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Neuro- 101 Anisocoria case studies

William L Hills, MD, OD Neuro-ophthalmology Casey Eye Institute OHSU Portland, OR Objectives

W Review pupillary pathways W Review exam W Discuss several cases of anisocoria W Discuss pharmacologic pupillary testing W Nothing to disclose Pupil Anatomy

W W Stroma W Density and pigmentation determine color W Lisch nodules- pigmented iris hamartomas, NF1 W Sphincter W Innervated by parasympathetic fibers via CNIII W Dilator W Innervated by sympathetic fibers W Pigmented epithelium Pupil Anatomy

W Pupil W Function W In conjunction with the iris controls the amount of light entering the eye Pupillary light reflex

W Afferent limb W Efferent limb W Parasympathetic W Sympathetic Pupillary light reflex

W Afferent limb W W W Optic chiasm W Optic tract W Pretectal nuclei Pupillary light reflex

W Efferent limb W Parasympathetic W Edinger-Westphal subnucleus of CNIII W Pupilloconstrictor fibers superficial aspect of CNIII W Synapse in the ciliary ganglion Pupillary light reflex

W Efferent limb W Parasympathetic W Edinger-Westphal subnucleus of CNIII W Pupilloconstrictor fibers superficial aspect of CNIII W Synapse in the ciliary ganglion Pupillary light reflex

W Efferent limb W Parasympathetic W Edinger-Westphal subnucleus of CNIII W Pupilloconstrictor fibers superficial aspect of the W Synapse in the ciliary ganglion Pupillary light reflex

W Efferent limb W Sympathetic W First (central) neuron W Second (preganglionic) neuron W Third (postganglionic) neuron Pupil Examination

W Normal W Both should constrict symmetrically when a light source is shown to each eye W Direct Response W The constriction of the pupil being presented with light W Consensual Response W The constriction of the pupil NOT being presented with light Pupil Examination

W Size can usually be measured by the sample pupils on a Near Card

http://umed.med.utah.edu/neuronet/lectures/2002/neuro_opthpics/Basics1.jpg Pupil Examination

W Symmetry WAre the pupils the same size? WIf not is the difference greater in the light or in the dark? WThe “problem” pupil is: WThe smaller pupil if the asymmetry is greater in the dark WThe larger pupil if the asymmetry is greater in the light Case 1

W A 43 year old banker W sudden left eye W referred for new left pupillary dilation W No headache, pain, W No PMHx blur, W No trauma Case 1

W BP 160/70 W HR 108 bpm W DVAs W OD 20/20 W OS 20/20 W Color vision W 10/10 Isihara plates each eye Case 1

OD OS

3mm Light 5mm

7mm Dark 8mm brisk rxn sluggish

+ Near +

-- RAPD -- Case 1

W Intrapalpebral W Cover testing distance W OD 12mm 5 pd RH W OS 10mm 2 pd RH 4 pd RH 2 pd RH Tr exo

0 hyper

Dx: Incomplete pupil involving Third nerve palsy Now What??

W Urgent CT/CTA Head W Rule out W Where? W Pcomm W Where else? W Cavernous Sinus

BCSC Neuro-Ophthalmology 1999 Case 2

W 79 year old man W retired accountant W 20 year h/o HTN W 4 yrs of poorly controlled DMII W two weeks ago W sudden onset diplopia W painless W binocular W diagonal W right Case 2

W BP 124/76 OD OS W HR 78 bpm W DVAc 3.5 mm Light 3.5 mm W OD 20/20 -2 W OS 20/25- W color testing 5.0 mm Dark 5.0 mm W 10/10 plates each eye W stereopsis testing Brisk Rxn Brisk W 1/3 animals W 3/9 circles None APD None W Confrontation visual fields W full to finger counting each eye Case 2

W 4 pd LHT Dx: microvascular pupil W variable 6 to 20 pd R XT sparing third nerve W OD palsy W Adduct -4 W Supra -3 W Infra -3 W Abd 0 W DFE: broadened arterial light reflex Case 2: 4 weeks follow up

W 2-4 pd L HT OD OS W variable 18 to 20 pd 5.0 mm Light 3.5 mm XT W complete ptosis 6.0 mm Dark 4.0 mm

Sluggish Rxn Brisk

None APD None Neuroimaging Case 2

Image courtesy of Amirsis Normal Basilar Artery NeuroimagingNeuroimaging CaseCase 22 NeuroimagingNeuroimaging CaseCase 22

Case 3

W 18 yo female CNA W Neuro Exam W Presents to ER W Normal strength W HA W Symmetric reflexes W Holocephalic W Constant W No cerebellar findings W No trauma W ie no W Dilated right pupil x 4 hrs W Blurred vision W Dist and near OU W OD monoc diplopia Case 3

OD OS

7.5 mm Light 4.0 mm

7.5 mm Dark 6.0 mm

NonRx Rxn Brisk

None APD None Case 3

W Do you scan her? W No, if no other neuro findings W Yes, if CNIII paresis, other neuro findings W Urgent CT/CTA “Negative” W 0.125% ou W No change W Pilocarpine 1% ou W OS constricts W OD no change W DX? W Pharmacologic Pupil W She admits to putting scopolomine in OD Case 3

W What if both pupils constricted to 1% pilocarpine? W CNIII palsy W Hi resolution MRI/A W cerebral angio Pharmacologic

