NEURO-OPHTHALMIC PEARLS

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1 NEURO-OPHTHALMIC PEARLS ROSA ANA TANG, MD,MPH,MBA MS EYE CARE-UHCO N-O Emergencies High Anxiety Level 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] 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. 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. NeuroNeuro-ophthalmic Emergencies Excessive delay in diagnosis Pituitary apoplexy: apoplexy: permanently blind if no intervention Compressive optic neuropathies: neuropathies: mostly if due to pituitary tumors as reversibility is tied to chronicity Myasthenia / thyroid disorders: disorders: due to their systemic associations and health related issues [thyroid storm and myasthenic crisis] Pearl:TOP TEN DIAGNOSES YOU DON DON T WANT TO MISS 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]

2 Pearl: TOP TEN DIAGNOSES YOU DON T WANT TO MISS 6)Pituitary apoplexy: permanently blind if no intervention 7)Progressive visual loss: Compressive optic neuropathies: mostly if due to pituitary tumors as reversibility is tied to chronicity 8)Myasthenia : due to their systemic associations and health related issues [ myasthenic crisis] Neuro symptoms that make us PANIC 1. Acute diplopia: can be a killer [ie: aneurysm/myasthenia] - always make sure what the underlying cause is Pearl:TOP TEN DIAGNOSES YOU DON T WANT TO MISS 9. GCA 10. PAPILLEDEMA Three sx that may cause us to PANIC too!!! 3) Visual field defects [BTH and acute] 4) Painful anisocoria [dissection] 5) Numbness [with or without pain] 2.Papilledema: the cause can kill the patient SIGN: PUPIL ABORMALITY : Anisocoria P =pupil abnormality

3 In a patient with a Unilateral fixed dilated pupil : Anisocoria Normal light reaction Abnormal light reaction physiologic Adie s tonic Horner s 3rd nerve palsy pharmacologic sphincter damage LOOK FOR : ANY HINT OF III CN PARESIS OR PTOSIS. LIGHT-NEAR DISSOCIATION OF PUPIL : TONIC PUPIL EVIDENCE OF ANT. SEGMENT TRAUMA IN SLIT LAMP EXAM. Dilation lag: Question : what if there is greater anisocoria in dark In light Anisocoria w/ dilation lag In dark in 1st 5 seconds P= pupil abnormality In Dark after seconds How to localize the lesion in Horner s syndrome Localization of the lesion according to the symptoms in Horner s Syndrome First-order neuron lesions Historical : based on associated symptoms. Clinical: Based on associated signs. Based on pharmacological testing. Second-order neuron lesions Hemisensory loss Preceded by trauma Dysarthria Facial, neck, axillary, shoulder or Dysphagia Third-order neuron lesions Include diplopia from sixth nerve palsy arm pain Numbness in the distribution Ataxia Cough of the first or second division of Vertigo Hemoptysis the trigeminal nerve Nystagmus History of thoracic or neck surgery Pain. History of chest tube or central venous catheter placement Neck swelling. HISTORICAL DIAGNOSIS OF HORNER S

4 Horner s Etiology 50% idiopathic 50% secondary Syndromes: Lateral medullary or Wallenberg: central Cavernous sinus: post-ganglionic +VI CN ipsilateral Apraclonidine (0.5% or 1%) Iopidine Apraclonidine is an ocular hypotensive agent. It is a weak, direct-acting alpha-1 receptor agonist. Apraclonidine has little to no effect on a normal pupil size. Pharmacological :How to confirm Horner s syndrome-cocaine testing Cocaine blocks the re-uptake of the neurotransmitter Norepinephrine in presynaptic terminal causing dilation of the normal pupil In Horner s: no dilation of the tested pupil is seen with cocaine. This is due to lack of Norepinephrine molecules on the pre-synaptic vesicle : there is nothing to block. Tells us THERE IS A Horner s but not the level of the lesion. APRACLONIDINE 0.5 % Should eliminate or reverse the anisocoria [small Horner s pupil becomes larger and normal stays same size]. Reverses the ptosis. Read after 30 minutes of instillation. Where is the Horner s lesion- Paredrine (Hydroxyamphetamine) Test Where is the Horner s lesion- Paredrine (Hydroxyamphetamine) Test 1. Hydroxyamphetamine releases norepinephrine from the stores in the post-ganglionic neuron causing dilation of the pupil. 3. First and second order neuron dysfunction-no effect of Paredrine as this substance only works at the level of the third order(postganglionic neuron) hence pupil DILATES with paredrine as the normal pupil does.

