Clinical Case Challenges In Neuro-Optometry III Thomas J. Landgraf, O.D., F.A.A.O.

Clinical Case Challenges In Neuro-Optometry III

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

Before We Get Started….. On our LAST hour

together Foundations & Support

Groups Myasthenia Gravis Multiple Sclerosis

Case #6: Don’t Assume Anything

Or “The Case Of The Chronic Sixth Nerve Palsy”70 yo maleDiplopia with isolated abduction deficit OS

Other eye movements intact

No other significant neuro-eye findings

Case #6: Don’t Assume Anything Or “The Case Of The

Chronic Sixth Nerve Palsy” Most likely a case of

ischemic sixth nerve palsy Patient of vasculopathic age Sixth nerve palsy is isolated

May follow the patient without neuro-imaging

Expectation of improvement or resolution in 8-12 weeks

Case #6: Don’t Assume Anything Or “The Case Of The Chronic Sixth

Nerve Palsy” No expected recovery in 6-8 weeks upon re-

exam Refer to neurologist MRI with gadolinium

DDx includes: a mass lesion Pons, along the clivus, in the nasopharynx, at the

base of the brain, in the cavernous sinus, in the orbit


Or “The Case Of The Chronic Sixth Nerve Palsy”MRI with gadolinium

Large pontine mass consistent with glioma A death sentence in a young person Elderly, grows very slowly


Or “The Case Of The Chronic Sixth Nerve Palsy”Neurosurgical consult

Monitor without intervention

Prismatic correction

CN VI Palsy

BackgroundCommon cause of horizontal diplopiaMost commonly affected of the ocular motor nerves

CN VI Palsy

BackgroundMost emergent

Age 40 and underIf non-isolated

Think microvascular in elderly If isolated, most patients recover fully and may not

require referral

CN VI Palsy

Background: AnatomyLongest subarachnoid courseNucleus in the pons Innervates the ipsilateral lateral rectus

T2-weighted MRI in a patient with a chronic CN VI palsy shows a left pontine glioma

CN VI Palsy

Diagnosis: Who?Any ageVariety of causes

Ischemia most common in adults Elderly with DM and HTN

CN VI Palsy Diagnosis: Who?

Variety of causesNeoplasm

Mass lesion of CNS most common in children and young adults MRI of large posterior fossa tumor associated with hydrocephalus

and CN VI palsy in a 6 yoInflammation

Post-viral and ear infections in kidsAneurysmTrauma

CN VI Palsy

Diagnosis: SymptomsDiplopia

Binocular or monocular?Horizontal or vertical?

Pain, especially if growing lesion in the cavernous sinus

Additional neurologic signsDepends on the etiology

CN VI Palsy

Diagnosis: SignsAbduction deficit

EsotropiaMaximum on gaze to the side of the

palsy

Head turn

CN VI Palsy

Diagnosis: additional signs dependent on etiologyPapilledema with nausea, vomiting, tinnitus, HA’sProptosis Ptosis Increased ESR with HA and jaw claudicationRetraction of globe and narrowing of lid fissure on

attempted abduction

CN VI Palsy

Differential Diagnosis Ischemia InflammationNeoplasmAneurysmTrauma

CN VI Palsy

Ancillary Tests: Optometric In-OfficeVisual fieldsForced duction?

CN VI Palsy

Ancillary Tests: Referral Indications for Neuro-Imaging

Emergent if age < 30 yearsHead traumaPainNon-isolated Other etiologies besides microvascular, myasthenia gravis,

thyroid, Giant Cell, congenital

CN VI Palsy Ancillary Tests: Referral

Indications for Neuro-Imaging Consult with neuro-eye doc or neurologist reassures: “that level of comfort thing again”

Workup if microvascular: DM, HTN

CN VI Palsy

ManagementMicrovascular, trauma, idiopathic

Resolve spontaneously within 6 monthsComfort: patch, blur, block, Botox for temporary treatmentIf need long-term: prism, surgery

CN VI Palsy

ManagementFollow-up

CN VI every 6 weeks over 6 monthsIf you expect improvement?Neuro consult if no improvement

CN VI Palsy

My Clinical ExperienceAll isolated (majority) have been:

ElderlyMicrovascularIf it all makes sense, I hold off on the “Neuro-massage”

All non-isolated: YoungerPoor prognosis

Case #7: A Quickie

On-call ResidentPtosis ODSeveral months prior Ptosis OS

Seen by another resident

Don’t assume you are smarter than another residentDocumentation was correct

What is really going on?