W no constriction with 1% pilocarpine W Adie’s reacts to 0.125% pilocarpine W Compressive lesion reacts to 1% pilocarpine W patch W Jimson weed, henbane W Nebulized asthma treatments Case 4

W 29 yo RHWF W 4/2007 W W Recent RN grad Pupil asymmetry W Variable/intermittent W PMhx W OD>OS sometimes W with Aura W 9/2007 W OD consistently

OD OS

4.0 mm Light 5.0 mm

4.0 mm Dark 7.0 mm RLF

NonRx Rxn Brisk

None APD None

RLLF Case 4

W What other pupil testing can be done? Dark at 5 sec W Near W +Near-light dissociation W What else? W Slit Lamp W +Sectorial constriction Dark at 15 sec W Reflexes W Intact W Corneal sensation W decreased Case 4

W What next? W Pilocarpine ou W 0.0125%

Before pilo ou W Dx: W Adie’s Pupil

After pilo ou Rock Star

W 18 yo boy OD OS W Electric guitar player 4.0 mm Light 3.5 mm W Long hair W Wears corduroy pants 5.5 mm Dark 4.5 mm

W Mom recently noted Brisk Rxn Brisk pupils large and different sizes None APD None sometimes Rock Star

W What to do? W Family Album W 2% W Both dilate equally W What is this? W Physiologic Anisocoria W In Dim illumination W 20% of normal population W Anisocoria 0.4mm or more W In bright illum W 10% of normal pop W Anisocoria of 1.0mm or more is rare W Variable Case 6

W 9 yo LH boy W Fall from highchair OD OS age 2 3.0 mm Light 4.5 mm W Asymmetric pupils 4.0 mm Dark 6.0 mm W OD Ptosis in past year W Pupils variable Brisk Rxn Brisk

W Worse when tired None APD None Case 6

RLF RLLF

DF5 DF15 Case 6

W What next? W Family Album W Cocaine ou W 10% W Dx:

W Horner’s Syndrome Before cocaine W What next? W Hydroxyamphetamine W Both pupils dilate W 1st vs 2nd vs 3rd? W 1st or 2nd W Likely congenital W Iris heterchromia W Superior and Inferior lid After cocaine ptosis W Breach? Pharmacologic diagnosis of Horner’s syndrome W Cocaine test W Hydroxyamphetamine Wblocks reuptake of W releases stored NE at n-m vesicles of NE only junction from an intact postganglionic W 2 drops of 10% terminal bouton cocaine W 1 drop of 1% W after 1 hour, a normal hydroxyamphetamine pupil dilates more W Postganglionic than a Horner’s pupil Horner’s pupil does not dilate as well or at all Iopidine () W weak direct action on W strong alpha 2 alpha 1 receptors receptor agonist W minimal to no effect W Decreases the release on normal pupils of NE W HS - denervation W Therefore, will supersensitivity alpha1 constrict normal pupil W responsive to W Reversal of anisocoria apraclonidine W Does not differentiate 1st, 2nd, or 3rd order Freedman KA, Brown KM. Topical aproclonidine in diagnosis of suspected horner syndrome. J Neuro-Ophthalmol 2005;25: 83–85. lesion Trucker Bob

W 66 yo RH retired Trucker W Noted OD dilation W PMHx 6/2007 W Hereditary Hemorrhagic telangectasia W No assoc HA, trauma, W Hypertension pain, diplopia W Left Lung AVM stenting 2004 W Migraine HA W Left sided W Associated L ptosis W No unilateral epiphoria or rhinorrhea Trucker Bob

OD OS

4.0 mm Light 3.0 mm

6.0 mm Dark 4.0 mm Room lights

Brisk Rxn Brisk

None APD None

DirectDirect OD 5 sec darkdark 15 sec dark

40 mins p cocaine Now what?

4040 minsmins p hydroxyhydroxy Pre-hydroxyamphetamine Case 8 OD OS 4.0 mm Light 7.0 mm

W 26 yo OR RN 6.0 mm Dark 8.0 mm W Noted to have dilated left pupil Brisk Rxn Sluggish

W No HA, pain, diplopia None APD None W Vague Blur W DVAs 20/20 each eye W NVAs 20/20 each eye W Orthophoric dist & near OD OS Case 8 4.0 mm Light 4.0 mm

W 2 hours later 6.0 mm Dark 6.0 mm

W She remembers that she Brisk Rxn Brisk has migraine HA W Freq once/month None APD None W What is this??

W B9 Episodic Mydriasis W Scan her? W No Relative Afferent Pupillary Defect

W Swinging Flashlight Test W When light is shown in one eye then IMMEDIATELY moved to the other eye the consensual response is seen in the second eye W Relative Afferent Pupillary Defect W Consensual > Direct

http://commonspot.aao.org/aaoesite/promo/business/images/06_4Techniques2_webfinal.jpg RAPD ƒRelative asymmetric visual input ƒOptic nerve/tract damage ƒMay be caused by severe retinal damage ƒIs not increased by corneal or opacities ƒMay be used to identify worsening or improvement Relative Afferent Pupillary Defect

W Etiologies of RAPD W W W Compressive W Tumor W Aneurysm W Ischemic W NAION W AAION – GCA W Ocular emergency