neuro eye disease grand rounds - afos2020.org
TRANSCRIPT
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Neuro Eye Disease Grand Rounds
Eric E. Schmidt, OD, FAAOOmni Eye SpecialistsWilmington, NC
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The Neuro Eye Exam: History
l Most important part of the examl Vital statisticsl Chief complaint – Clarify, Qualify, Quantifyl Medical historyl Social historyl Medications
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The Neuro Eye Exam: Examination“The hunt begins!”
l Stop, look and listenl Visual acuity – Remember the pinhole!l Amsler gridl Red cap testl Color visionl Pupils, pupils, pupils
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Examination – “The hunt continues”
l EOM testing– Normal– Forced duction
l Confrontation fieldsl Facial AGl Refractionl Anterior segmentl Optic nerve head evaluationl Retinal Other tests- CT/MRI, lab tests, perimetry
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The Neuro Eye Exam
l Diagnosis – “The 90% Solution”
l Management – “Making The Arrest”
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The Case Of The Chubby Disk
l 29 y/o WF cc: blurred vision OS, OS seems to be “pulling” for last 3 wks– HA on L side– Feels like pressure (a suction cup) on OS– Seems like a skim over OS
Meds: nonePast hx of “migraines”
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Chubby exam
l VA – OD 20/25, OS 20/20 (blurry), poor endpoint on refraction
l PERRL mg (-)l EOM – no restrictionl SLE – wnl OUl IOP- 20mm OD, OSl Fundus – as shown
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Chubby disk, Question 1: What is the most correct diagnosis?
l 1. Papilledemal 2. Bilateral disk edemal 3. Pseudotumor cerebril 4. Brain tumorl 5. Optic disk drusen
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Disc edema – differential diagnosis
l Intracranial tumorl Elevated ICP – Papilledema, PTCl Vascular/Ischemic – IONl Inflammatory – Optic neuritisl Systemicl Orbital tumors
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Papilledema
l Bilateral disk edemal NFL opacification and hemesl Hazy retinal vesselsl Paton’s linesl (-) SVPl Disk hyperemia, exudates and CWS
l Papilledema is always caused by increased ICP!!
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Chubby disk, question 2: What is your next move?
l 1. CT Scanl 2. ESR/C-Reactive proteinl 3. Lumbar puncturel 4. MRIl 5. Refer to Jim Thimons!!l 6. Visual field
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Given the VF result would you next?
l 1. CT Scanl 2. ESR/ C-Reactive proteinl 3. Lumbar puncturel 4. MRIl 5. Refer to Oh Great One!!!
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Chubby disk, question 4:
l MRI showed no mass lesion, but partial empty sella – What are you going to do now?– 1. ESR/C-Reactive protein– 2. Neurology referral– 3. Neuro-eye referral– 4. Lumbar puncture– 5. Prednisone 80mg daily– 6. Diamox 500mg po BID
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The Hunt For The Cause
l MRI – Partial empty sellal LP – Opening ICP 402
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Chubby question 5: Now what is the diagnosis?
l 1. Meningitisl 2. Benign Idiopathic Intracranial
Hypertensionl 3. Pseudotumor cerebril 4. Viral encephalitis
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Chubby question 6: How are you going to treat this?
l Acetazolamide 250mg BIDl Acetazolamide 500mg sequels QDl Prednisone 60mg QDl Serial Lumbar puncturesl Topamax 60mg QDl Optic nerve sheath defenestration
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Idiopathic Intracranial Hypertension
l Bilateral disk edema (papilledema)l Due to increased CSFl Tx: Diamox
– Weight loss– Oral steroids– Weight loss– Topamax– Repeat LP– Cerebral shunt– ON defenestration
l WEIGHT LOSS!!, WEIGHT LOSS!!, WEIGHT LOSS!l Lose Weight !*&@*(^^(@!