Myasthenia Gravis

BackgroundAutoimmune Disease

Autoantibodies against acetylcholine receptors

Abnormal fatigueability of muscles under voluntary control

Usually orbital and facial muscles

Myastenia Gravis

BackgroundPrevalence: 1:20,000

But we see it!Ocular involvement: 90%Account for initial complaint in 75%

Myasthenia Gravis

Diagnosis: Who?Females under 50 / 7:3Males peak in late 50’sAssociated conditions: thymoma, thyroid disease,

diabetes, lupus, rheumatoid

Myasthenia Gravis

Diagnosis: SymptomsMajority present with ocular

symptomsPtosis: asymmetricDiplopia: any motility defectAnd spreadVariability of ocular fatigue

Worse at the end of the day Hx, Hx, Hx!

Myasthenia Gravis

Diagnosis: SymptomsNon-Ocular

Within two years of ocularLimb fatigueFacial muscle weakness

Difficulty breathing, chewing, talking, swallowing

Myasthenia Gravis

Diagnosis: SignsPtosis & EOM involvementCogan’s lid twitchExposure keratitisOphthalmoplegia

Orbicularis oculi weakness

Myasthenia Gravis

Differential DiagnosisPupils are never affectedNo eye painThyroid ophthalmopathy, INO (Internuclear Ophthalmoplegia), orbital

pseudotumor, botulism, myotonic dystrophy, Chronic Progressive External Ophthalmoplegia

Myasthenia Gravis

Ancillary Tests: Optometric In-OfficeMeasure palpebral apertures

Pupil center to upper lid margin

Sustained up gazeSqueezing of eyelids closed Initial VF

Myasthenia Gravis Ancillary Tests: Optometric In-

Office Ice Pack Test

5 minutes Improves neuromuscular transmission

ptosis Safe, speedy, easy and with relatively high

sensitivity and specificity

Sleep Test: eyes closed for 30 minutes FAT (Family Album Topography) Scan

Myasthenia Gravis

Ancillary Tests: ReferralTensilon (Endrophonium HCL) Test

IV 10 mg of TensilonWhy refer in Tennessee?

Rate of complications low but life-threatening Hypotension, bradycardia, cardiac arrest, respiratory arrest, seizures,

vomiting

Improves eyelid / motility defectAnticholinesterase

Myasthenia Gravis

Ancillary Tests: ReferralEMG (Electomyography)Acetylcholine antibody receptor test

Myasthenia Gravis

ManagementReferral

Neurologist, neuro-eye docInternist or PCP

Lab testing for associated conditionsCT scan of chest / mediastinum for thymoma

Myasthenia Gravis

Management: MedicalAnticholinesterases, steroids, immunosuppressantsThymectomyPlasmapharesis IV gammaglobulin

Myasthenia Gravis

Management: OptometricLid crutches, tapeOcclusionRarely prism, ptosis or strabismus surgeryFollow-up as needed post-diagnosis

Monitor for steroid side effects

Myasthenia Gravis

My Clinical ExperienceAm I missing this?Teaching about it may help…..

Case #8: OK To Not Refer?

History2006 68 yo Caucasian female

My patient since 1997“my glaucoma drops are too expensive”

Alphagan-P bid OU

H/O thinner that normal pachymetry OUH/O highest tonometry 20 mm Hg OU


History: Of interest to us today…..1997: first visit

Referred for pupil and glaucoma work-up Anisicoria noted over 10 years ago


HistoryHTN, hypercholesterolemiaH/O bypassHyzarr, Metiprolol, Lipitor, Aspirin, Lyrica, vitamins

No wonder the glc drop is too expensive

POAG, Horner’s Syndrome, ERM


ExamBVA: 20/25+, 20/25+Pupils: anisocora

Dim illumination: 4, 6 mmBright illumination: 3, 4 mm

Ptosis: 1 mm upper lid ODEOM’s: FROMConfrontation fields: FTFC OU


ExamSLX: essentially normal OU, mild NS OUT(a): 16, 15DFE

.7/.7 OD, .6/.6 OSMacular pigmentary changes periphery clear OU

Yearly HRT, VF

Case #8: OK To Not Refer

Latest VF OD


Latest VF OS


Latest HRT OD


Latest HRT OS


Assessment1. POAG OU

Stable HRT, VF, ONH appearance, IOPAlphagan-P too expensive

2. H/O Horner’s Syndrome ODBenign and stable

3. Macular pigmentary changes


Plan:1. Switch Alphagan-P to Brimonidine, RTC 1 month

IOP check, Education potential side effects of Brimonidine

2,3. To monitor


After preparing this lectureLooked way back in the record again10/5/83: “Anisocoria noted 1-2 years ago; neuro-

opthalmology work-up with no known causes” I never got the cocaine in ‘97Good idea eventually:

IopidineParedrine or Pholedrine

Horner’s Syndrome

Background1852: Claude Bernard first noted experimentally1869: Swiss ophthalmologist Johann Friedrich Horner

notedShould be called?

