Neurological Eye Disease

1. Neurological Eye Disease Kelli Shaon, O.D.

2. Pupil Testing • Are pupil equal in size or is there anisocoria? • If unequal? Is the difference in size… • Greater in light • Greater in dark • Similar both in light and dark • Is convergence intact? • Need to assess it APD is present • http://www.richmondeye.com/apd.asp

3. APD testing in 1 Reactive pupil • Upper series • Left. Pupils in dim light. • Middle. A normal response is seen when light shown in the OD • Right. When light is presented to OS the right pupil dilates. This is (+) for an APD. This indicates a probable optic nerve or retinal lesion. • Lower series • Left. Pupils in dim light. • Middle. A normal response is seen when light is shown in the OD • Right. When light is presented to OS the right pupil does not change size or re-dilate. There is no APD.

4. Pupils • Horner’s • Decrease in sympathetic innervation – miotic pupil • Adie’s Tonic pupil • Parasympathetic paralysis – dilated pupil • Pupillary Light-near dissociation • Argyll Robertson Pupil – miotic pupil • Parinaud’s Syndrome (Dorsal Midbrain) • Bilateral – Mid-dilated pupils • Light-near dissociation • Associated Supranuclear Upgaze palsy

5. Horner’s Syndrome • Disturbance in the sympathetic pathway • Central: Hypothalamus to the 1st synapse in Ciliospinal center of Budge, (C8-T2?) of the spinal cord • Cause - Stroke, MS, Vertebral artery dissection • Pre-ganglionic: Over the apex of the lung to 2nd synapse in the neck at the Superior cervical ganglion • Cause – Lung tumor, Neck SX (trauma), Breast tumor, thyroid adenoma • Post-ganglionic: from the neck through the cavernous sinus (near the carotid) to the muscle of Mueller • Carotid artery dissection, Tumor/Surgery of neck, cluster or migraine headaches, Cavernous sinus inflammation • Triad: miosis, ptosis, anhydrosis(post-ganglionic) • Miosis >> in dim illumination (doesn’t dilate normally)

6. Horner’s Syndrome • Cocaine test 2%-10%: • If no dilation/less than normal: (+) result • If dilation present: (-) result • Hydroxyamphetamine 1% (Paradrine): • Used to distinguish Central/Pre-ganglionic from Post-ganglionic lesion • Central/Pre-ganglionic – will dilate because NE is released from nerve endings • Post-ganglionic lesion – will NOT dilate because no NE is released • Only 40-97% sensitive is determining location Need to wait a minimum of 24-48 between performing the different tests

7. Horner’s Management • Work-up • Take a good history • Headaches, surgery/trauma, pain (neck), etc. • CBC with diff • CT scan of chest – to evaluate for apical lung tumor (Pancoat’s tumor) • MRI of brain & neck • Carotid doppler ultrasound vs MRA of head/neck

8. Adie’s Tonic Pupil • Dilated pupil > in bright lights (doesn’t constrict) – pt may be photophobic • Minimal or no Reaction to light • May become smaller over time • Slow, tonic constriction to convergence with (slow redilation) – blurred near vision • May notice vermiform (sectoral) movement of iris • More common in younger women>men • May have absence of deep tendon reflex (Adie’s Syndrome)

9. Adie’s Tonic Pupil • Benign condition, but need to r/o out other causes of light-near dissociation • Argyll-Robertson pupil (usually extremely miotic) • Parinaud’s Syndrome (Bilateral; convergence normal (not tonic), Paralysis of upgaze common • Etiology: idiopathic, viral, trauma, or diabetes mellitus • Pilocarpine supersensitivity 0.125% - will constrict a Adie pupil (will not constrict a normal pupil)

10. Argyll-Robertson Pupils • Bilateral • Small (often very small) • Poor/if any reaction to light • Normal convergence • Etiology: Neuro-syphilis (tertiary) • Exam- look for other signs (interstitial keratitis, salt-n-pepper fundus, papillitis, uveitis • Work-up: VDRL (RPR) & FTA-ABS (MHA-TP) & consider lumbar puncture to look for Spirochete in CSF

11. Parinaud’s Syndrome • AKA: Dorsal midbrain syndrome • Bilateral • Light-near dissociation • Normal (not tonic) convergence • Mid-dilated pupils • Inability to look up (supranuclear palsy) • Convergence retraction nystagmus • Pineal tumors, hydrocephalus, and MS are main causes • Need to obtain MRI/CT is suspected

12. Diplopia & Motility deficits • CN III palsy • CN IV palsy • CN VI palsy • Multiple Cranial nerve Palsies – Cavernous sinus syndrome

13. Diplopia • Questions to ask : • Does it go away when covering either eye • Monocular (Ref. Error, Corneal, Cataract, Retinal) • Binocular (Most often EOM &/or neurological) • Vertical vs. horizontal vs. oblique • Onset – gradual vs. rapid • Pain associated or not • Is the double vision slightly separated or extensive? • Small vs. Large angle • Is it worse when looking in other directions? • Comitant vs. Non-comitant

14. CN III Palsy • Innervates SR, IR, MR, IO, and levator • Superior division: SR & levator • Inferior division: IR, MR, IO,& also carries pupillary fibers to the iris sphincter • Can have external (motility impaired) or internal ophthalmoplegia (pupil affected) • Need to describe if complete or partial CN III palsy & if pupil involved or pupil-sparing

15. CN III Palsy • Diplopia will vary depending on involvement • Complete Palsy – eye is down & out • Superior division palsy – inability to look up with ptosis • Inferior division – inability to look adduct or look down (pupil may be involved) • Pupil-involved – fixed, dilated pupil • Pupil-sparing – normal reaction

16. CN III Palsy • Pupillary fibers run peripheral of the nerve • Less likely affected in microvascular etiology (DM,HTN, Hyperlipidemia) • More likely affected if compressive lesion or aneurysm pressing on the nerve & pupil fibers • Painful Oculomotor palsy involving the pupil is usually due to aneurysm (often PCOM) • Complete Oculomotor palsy that spares the pupil is usually caused by diabetic neuropathy.