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Chubby’s sequelae
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The Case of 2 Eggs, 4 Pancakes and 8 Strips of Bacon!
l 70 y/o WM sat down for breakfast when he suddenly experienced horizontal double vision
l Felt very “woozy.” “like I’ve lost my depth perception.”
l Denies paresthesia or weaknessl Meds - ASA
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Examination
l VA OD 20/30 ph NI, OS 20/30 ph NIl EOM – OD no restriction
OS no adductionCT – 50pd LXT (OS down and out)
10 pd RH
PERRL mg(-)
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Examination
l Slit – wnl (-)RIl Fundus – D,M,V,P wnl OU
– (-) HR,DR,disc swellingl BP – 150/70l (-) Bruitl Neurologic survey – neg other than EOM
palsyl ?Diagnosis?
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2 Breakfasts. Question 1:
l What is his most accurate diagnosis?– 1. Complete CN3 palsy– 2. Partial CN 3 paresis– 3. Diabetic neuropathy– 4. CN4 palsy– 5. CN6 paresis – 6. Left adduction deficit
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Some Diplopia Rules
l Crossed diplopia – exo deviation (MR)l Uncrossed diplopia – eso deviation (LR)l Worse at distance – lateral musclesl Worse at distance – rectus musclel Worse at near – medial musclel Worse at near – oblique muscle
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The 4 Questions of Diplopia
l 1). Monocular or binocular?
l 2. Horizontal or vertical?
l 3.Is it worse in any direction of gaze?
l 4.Diplopia greater at distance or near?
l Identifies CN 3,4, or 6
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The Rule Of The Pupil
l In all cases of diplopia or ptosis – check the pupil!!!!
– Pupil spared – diabetes– Pupil blown – aneurysm– Pupil miotic - inflammatory
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2 Breakfasts, question 2;
l What is the most likely etiology?– 1. Aneurysm– 2. Diabetes– 3. CVA– 4. Intracranial Tumor– 5. Trauma
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2 Breakfasts, question 3:
l What should you do next?– 1. Carotid ultrasound– 2. Blood work (CBC, FBS, ESR)– 3. MRA– 4. MRI– 5. CT scan– 6. VF– 7. ER referral
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CN 3 Neuropathy
l Horizontal diplopia
l Worsens on contralateral gaze
l Check the lids and pupils
l Can there possibly be a vertical component?
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CN 3 Neuropathy Causes
l Adults– 20% Aneurysm– 20% Vascular– 15% Trauma– 45% Other
l Children– 45% Congenital– 20% Traumatic– 10% Neoplasm– 7% Aneurysm
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CN 3 Management
l <40 y/o– CT Scan– Angiogram– MRA
–
l Remember the rule of the pupil
l >40 y/o– BP, CBC, FBS– RTO daily x 1 week– RTO weekly x 1 mth– RTO monthly x 3 mths
l CT scan if worsening or no improvement
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HOWEVER!!!!
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CN 4 Neuropathy
l Vertical diplopia
l Worsens upon contralateral gaze
l Diplopia worse at near
l Head tilt likely
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CN 4 Neuropathy Causes
l 40% Trauma
l 20% Vascular
l 10% Neoplasm
l 10% Aneurysm
l 20% Unknown
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CN 6 Neuropathy
l Horizontal Diplopia
l Diplopia worsens on ipsilateral gaze
l Diplopia worse at distance
l Most common ocular palsy
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CN 6 Neuropathy Causes
l Vascular (esp if unilateral)l Neoplasml MSl Subarachnoid hemorrhagel Meningitisl Traumal 30% Idiopathicl Bilateral 6th is never from infarction
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CN 6 Neuropathy Management
l Adults– Blood work– CT scan if progressive– Evaluate for increased ICP– Pain as a prognostic sign
l Children – 33% tumors– All kids with acute 6th need MRI
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2 Breakfasts - resolution
l BP – normal
l BS – 117
l CBC – normal ESR – 27mm/hr
l CT scan – massive sinusitis
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So Tell Me Oh Great One, How Did Your Patient Fare?
l CT Scan – Massive sinusitisl Oral Antibiotics x 3 weeksl Refused to cook anymore!