Bernard’s SyndromeBernard-Horner’s Syndrome

Horner’s Syndrome

Background: Sympathetic Anatomy of Eye & FaceFirst order sympathetic neuron

Begins in ipsilateral hypothalamusDescends through midbrain, pons, and medullaEnds at ciliospinal center of Budge at levels of C8-T1 in the

spinal cord

Horner’s Syndrome Background: Sympathetic

Anatomy of Eye & Face Second order pre-ganglionic

fibers Leave the ciliospinal center of

Budge Pass the pulmonary apex Travel up along carotid artery

sheath End in superior cervical

ganglion near bifurcation of common carotid

Horner’s Syndrome Background: Sympathetic Anatomy

of Eye & Face Third order post-ganglionic neuron

Travels along internal carotid artery to the cavernous sinus

Leave the internal carotid, travel with the abducens and join the ophthalmic division of the trigeminal

Enter the orbit with the naso-ciliary branch

Horner’s Syndrome Background: Sympathetic Anatomy of Eye

& Face In the orbit, the sympathetic fibers pass through

the ciliary ganglion Join the two long ciliary branches of the

nasociliary nerve and innervate the iris dilator muscle

Other sympathetic branches travel with branches of the ophthalmic artery to innervate the lacrimal gland, Muller’s muscles, and orbital vessels

Sympathetic fibers controlling facial sweating travel with the external carotid artery

Horner’s Syndrome

Why?Dysfunction of the sympathetic innervation to eye and

parts of face Interruption of oculosympathetic nerve supply

somewhere between hypothalamus and the eye

Horner’s Syndrome

Diagnosis: Who?No predilection

Age, race, gender, geographics

CongenitalPresents by age two with heterochromia

Horner’s Syndrome

Diagnosis: SignsPtosis Mullers muscle

1-2 mm upper eyelid

Reverse ptosisSlight elevation lower eyelid

Miosis iris dilator muscle

Horner’s Syndrome

Diagnosis: SignsAnisicoria > darkness

Dilator iris muscle normally more active

Dilation lag: prolonged redilation of the pupil after dimming the light

Hypochromia Iridis: typical if congenitalAnhidrosis

Horner’s Syndrome Differential Diagnosis: not limited

to….. Pre-Ganglionic ( First and Second-Order)

LESS COMMON BUT MORE OMINOUS

Pancoast Tumor Tuberculosis Aortic dissection Internal carotid artery dissection

MRA (MR angiography) Trauma Stroke, syphilis, tumor, MS,

lymphadenopathy

Horner’s Syndrome Differential Diagnosis

Post-Ganglionic (Third-Order) Aneurysm Atherosclerosis Herpes Zoster Trauma Sinusitis

Painful Horner’s Carotid dissection until proven othewise

Horner’s Syndrome

Differential DiagnosisVs Third Nerve Palsy

PtosisAnisicoria exaggerated in dim illumination Horner’s

Horner’s Syndrome

Ancillary Tests: Optometric In-Office?Pupil Testing 1: Horner’s?

Cocaine blocks reuptake of norepinephrine at the sympathetic nerve endings

Dilates normal eye after an hour No dilation in Horner’s due to lack of norepinephrine at nerve

endings

Availability of cocaine 10% solution?

Horner’s Syndrome Ancillary Tests: Optometric In-Office

Pupil Testing 1: Horner’s? Alternative: Iopidine (Apraclonidine)

Weak, direct action on alpha-1 receptors Normal: no dilation Horner’s: dennervation supersensitivity to

norepinephrine increase in alpha-1 receptors in iris stroma dilation

Horner’s Syndrome Ancillary Tests: Optometric

In-Office Pupil Testing 2: pre- or post-

ganglionic? Paredrine 1%

(Hydroxyamphetamine) releases norepinephrine from stores in nerve endings

Mydriasis in a normal pupil No mydriasis in a post-

ganglionic Horner’s due to destroyed nerve endings

“Fail-safe”? Postive Paredrine Test OD

did not dilate

Horner’s Syndrome

Ancillary Tests: Optometric In-OfficePupil Testing 2: pre- or post-ganglionic?Alternative: Pholedrine 5%

With third neuron damageHorner’s pupil will not dilatePost-ganglionic lesionIf dilates

Horner’s Syndrome

Management: Referral unless congenitalNeurologist, Neuro-Eye DocPCP, InternistCardiologist, Oncologist, Vascular SurgeonNo treatment to improve or reverse the condition

Horner’s Syndrome My Clinical Experience

Some pharmacist in Memphis is upset with me!

Glad we have Iopidine now

Clinical Case Challenges In Neuro-Optometry III Thomas J. Landgraf, O.D., F.A.A.O.

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Transcript of Clinical Case Challenges In Neuro-Optometry III Thomas J. Landgraf, O.D., F.A.A.O.