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 neuro- ophthalmic emergencies • Have fun!!

Take Home Points • 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 6 th • 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

NEURO-OPHTHALMOLOGY: X MARKS THE SPOT! Divided into Afferent and Efferent Systems ALL ABOUT LOCALIZATION!

Neuro-Op in a Nutshell LOCALIZATION OF AFFERENT DYSFUNCTION • Globe / Retina • Optic Nerve • Chiasm • Optic Tract • Optic Radiations

LOCALIZATION EFFERENT DYSFUNCTION Muscle Junction Nerve ­ Orbit ­ Orbital Apex ­ Cavernous sinus ­ Subarachnoid space Brainstem • Nucleus • Internuclear Supranuclear

ACUTE VISION LOSS

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"

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

VISUAL FIELDS

2 DECADES SINCE THE ONTT What Has Changed?

LET’S CHANGE THE STORY

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

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

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

NEXT DAY 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

TRANSIENT MONOCULAR VISION LOSS

TRANSIENT MONOCULAR VISION LOSS MANAGE AS A TRANSIENT ISCHEMIC ATTACK OR MINOR STROKE • Neuroimaging • Vascular imaging • Cardiac evaluation • Risk Factor Management • Anti-platelet therapy • Hypercoaguable work-up

DOUBLE VISION

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

EXAMINATION Right Left Visual acuity (cc) 20/20 20/20-1 Color (HRR) 6/6 6/6 Normal Pupils External Examination Normal 0 0 0 0 0 0 -0.5 0 0 0 0 0 0 0 0 0 LEFT RIGHT

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

Courtesy of M. Amans MD.

THINK TWICE ABOUT A YOUNG 6TH

HISTORY OF PRESENT ILLNESS 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 Courtesy of Z. Haq, MD.

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

3 days prior 2 days prior 1 day prior Courtesy of Z. Haq, MD.

MR BRAIN + MRA WWO Axial T1 Fat Suppression Axial T2 FLAIR Signal abnormality involving Relatively diminished enhancement of the left ifrontal lobe left orbital contents

POD#2 POD#1 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 Dusky gray tissue Web-like mold Courtesy of Z. Haq, MD.

MUCORMYCOSIS Filaments Non-septate H&E hyphae High magnification Wide angle branching Courtesy of J. Crawford, MD.

Angioinvasion Courtesy of J. Crawford, MD.

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.

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

PROGNOSIS Mortality = 50.3% (based on largest review to date) Negative factors Advanced age Low absolute neutrophil count (< 500/ 𝛎 l)* 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.

CASE • 65 yo Man • Acute onset headache, blurred vision, double vision • Labile blood pressure

A UNILATERAL TEMPORAL VISUAL FIELD CUT = CHIASMAL LESION!

FUNDI

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)

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

PITUITARY APOPLEXY

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

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

MANAGEMENT • Transfer patient to Neurosurgery & Neuro ICU • Initiate IV steroids (life saving) • Monitor electrolytes closely

Conclusions • 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 6 th • 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