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Singin’ The Blues
l 84 y/o WF referred in for “papilledema”l She had no complaints, no ocular symptoms
and had not noticed any change in visionl CVA 3 yrs priorl Meds: Celebrex, Tylenol
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Vida’s Exam
l VA – OD LP, OS -20/200 ph 20/80l Pupils – OD 6mm, oval, sluggish (+)APD
OS 4mm sluggish responsel SLE OD 1+ stromal haze, PCL
OS PCL, limbal pannus, 3+ inf endo pigment
l Fundus – as shown
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Which is the acutely involved eye?
l 1. Right
l 2. Left
l 3. Both
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What’s Vida’s Diagnosis?
l 1.Optic atrophy OD, papilledema OSl 2.Papilledemal 3.Optic atrophy OD, ION OSl 4.Optic atrophy OD, Optic neuritis OSl 5. CRVO OSl 6.Foster-Kennedy Sxl 7. Brain tumor
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What is the most appropriate next step?
l 1.Disk photos, recheck 1 weekl 2. MRIl 3. FBSl 4. Intravenous Fluorescein angiographyl 5. TA Biopsyl 6. ESR/C-RPl 7. Prednisone 80mg po
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ESR = 89mm/hr , C-RP elevated Now What?
l 1. TA biopsyl 2. Medrol dose pakl 3. Refer to internistl 4. Prednisone 80mgl 5. Refer to neurologistl 6. MRIl 7. IV methylprednisolonel 8. LP
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Now what is the diagnosis?
l 1. Foster-Kennedy Syndromel 2. Old ION OD, Acute ION OSl 3. Old ION OD, CRVO OSl 4. Giant cell arteritisl 5. Optic nerve hypoplasia OD, ION OS
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Headache awareness day
l 47 y/oWF l CC: Episodic visual fluctuation
Severe HA on top of head x 4 mthsNumbness on L side of face
l Saw neurologist 3 mths prior– Normal CT– Dx: Migraine syndrome– Symptoms no better since migraine tx
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My Exam
l VA OD 20/25 OS 20/25l Improved to 20/20 with more (+)l SLE: Normall IOP - 14 OD, 16 OSl Disks – as shown
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What Are You Going To Do Next?
l 1. Repeat CT scanl 2. Refer to (a different) neurologistl 3. MRIl 4. Fundus photos and follow-up 1 monthl 5. OCTl 6. VF
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Name That Visual Field!
l 1. Bitemporal hemianopsial 2. Left homonymous hemianopsial 3. Right homonymous hemianopsial 4. Double arcuate scotoma OUl 5. Nasal step
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This patient’s VF
l Left homonymous hemianopsia, denser above than below
l What is the most likely etiology?l What do you do now?
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Where is the lesion most likely located?
l 1. Left optic nervel 2. Optic chiasml 3. Parietal lobel 4. Temporal lobel 5. Occipital lobe
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Visual Fields – Rules of the Road
l Defect in 1 eye – retina or ipsilateral ON or tractl Bitemporal – optic chiasm lesionl Homonymous defects – posterior fossa lesions on
ipsilateral side– Denser above – temporal lobe– Denser below – parietal lobe– The more congruous, the more posterior the lesion
l Congruous lesion w/ macular sparing – occipital lobe lesion
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What do you want to do next for this patient?
l 1. CT scanl 2. Lumbar puncturel 3. MRAl 4. MRIl 5. Neurology referrall 6. Neuro-surgery referral
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1 Final Question
l Why was the first CT normal?
penguin_.mpeg
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A Tale of Transient Visual Loss
l 79 y/o WM called saying that he “Goes blind OD”l Happened 2 times yesterday, “blindness” lasts only 5
minutes, only ODl Describes it as a curtain that rising which gets
blacker over that timel Complete blackness occursl Gradually clears upl Been occurring for 2 mths
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Transient Loss Continues
l Med hx:– 3 CVA– Aneurysm– ASA, verapamil
l Oc hx:– SRNVM w/subsequent macular scar OS– Bilateral cataract extraction
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The Exam
l VA OD 20/25, OS 20/100l Pupils - 4mm round and reactive ODl 6mm oval and reactive OSl SLE – OD PCL
OS PCL w/ 2+ PCO, nasal subconj heme
l DFE – OD as shownOS – chorioretinal macular scar
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What is his diagnosis?
l 1. Amaurosis fugaxl 2. Branch retinal vein occlusionl 3. Ischemic optic neuropathyl 4. Retinal embolusl 5. Transient Ischemic attack (TIA)
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What is your next move?
