Post on 01-Dec-2020
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
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
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
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.
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
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.
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.
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
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.
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.
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
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
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