5 Congenital Horner s Syndrome Heterochromia with LIGHTER IRIS in Horner s eye most distinct feature Normal pigmentation of iris depends on the sympathetic innervation 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 painful Horner s P=pupil abnormality Etiology of Post ganglionic Horner s 1. Internal carotid A. dissection - Acute Unilateral headache or facial pain. 2. Cluster headache- Transient postganglionic Horner s syndrome with episodes of excruciating hemicranial headaches. 3. Trauma- base of skull fractures 4. Cavernous sinus Lesion SIGN: PUPIL MOST IMPORTANT OBJECTIVE PUPIL SIGN IN UNILATERAL VISUAL LOSS CASES SIGN: DISC EDEMA SIGN : corneal anesthesia In the absence of corneal disease What does this means? IF BILATERAL: MUST EXCLUDE PAPILLEDEMA

6 Increased ICP Swollen optic nerves Bilateral ON swelling is likely due to increased ICP First investigation should be imaging with brain MRI to exclude brain tumor Increased Intracranial Pressure with abnormal Imaging and/or CSF (other than high pressure) MOVIE pneumonic:to be excluded FIRST!!!!!!!!!!!! while monitoring VA/fields M Mass/Meningitis O Obstructive Hydrocephalus V Venous Hypertension I Infectious Causes (Abscess/Meningitis) E Pearl The diagnosis of pseudotumor cerebri is a diagnosis of exclusion This is based on a specific diagnostic criteria as follows: Hx: no sx other than HA, tinnitus,dv,tvo. Exam :disc swelling normal BP Normal MRI and CSF except for high pressure Japanese Pearl!! BILATERAL DISC EDEMA ALWAYS CHECK THE BLOOD PRESSURE AS MALIGNANT HYPERTENSION CAN PRESENT WITH THIS FUNDUS PICTURE E dema NEDS2001 (Non Infectious meningeal ) Modified Dandy criteria of IIH Ref: Friedman &Jacobson : Neurology :59: ,2002 Symptoms and signs of increased ICP Otherwise normal neurologic exam Normal level of alertness Neurodiagnostic normal except elevated ICP No other cause of increased ICP present. Secondary Pseudotumor syndromes All imaging negative, including MRI, MRA, MRV, Angiogram, CAT scan Looks like primary pseudotumor cerebri but there is something else that may be precipitating it.

7 Typical Patient:- PTC Clinical Associations Obese female of childbearing age General population: 1 : 100,000 SIGN: DISC EDEMA-WHAT ABOUT THIS PICTURE? Obesity Recent weight gain Pregnancy? Women who are 20% greater ideal body weight: 19.3 : 100,000 Female : Male 8:1 ODEMS type I NEURORETINITIS UNI OR BILATERAL ODEMS -ATYPICAL Vascular entities AION: rare Papilledema-CHRONIC Malignant Hypertension: bilateral TYPICAL : Associated to infections Viral Syphilis Cat Scratch Lyme s TB Toxoplasma /Toxocara Idiopathic Pearls True ODEMS is idiopathic, often with optic disc edema as the presenting sign & 2 weeks later the star follows ODEMS IS NOT SEEN PATIENTS. IN MS Neuroretinitis with specific etiologies should be treated appropriately Pearl Every new patient c/o blurry vision and you cannot correct to 20/20 OR you find elevated discs should have at least confrontation VF & pupil check for RAPD Automated perimetry for those who have lots of Sx and no findings.

8 SIGN: PROPTOSIS MOST COMMON CAUSE UNI OR BILATERAL : THYROID IN CHILDREN UNILATERAL AND ACUTE-THINK ORBITAL CELLULITIS IF PULSATILE TINNITUS/BRUIT THINK CCF TRIO TYPE II EOM enlargement marked /asymmetric with myositis and restrictive myopathy : IR & MR most common involved : can t look up. (IR>MR>SR>LR) With restrictive myopathy the eye is pulled in the direction of the involved muscle. Goals of TreatmentThyroid Protection of visual acuity Control of inflammation Correction of muscle dysfunction Reduction of proptosis Improvement in cosmetic appearance TRIO TYPE I Occurs most often in women. Symmetric proptosis. Symmetric eyelid retraction. Minimal orbital inflammation. Minimal or no myopathy, however EOMS may be large due to edema not myositis. Corneal exposure may be considerable. Clinical PresentationsThyroid Mild orbitopathy Moderate Optic orbitopathy neuropathy TRIO-TREATMENT Localized protective/lubrication. Medical anti-inflammatory: high dose ( of prednisone QD) for few weeks. Medical : orbital radiation is preferred for patients over age 55 ( Ref: Martin &Corbett ). Surgical for visual loss :orbital decompression if medical treatment fails. Surgical for motility/lid : only when orbital findings stabilize. Radiotherapy : less and less likely to be of any help