17. CN III Palsy • Management: • Pupil involved – STAT MRI &/or MRA • Pupil sparing – if associated vascular disease, can monitor closely or may still want to get MRI • R/O out MG (with Tensilon test) • Need to r/o GCA, order ESR & CRP

18. CN IV palsy • Innervation of SO • Involved eye will be hypertrophic • Diplopia is oblique • May have head tilt to contralateral side of affected eye with small chin tuck • Only nerve to exit brainstem dorsally, so is highly susceptible to head trauma • Other etiologies: Vascular, infection, and aneurysm

19. CN IV palsy • Work-up • Isolate with Parks 3 step test or Maddox rod test • Ask about history of head trauma • R/O out MG & GCA (is suspected) • Check BP, blood sugar (if vascular suspected) • Consider MRI if younger patient with no history of head trauma or if it does NOT resolve within 3 months • Management: • If newly acquired: Follow-up q 1-3 months (based on etiology) • If small angle & stable – prisms can be used • Occlusion patch can be used to relieve symptoms (if no risk of amblyopia)

20. CN VI palsy • Innervates Lateral rectus • Longest intracranial course • Horizontal diplopia • Esotropia • Limited abduction • May have head turn to ipsilateral side • Etiology: Vascular**, trauma, inflammatory/infection, tumor, congenital

21. CN VI palsy • Work-up & Management: Same as CN IV palsy • Congenital CN IV palsy: • Duane’s (Type I): - • limited abduction (normal adduction), globe retraction on adduction, and narrowing palpebral fissure on eye movement • Mobius syndrome: • absence or underdevelopment of the 6th and 7th cranial nerve. So will lack facial expression and will have esotropia (often bilateral)

22. Multiple Ocular Nerve Palsies • Cavernous Sinus Syndrome • On either side of the pituitary gland • CN III, IV, and V are in the lateral wall • CN VI run through the body • Internal carotid runs centrally

23. Multiple Ocular Nerve Palsies • Cavernous Sinus (CS) Syndrome • Mass lesions (tumor or aneurysm) within the cavernous sinus often produces multiple CN involvement, pain (CN V), possible Horner’s syndrome • Etiology: Meningioma, Pituitary apoplexy, Metastatic lesions, Arteriovenous fistula, intracavernous aneurysm, mucomycosis (ie. Diabetics), cavernous sinus thrombosis • Management: STAT CT/MRI

24. Multiple Ocular Nerve Palsies • Superior Orbital Fissure Syndrome (Tolosa-Hunt Syndrome ) • Location is at the most anterior border of CS • Similar signs as Cavernous Sinus (CS) Syndrome, except much more painful unilateral ophthalmoplegia • Etiology: granulomatous infiltration of nerves and muscles • Orbital Apex Syndrome • Location is in orbital apex • Proptosis is common • Same CN involved but also optic nerve involvement common – must suspect if Optic neuropathy with multiple CN palsies

25. Intranuclear Ophthalmoplegia (INO) • Lesion at the MLF • Adduction deficit (on ipsilateral side) • Abduction nystagmus (in contralateral eye) • Often associated with what disease?

26. 1 ½ syndrome • Lesion at the PPRF or the sixth nerve nucleus AND at the MLF • Ipsilateral gaze palsy with a contralateral INO • Ipsilateral eye can not adduct or abduct (no lateral movement) • Contralateral eye can ONLY Adduct • MLF damage results in – • No ipsilateral adduction (no MR innervation) • Abducens nucleus damage results in – • No ipsilateral abduction (no LR innervation) • No contralateral adduction (no MR innervation)

27. Myasthenia Gravis (MG) • Causes by antibodies interfering with acetylcholine receptors – not allowing nerve signals • Is a systemic condition • Symptoms/Signs: • Ptosis (worse at then end of the day) • Diplopia (worse at then end of the day) • Cogan’s lid twitch – (upward overshoot lid twitch when shifting from inferior to primary gaze) • Facial muscle weakness • Limb weakness • Difficulty swallowing or breathing*** (life threatening) • Change in voice

28. Myasthenia Gravis (MG) • Testing/Management: • Tensilon (endophonium) IV – will cause reversal of lid & motility disorders immediately • Ice test – Ice pack over ptotic lid for ~2 min will improve ptosis • Rest test (sleep test) – keep eyes close for 20 minutes or take small nap and often improves • Consult with PCP to have Ach receptor antibody essay test performed & systemic management

29. Common VF defects • Hemianopia or quadranopia • “PITS” • More congruous – closer to occipital lobe • Closer to occipital tip – closer to macula (more field involved) • Deeper in occipital lobe – more peripheral VF involvement • More incongruous – more anterior in visual pathway • Bitemporal defects • Chiasmal • Altitudinal • Monocular – ION • Bilateral – bilateral occipital lobe lesions