizedMG in 50-70 % of patients. Usually generalizes within 2 years of

onset of ocular symptoms

Laboratory tests in MG

Anti-acetylcholine receptor bindingantibodies should be measuredPositive in 50 % of Ocular MG and 90%of Systemic ( Generalized MG)

Less commonantibody:MUSK

Seen in Ach Receptor Ab negativemyasthenia.Can be seen with ocular myasthenia butrarely.Worse prognosis in regards to systemicsymptoms.

M.G.-Management -Adjustments

Change medications that canexacerbate or cause

Education (rest, pacing, diet,temperature, stress)

For ocular (ptosis crutches,prisms, patching)

SIGN: PTOSIS

<2 mm: Horner’s - tip: look for brother’skiss sign [narrow fissure] and miosis>2 mm:Mechanical: isolated [lev dehiscence]Myopathic: variability= MGDiplopia: look for IIIrd CNP

Acute double vision

Beware of calling decompensatedstrabismus any case that presents withacute diplopia and no clear cut CNparesis.

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Acute Painful Diplopia

Aneurysm –about to RuptureDissection VB- about to happenPituitary Apoplexy- shock for lack of steroidsMucormycosis Orbit and Cavernous SinusBasilar Meningitis- TB, cryptoGiant Cell arteritisTolosa Hunt : ALWAYS a Dx of Exclusion

Pearl

All pts with PARTIAL IIIRD CNPARESIS whether or not the pupil isinvolved need URGENT imaging toexclude an aneurysm

III CN paresis and therule of the pupil

SIGN: PTOSIS WITH FELLOWTRAVELERS [EOM+PUPIL]

INVOVEMENT

Isolated CN paresisWhen is ischemic or microvascular?

MONONEURITIS

Should be truly ISOLATED so need Neuro-ophthalmic exam.Who are the vasculopaths that get ischemic CNP:

**Diabetics by far : check Hg A1 C**Rarely in A. Hypertensive : 7/1 ratio w/DM**Higher risk HBP + Smokers

should be a diagnosis of exclusion in a nonvasculopath.Should resolve in 3 months (90 day palsy).Don’t forget Giant Cell Arteritis as a cause in elderly.

Sign: T for TEMPORALARTERITIS

KeyDiagnostic/Management

Issue: Is it GCA?Jaw Claudication/ HA: high riskTransient Vision Loss Almost never in NAION 27 % of cases with AAION (Hayreh)

Choroidal Filling Delay on FFA 100% of patients with AAION (94 cases

Hayreh)WESR/CRP (Acute Phase Plasma Protein) If CRP > 2.45 mg/dl combined with ESR > 47

mm/hr is 97% specific for GCA.DIPLOPIA IN 10-15% IS PRESENTATION

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Optic Nerve:(a) ION:AAIONAION: Anterior with markedly

pallid GLOBAL edema .(b) Cup-to-disc ratio greater than 0.2 in

fellow eyes.

GCA-OPTIC NERVEINVOLVEMENT

HOW TO HELP DIAGNOSINGGCA

Clinical suspicion : the most importantone.Laboratory markers :

-WESR- CRP

high platelet count

PEARL -HARBOR

Don’t miss GCA: think of it on everyonethat is >50 yr old with:Transient LOV one eyeTransient /PERMANENT diplopiaAION if disc white more likelyCilio retinal artery occlusionTonic pupil one eye in elderlyIsolated CN paresis

Arteritic Ischemic Optic Neuropathy(Temporal Arteritis)

Give SteroidsIMMEDIATELY

NAION

RISK FACTORS:Cupless discDM,HBP,Lipids+SmokingSpinal surgerySleep apneaHyperhomocysteinemiain young pt

Linked to:AMIODARONEVIAGRA/CIALISINTERFERON

PEARL-NEURO OPHEMERGENCIES

LIFE THREATENING• 1)Double vision due to third nerve palsy

due to aneurysm• 2)Bilateral disc swelling due to brain

tumor herniating or venous thrombosiscausing stroke

• 3) Acute bilateral /unilateralophthalmoplegia from pituitary apoplexy

Page 13: N-O Emergencies Pearls:Important Diagnosis · 2011. 3. 6. · NEURO-OPHTHALMIC PEARLS ROSA ANA TANG, MD,MPH,MBA MS EYE CARE-UHCO- 2011 N-O Emergencies Pearls:Important Diagnosis Severe

PEARL-NEURO OPHEMERGENCIES

BILATERAL SIGHT THREATENING• Acute LOV from pituitary apoplexy or

from GCA.[ ON involvement]• Acute HH from stroke related to

CAROTID DISSECTIONUNILATERAL SIGHT THREATENING• Acute LOV in GCA• CRAO from embolic disease or GCA

LAST PEARL-THREECOMMON MISTAKES

THAT CAN LEAD TO PERMANENTBLINDNESS OR DEATH ARE:1) Not suspecting the possibility ofserious orbital or brain disease as thecause of the patient’s eye complaints.2)Not performing a careful and thoroughhistory and examination.3)No referring pts for ConsultationEARLY and URGENTLY when needed