9 Pituitary tumors and the optometrist Pituitary tumors in adults present a wide spectrum of symptoms and physical findings many of which affect vision. Visual symptoms are gradual in onset due to the benign histopathology of these tumors and its location. Pituitary tumor treatment PITUITARY GLAND TUMOR CLINICAL PRESENTATION Hormone Mass effect Galactorrhea/ Amenorrhea Acromegaly Cushing s Hypopituitarism Neuro-ophthalmologic ( vision loss, diplopia) Cerebrospinal fluid (CSF) leak In pituitary disorders Goals of pituitary tumor treatment : control of tumor growth Routine visual field examinations after treatment: 1st year every 3 months 2nd to 5th year every year Every 2 years there after normalize pituitary function preservation or restoration of visual function. PITUITARY APOPLEXY TRIAD SEVERE HEADACHE WITH SX OF SAH ACUTE DIPLOPIA : III OR VI BUT TOTAL OPHTHALMOPLEGIA MOST LIKELY VISION LOSS /BTH Savino et al Sign: PTOSIS 1.Isolated ptosis with no DV and normal pupils is SELDOM an emergency Ocular myopathy [MG] rarely presents emergently if there is only ptosis unless can t swallow or breath-then we are in trouble.

10 Purely Ocular Myasthenia Initial presentation of MG in up to 70% Ocular precedes clinically generalized MG in % of patients. Laboratory tests in MG Anti-acetylcholine receptor binding antibodies should be measured Positive in 50 % of Ocular MG and 90% of Systemic ( Generalized MG) Usually generalizes within 2 years of onset of ocular symptoms Less common antibody:musk Seen in Ach Receptor Ab negative myasthenia. Can be seen with ocular myasthenia but rarely. Worse prognosis in regards to systemic symptoms. M.G.-Management -Adjustments Change medications that can exacerbate or cause Education (rest, pacing, diet, temperature, stress) For ocular (ptosis crutches, prisms, patching) SIGN: PTOSIS Acute double vision <2 mm: Horner s - tip: look for brother s kiss sign [narrow fissure] and miosis >2 mm: Mechanical: isolated [lev dehiscence] Myopathic: variability= MG Diplopia: look for IIIrd CNP Beware of calling decompensated strabismus any case that presents with acute diplopia and no clear cut CN paresis.

11 Acute Painful Diplopia Aneurysm about to Rupture Dissection VB- about to happen Pituitary Apoplexy- shock for lack of steroids Mucormycosis Orbit and Cavernous Sinus Basilar Meningitis- TB, crypto Giant Cell arteritis Tolosa Hunt : ALWAYS a Dx of Exclusion Pearl All pts with PARTIAL IIIRD CN PARESIS whether or not the pupil is involved need URGENT imaging to exclude an aneurysm Isolated CN paresis When is ischemic or microvascular? MONONEURITIS III CN paresis and the rule of the pupil SIGN: PTOSIS WITH FELLOW TRAVELERS [EOM+PUPIL] INVOVEMENT Should be truly ISOLATED so need Neuroophthalmic 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 non vasculopath. Should resolve in 3 months (90 day palsy). Don t forget Giant Cell Arteritis as a cause in elderly. Key Diagnostic/Management Issue: Is it GCA? Sign: T for TEMPORAL ARTERITIS Jaw Claudication/ HA: high risk Transient 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

12 GCA-OPTIC NERVE INVOLVEMENT Optic Nerve: (a) ION:AAION AION: Anterior with markedly pallid GLOBAL edema. (b) Cup-to-disc ratio greater than 0.2 in fellow eyes. PEARL -HARBOR HOW TO HELP DIAGNOSING GCA Clinical suspicion : the most important one. Laboratory markers : -WESR - CRP high platelet count Arteritic Ischemic Optic Neuropathy (Temporal Arteritis) Don t miss GCA: think of it on everyone that is >50 yr old with: Transient LOV one eye Transient /PERMANENT diplopia AION if disc white more likely Cilio retinal artery occlusion Tonic pupil one eye in elderly Isolated CN paresis NAION Linked to: AMIODARONE VIAGRA/CIALIS INTERFERON RISK FACTORS: Cupless disc DM,HBP,Lipids+ Smoking Spinal surgery Sleep apnea Hyperhomocysteinemia in young pt Give Steroids IMMEDIATELY PEARL-NEURO OPH EMERGENCIES LIFE THREATENING 1)Double vision due to third nerve palsy due to aneurysm 2)Bilateral disc swelling due to brain tumor herniating or venous thrombosis causing stroke 3) Acute bilateral /unilateral ophthalmoplegia from pituitary apoplexy

13 PEARL-NEURO OPH EMERGENCIES BILATERAL SIGHT THREATENING Acute LOV from pituitary apoplexy or from GCA.[ ON involvement] Acute HH from stroke related to CAROTID DISSECTION UNILATERAL SIGHT THREATENING Acute LOV in GCA CRAO from embolic disease or GCA LAST PEARL-THREE COMMON MISTAKES THAT CAN LEAD TO PERMANENT BLINDNESS OR DEATH ARE: 1) Not suspecting the possibility of serious orbital or brain disease as the cause of the patient s eye complaints. 2)Not performing a careful and thorough history and examination. 3)No referring pts for Consultation EARLY and URGENTLY when needed