NEURO-OPHTHALMIC DIAGNOSES NOT TO MISS Nailyn Rasool Assistant - - PowerPoint PPT Presentation

neuro ophthalmic diagnoses not to miss
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NEURO-OPHTHALMIC DIAGNOSES NOT TO MISS Nailyn Rasool Assistant - - PowerPoint PPT Presentation

NEURO-OPHTHALMIC DIAGNOSES NOT TO MISS Nailyn Rasool Assistant Professor of Neurology and Ophthalmology University of California, San Francisco OBJECTIVES Become comfortable with the neuro-ophthalmic examination Identify and manage


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NEURO-OPHTHALMIC DIAGNOSES NOT TO MISS

Nailyn Rasool Assistant Professor of Neurology and Ophthalmology University of California, San Francisco

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OBJECTIVES

  • Become comfortable with the

neuro-ophthalmic examination

  • Identify and manage neuro-
  • phthalmic emergencies
  • Have fun!!
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  • Not all Optic Neuritis is made equal
  • Transient Monocular Vision loss is a TIA
  • Don’t forget GCA! (This is neuro-op after all!)
  • Think twice about a young 6th
  • If the MRI doesn’t match the patient – check again
  • A Temporal Visual Field Defect = Optic Chiasm until proven otherwise
  • Even if it’s just in one eye

Take Home Points

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NEURO-OPHTHALMOLOGY: X MARKS THE SPOT!

Divided into Afferent and Efferent Systems

ALL ABOUT LOCALIZATION!

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Neuro-Op in a Nutshell

LOCALIZATION OF AFFERENT DYSFUNCTION

  • Globe / Retina
  • Optic Nerve
  • Chiasm
  • Optic Tract
  • Optic Radiations
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LOCALIZATION EFFERENT DYSFUNCTION

Muscle Junction Nerve ­ Orbit ­ Orbital Apex ­ Cavernous sinus ­ Subarachnoid space Brainstem

  • Nucleus
  • Internuclear

Supranuclear

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ACUTE VISION LOSS

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HISTORY OF PRESENT ILLNESS

  • 32 year old Asian woman
  • “For the past 2 days it looks like I’m

looking through a dirty glass in my right eye”

  • “I have some discomfort when I move my

eye"

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Right Left Visual acuity (cc) 20/800 20/20-1 Color (HRR) 3/6 6/6 Pupils Right RAPD External Examination Normal Neuro-exam Normal Normal

EXAMINATION

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VISUAL FIELDS

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2 DECADES SINCE THE ONTT

What Has Changed?

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LET’S CHANGE THE STORY

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DOES ANY OF THIS MATTER?

MS disease-modifying therapies aggravate NMOSD and can result in relapses and worse outcomes Includes IFN-beta, natalizumab, fingolimod and alemtuzumab Early appropriate therapy results in reduced disability and recurrences

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HISTORY OF PRESENT ILLNESS

  • 45 year old East Asian woman
  • “I was working on my computer

yesterday and things became dark in my left eye – like a shade. It lasted around two minutes and then slowly resolved. There was no pain.”

  • I’m fine now
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Right Left Visual acuity (cc) 20/20-2 20/20-1 Color 6/6 6/6 Pupils Normal Visual Fields Normal Neuro-Exam Normal Optic nerves Normal

EXAMINATION

Alright, you can go. Please get a CT head and carotid ultrasound done later this week!

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Right Left Visual acuity (cc) 20/20-2 Count Fingers Color 6/6 Unable Pupils Left RAPD Visual Fields Normal Diffuse loss Neuro-Exam Normal

NEXT DAY

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TRANSIENT MONOCULAR VISION LOSS

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MANAGE AS A TRANSIENT ISCHEMIC ATTACK OR MINOR STROKE

  • Neuroimaging
  • Vascular imaging
  • Cardiac evaluation
  • Risk Factor Management
  • Anti-platelet therapy
  • Hypercoaguable work-up

TRANSIENT MONOCULAR VISION LOSS

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DOUBLE VISION

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HISTORY OF PRESENT ILLNESS

  • 42 year old Caucasian Male
  • “2 weeks ago I began to see double side-

by-side. Its worse looking far away and to the right. But its better now. Now I only notice it when i’m really looking in the

  • distance. Up close is much better.”
  • Some headaches when I lay flat
  • Ya, I guess when I sleep I hear my heart

beating– but that’s been going on a while

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Right Left Visual acuity (cc) 20/20 20/20-1 Color (HRR) 6/6 6/6 Pupils Normal External Examination Normal

EXAMINATION

  • 0.5

RIGHT LEFT

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DISCUSSION

  • Isolated 6th nerve palsy
  • No brainstem / long tract signs
  • No adjacent CN affected
  • No Horners
  • No optic nerve swelling
  • No Diabetes or vasculopathy
  • Young
  • No Trauma
  • Young 6th’s (<50)
  • 33% Intracranial Tumor
  • 24% Demyelinating
  • 9% Post-viral
  • 7% IIH
  • 7% Meningitis
  • Older 6th’s (>50) + Vascular RF
  • Microvascular most common!!
  • Don’t forget GCA!
  • Should resolve over 3 months
  • Follow closely to ensure

improvement

  • Not BILATERAL
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Courtesy of M. Amans MD.