l 1. Auscultate for bruitl 2. Carotid doppler ultrasoundl 3. ESR/ C-RPl 4. OCTl 5. Temporal artery biopsyl 6. VF
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Transient Tale Part III
l Bruit R Carotidl ESR – 35mm/hrl Doppler ultrasound – Clinically significant
stenosis R Common carotid
l Immediate referral to vascular surgeon –endarterectomy
l 3 yrs later – VA 20/25 OD, no more sxs
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The Case Of The Friendly Preacher’s Wife
l 38 y/o WF complaining of a “lazy” RUL x 1 mth.
l Lid droop seems worse in AMl HA over OD for past 3 days, otherwise she
feels normall Px denies redness, d/c, trauma. VA to her
seems normal.
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Examination
l VA OD 20/20 OS 20/20 w/CLl Externals as shownl Pupils OD OS
– Light 3mm 3.5mm– Dark 3.5mm 5.5mm
– (-) APD
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5 Step Pupil Evaluation
l 1. Anisocoria greater in dim or bright light?l 2.What are the lid positions?l 3. Direct responsel 4. Afferent responsel 5. Near vs direct response
l PERRLA or PERRL mg(-) ?
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What Is The Preacher’s Wife’s Diagnosis?
l 1. Adie’s tonic pupill 2. Argyll-Robertson Pupill 3. Aneurysml 4. Horner’s Syndromel 5. Benign essential blepharospasml 6. Myasthenia gravisl 7. Pharmacologically induced anisocorial 8. She has the hots for her eye doctor!
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Anisocoria greater in bright or dim light?
l Dim light – sympathetic
l Bright light – parasympathetic
l If 1 pupil is bigger in bright light but smaller in dim light – Tonic Pupil
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Horner’s Syndrome-Oculosympathetic paresis
l Most common cause of a miotic pupil– Miosis– Ptosis– Anhydrosis– (-) APD– Kearne’s lower lid sign
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Horner’s syndrome
l Etiology– 0 – 20 Trauma– 30 -50 Neoplasm– 50+ Malignancy
l Definitive diagnosis made byl 1.pupillary dilation lag testl 2. The “C” test
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Practical Use For Cocaine in Your Office
l Instill 1 drop of 5% Cocaine– Normal eye dilates– Horner’s pupil will not dilate
– Positive diagnosis of Horner’s syndrome
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Paredrine test
l Localizes lesionl Helps us figure out the etiology
l 1% hydroxyamphetamine– If pupil dilates- pre-SCG lesion– If pupil does not dilate – post-SCG lesion
l Use pain as a prognosticator
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Horner’s Syndrome Causes
l 1st & 2nd order neuron lesions– Trauma– Intra-thoracic lesion– Tumors
l Pancoast’sl Thyroid neoplasml Malignancies
l Usually quieterl Get CT scan
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Horner’s Syndrome Causes
l 3rd order neuron lesion– Intracranial vascular or inflammatory condition
(Vascular HA, Aneurysm, Sinusitis, Cavernous Sinus Sx,Idiopathic)
– Get Head CT, MRI, MRA
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Friendly Preacher’s Wife
l Her OD pupil did not dilate with either cocaine or paredrine.
l What is her diagnosis?l Where does the lesion lie?
– 1. Head– 2. Neck– 3. Chest
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Preacher’s Wife cont.
l What is the most appropriate test to order?1) CT scan of head2) MRI of head3) MRA4) ESR5) Benign neglect
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The Friendly Preacher’s Wife
How did it all end up?
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As Many Disease As She Pleases
l 77 WFl Macular hole repair OS 8 yrs priorl Subsequent SRNVM w/ large macular scarl VA OD 20/20, OS HM@6’ – stable for 5 yrsl Recently complained of HA “alot” over OSl Says her vision OS is worsening, “it will go
black at times!”
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Exam
l VA OD 20/25- OS – LPl SLE – OD no change, OS – 2+ PCOl DFE –OD - D,M,V,P wnl OS small macular
bleed adjacent to macular scarl ONH - .1/.1 OD pink, .15/.15 OS large area
of PPAl What now?l Did we forget something?
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Ancillary Tests
l IVFA – no evidence of new SRNVMl OCT – Plush NFL, no SRNVMl ESR – 20mm/Hrl C-RP – 0.8
l What now? Is she just crazy?l Are you sure we haven’t overlooked
anything?
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Explain the VF result
l NOW what would you do?
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MRI
l Suprasellar mass with impingement on ONl Probable gliomal Underwent resection
l Craniopharyngioma!
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The Case Of Droopy Dora
l 71 y/o BF referred for recent onset of irritation OS.
l Also complains of blurry vision l HBP, arthritis,allergyl No hx of diplopia or eye turnl States that OS feels “weak”
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Droopy Dora exam
l BCVA OD 20/50, OS 20/50+2l PERRL mg(-)l CT – 15pd LXT, no EOM restrictionl SLE – OD 1+ NS, OS 1+NS, tr bulb inj,
dellen, (-)NaFl but dry areas OS>ODl IOP – 15OD, 16OSl C/D - .45/.45 OU D,M,V,P wnl
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I diagnosed dry eye, Rx’d Restasis OU BID
l Recheck 3 weeks(because of LXT)- and the cataracts must come out!
l CC: OS eyelid drooping x 1wkl VA 20/60 OUl Externals – CT -5LXT
– PA 10mm OD, 5mm OSl Disks flat, BP 186/86
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What is Dora’s diagnosis?
l 1. Dermatochalasisl 2. Mechanical ptosisl 3. Bell’s palsyl 4. 3rd nerve palsyl 5. 6th nerve palsyl 6. CVAl 7. Something else
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What is causing Dora to droop?
l 1. Hypertensionl 2. Diabetesl 3. Brain tumorl 4. Traumal 5. Aneurysml 6. CVAl 7. Myasthenia gravis
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More Dora Data
l ESR, FBS, CBC, carotid doppler all nll VA fluctuatesl At next visit - 20pd int LXT l Lids- ptosis was absent this AM but now
ptosis measures 3mm, “the droop changes”
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Now what is Dora’s diagnosis?
l 1.3rd nerve palsy due to aneurysml 2. 6th nerve palsy due to CVAl 3. MGl 4. Thyroid eye diseasel 5. Idiopathic 3rd nerve palsy
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What test could you order to confirm this?
l 1.Ach-receptor antibody testl 2. ESRl 3. Tensilon testl 4. MRI of orbits
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Ocular myasthenia gravis
l Disease of NMJl Variability and fatigability are keys to diagnosisl Cogan’s lid twitch, orbicularis weaknessl Vision may gradually deterioratel Diagnosis confirmed by tensilon test and Ach
antibody testl Tx – prednisone, pyridostigmine or monitorl Associated w/ thyroid dysfunction and thymoma
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Myasthenia gravis
l Autoimmune disorderl Weakness of voluntary musclesl Disease of younger women and older menl Ocular, systemic or bothl Disease of thymus gland, thyroid gland
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Ocular Myasthenia
l Alternating asymmetric bilateral ptosisl Worsens in bright lightl Worsens as day progressesl Myriad of EOM anomaliesl Fatigue phenomenon
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Suspect myasthenia if:
l Alternating/ variable diplopial Mixed non-localizable neuropathiesl Emotional traumal (+) tensilon test
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The Damsel In Distress
l 88y/o WF – Complains of “darkness” OSl Does not change, she woke up this way 3
days agol No pain, no HA, no photopsia or photophobial Med Hx- Synthroid, ASA, Simvastatin, Vit D,
Fel Normal affect to px??
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Damsel’s particulars
l VA – OD-20/40 , ph NIl OS -20/125, ph NIl EOM – no restrictionl SLE – normal; no AC rxn, no RIl IOP – 15OD, 18OSl Conf VF – Constricted OS- only sees
temporallyl Before DFE – anything else??
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What is your differential diagnosis?
l What tests do you want to do?
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Lab Results
l ESR – 86mm/hrl C-RP – 1.01 (elevated)l Elevated white count, l Elevated platelets
l What is the diagnosis?
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Now what?
l Refer to Neurol Refer to Retinal Refer for TA Biopsyl Refer to Pizzimenti!!l Begin steroid therapy