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THINK TWICE ABOUT A YOUNG 6TH

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60 yo F diagnosed with left sided Bell’s palsy and sinusitis 4 days prior PmHx: Alcohol abuse Treated with 1 week course of steroids and antibiotics . HbA1c 10.7% Starts to develop numbness on her left cheek and develops double vision 2 days later, loses vision in the left eye

HISTORY OF PRESENT ILLNESS

Courtesy of Z. Haq, MD.

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EXAMINATION

Right Left Visual acuity (cc) 20/20 NLP Color 6/6 None Visual Fields Full None Pupils Left RAPD Motility Normal Ptosis and Ophthalmoplegia Cranial Nerves Normal Decreased sensation in V1, V2 Left LMN 7th Poor hearing

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3 days prior 2 days prior 1 day prior

Courtesy of Z. Haq, MD.

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MR BRAIN + MRA WWO

Axial T1 Fat Suppression Relatively diminished enhancement of left orbital contents Axial T2 FLAIR Signal abnormality involving the left ifrontal lobe

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POD#1

POD#2

Dusky gray tissue Web-like mold

Pupil OD 3 mm and non- reactive + Right-sided hemiplegia ↓ Complete occlusion of left internal carotid artery 2/2 infectious thrombophlebitis Multifocal MCA/PCA watershed infarcts

Courtesy of Z. Haq, MD.

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Courtesy of J. Crawford, MD.

H&E High magnification

Filaments Non-septate hyphae Wide angle branching

MUCORMYCOSIS

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Courtesy of J. Crawford, MD.

Angioinvasion

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INVASIVE FUNGAL SINUSITIS DIAGNOSTIC CONSIDERATIONS

Signs and symptoms overlap with many other processes

  • Maintain a high index of suspicion in immunocompromised patietns

Nasal endoscopy (NOT sensitive): pallor +/- frank necrosis +/- eschar Imaging: MRI is more sensitive than CT ↑ tissue contrast enhancement (CE): active infection with inflammation ↓ tissue contrast enhancement (LoCE): devitalization and necrosis Histology (frozen sections): PPV ~ 100%, NPV = 50 to 72% Culture (speciation): only positive in 55 to 67% of histology-positive IFS cases

Kalin-Hajdu et al. Invasive fungal sinusitis: treatment of the orbit. COO. 2017. 28:522-533.

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THERAPEUTIC CONSIDERATIONS

Kalin-Hajdu et al. Invasive fungal sinusitis: treatment of the orbit. COO. 2017. 28:522-533.

Initiation of systemic anti-fungal medication and consider intraorbital antifungals Zygomycetes: liposomal amphotericin-B Aspergillus: voriconazole Endoscopic debridement of necrotic sinonasal tissue Low-risk procedure that confers improved survival (large case series) ↓ fungal load and ↑ access for medication and host immune system Reduce immune suppression when feasible Readily reversed in DM with control of hyperglycemia Hyperbaric oxygen?

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PROGNOSIS

Mortality = 50.3% (based on largest review to date) Negative factors Advanced age Low absolute neutrophil count (< 500/𝛎l)* Zygomycetes* Orbital involvement (50 to 60%)* Intracranial extension Positive factors DM Early detection with disease isolated to the nasal cavity Sinus debridement

Kalin-Hajdu et al. Invasive fungal sinusitis: treatment of the orbit. COO. 2017. 28:522-533.

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CASE

  • 65 yo Man
  • Acute onset headache, blurred vision, double vision
  • Labile blood pressure
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A UNILATERAL TEMPORAL VISUAL FIELD CUT = CHIASMAL LESION!

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FUNDI

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CAVERNOUS SINUS SYNDROME

  • Cavernous sinus contains:
  • CN III, IV, V1, V2, VI
  • Sympathetic fibers to eye
  • Internal carotid artery
  • Signs & Symptoms:
  • Ocular motor palsies (single or multiple; uni or

bilateral)

  • Severe headache
  • Numbness in V1 and or V2
  • Disturbance of vision (optic nerve or chiasm

which run ABOVE the cavernous sinuses)

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DDX OF CAVERNOUS SINUS SYNDROME

  • Carotid-cavernous fistula
  • Cavernous Sinus Thrombosis
  • Infection (Fungus: Mucor, Rhizopus)
  • Pituitary tumor or apoplexy
  • Tolosa Hunt Syndrome
  • Nasopharyngeal ca (Southern China)
  • Metastatic ca, lymphoma
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PITUITARY APOPLEXY

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PITUITARY APOPLEXY

  • Headache/Neck Pain
  • Photophobia
  • Nausea/Vomit
  • Ophthalmoplegia
  • Bilateral vision loss
  • Alteration of

consciousness

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PITUITARY APOPLEXY

  • May be the initial presentation of a pituitary tumor
  • May be precipitated by:
  • Trauma
  • Radiation
  • Anticoagulation
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MANAGEMENT

  • Transfer patient to Neurosurgery & Neuro ICU
  • Initiate IV steroids (life saving)
  • Monitor electrolytes closely
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  • Not all Optic Neuritis is made equal
  • Consider NMO/MOG in atypical cases
  • Amaurosis Fugax and Ocular Ischemia should be managed as a minor stroke / TIA
  • Don’t forget GCA!
  • Think twice about a young 6th
  • If the MRI doesn’t match the ophthalmoplegic patient – check again
  • Think Fungus!
  • A Temporal Visual Field Defect = Optic Chiasm
  • Even if it’s just in one eye

Conclusions

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