Neuro Ocular Grand Rounds Anthony B. Litwak, OD, FAAO VA Medical Center Baltimore, MD

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1 Neuro Ocular Grand Rounds Anthony B. Litwak, OD, FAAO VA Medical Center Baltimore, MD 58 YOWM! C/O I think there is something wrong with my vision, but I m not sure what it is.! +PMH for HTN, atrial fibrillation, ETOH, marijuana and cocaine abuse! POH unremarkable! Meds: 200mg Amiodarone! c/o My vision is acting funny. I m seeing double like I m drunk, even though I haven t been drinking. Does that make any sense? Case BA! BVA 20/200 OD 20/20 OS! EOM full without diplopia! CF dense superior defect OD and dense inferior defect OS! Pupils were equal, round and respond to light with no APD! Slit lamp pigmented corneal whirls OU! TA 15/15 Diagnosis! Bilateral AION vs. Papilledema Management! Lab work: o ESR: Normal o CRP: Normal o SPEP: No spike seen, normal pattern o HIV: Non-reactive! MRI: Possible slight asymmetry of left optic nerve larger than right, otherwise, normal scan of orbits/brain! CSF: Normal opening pressure and CSF composition! Thought to be bilateral non-arteritic anterior ischemic optic neuropathy 2^ to Amiodarone use. After consult with cardiology, Amiodarone was discontinued. VA remained 20/200 OD and 20/20 OS with superior visual loss in right eye and inferior visual field loss in left eye. Amiodarone! Most commonly prescribed medication for atrial fibrillation and other heart arrhythmias! Systemic toxicity results in 50% discontinuation! Toxicity includes lungs, thyroid, skin nervous system, liver and eyes! >90% of patients develop corneal deposits without significant ocular complications

2 Amiodarone and AION! 2% of patients on Amiodarone! Mean duration of therapy was 9 months! More common in males (74-84%) than females! Vision loss is more insidious than acute! 2/3 of cases are bilateral! No preference for disc size Amiodarone and AION! 1/3 of patients were asymptomatic! VA range 20/15 to LP! Median VA 20/30! 21% of patients had 20/200 or worse vision in one eye! VA improved in 58% of cases where Amiodarone was discontinued! Recommend DFE 6 mo, 9 mo and 12 mo after starting Amiodarone and q6mo thereafter Case EB! 30 YOBF with no PMH or POH! C/O of sudden decrease in vision in her left eye and eye pain x 2 days! VA 20/20 OD and 20/30 OS! CV 14/14 OD and 2/14 OS! EOM full but with pain OS! Pupil show left APD! Visual fields show an inferior arcuate defect OS! SL WNL! TA 17 OD and 20 OS! Gonio: open angles! See DFE What is Your Diagnosis?! Anterior Ischemic Optic Neuropathy! Optic Neuritis! Toxic Optic Neuropathy! Compressive Optic Neuropathy! Papilledema! Pseudotumor Cerebri! Optic Nerve Drusen! Glaucoma What is Your Next Step?! No further diagnostic workup necessary! Order MRI of head and orbit! Order lumbar puncture! Ultrasonography! Fluorescein angiography

3 ! Order blood work CBC, ESR, ANA, ACE, RPR and Lyme titer What is Your Treatment Plan?! No treatment necessary! ASA! Start 80 mg oral Prednisone! Start IV methylprednisolone! Beta Interferon! Acetazolamide 250 mg QID! Neuro consult What Should You Tell the Patient Regarding their Vision?! It will not get better but it probably won t get any worse! It will probably get worse and not improve! It should get better in one week! It may get worse, but should get better in a couple of weeks! The visual course varies so much that it is hard to say What is the Four-Year Risk of Developing MS?! Less than 10%! 10-20%! 20-30%! 30-40%! 40-50%! Greater than 50% What is the Greater Prognostic Factor for Developing MS After Developing Optic Neuritis?! Female gender! 3 or more UBO s on MRI! Less than 30 years of age! Previous optic neuritis in the fellow eye! Family history of MS Case HR! 38 YOBF! C/O dark spot in vision OD in superior field occurring transiently, a few times a day for seconds at a time! Ocular history unremarkable Systemic History! Hypertension for 16 years, controlled with Hydrolazine and Carvedilol! Recently had elevated BP for two weeks with highest recorded BP of 220/110 mm Hg. Treated with Clonidine, now better controlled.! DM II for 3 years, diet controlled! Nephropathy with dialysis for 4 years.

4 Case HR! Social History: o Never smoked, no drug or alcohol abuse! Other Pertinent History: o Was diagnosed with pre-eclampsia with all three pregnancies, with BP returning to normal in-between o Diagnosed with chronic HTN at age 30 o Recent pulsatile tinnitis Clinical Findings! BVA: 20/25-2 OD, 20/30+ OS with no PH improvement! Pupils: PERRL, negative APD! EOMs: Bilateral abduction deficit! Confrontation Fields: Full to Finger Count OU! BP: 176/100! BMI: 34.2! Diagnosis! Plan! Should the patient be scanned?! Should the patient have a lumbar puncture?! Blood work?! How to manage the high blood pressure Papilledema Pathophysiology As intracranial pressure rises, the pressure is transmitted to the optic nerve. The optic nerve sheath effectively squeezes the nerve, impeding axoplasmic transport. This causes a buildup of material at the level of the lamina cribrosa, resulting in swelling of the optic nerve head. Case FF! 56 YOAAM! +HTN! -POH, -FOH! Recent onset of muscle weakness in arms and legs making it difficult to walk, difficulty breathing and blurred vision in his right eye Clinical Findings! BVA 20/400 OD 20/20 OS! EOM full! CF constricted OD FTFC OS! Pupil show Right APD! SL! Cornea clear OU! AC shows 2+ cell/flare OD deep and quiet OS

5 ! Iris no NVI OU! TA 18/19! 2+ vitreous cells OD cl vitreous OS What is Your Diagnosis?! Anterior Ischemic Optic Neuropathy! Optic Neuritis! Toxic Optic Neuropathy! Compressive Optic Neuropathy! Orbital Tumor! Papilledema! Pseudotumor Cerebri! Infiltrative Optic Neuropathy What is Your Next Step?! No further diagnostic workup necessary! Order MRI of head and orbit! Order lumbar puncture! Ultrasonography! Fluorescein angiography! Chest X-ray! Order blood work CBC, ESR, ANA, ACE, RPR and Lyme titer Results! Lab test showed elevated ESR, positive ACE and multiple pulmonary lesions on CXR! Biopsy of the lesion was positive for Sarcoidosis! Patient was started on 80 mg of oral Prednisone Case CP! 38 YOWM! No PMH or POH! C/O of painless, progressive loss of vision OS x five years! VA 20/20 OD and 20/50 OS! CV 14/14 OD 12/14 OS! Was 20/20 OS five years ago by previous exam note! Pupil show a trace L APD! CF FTFC OD shows an inferior visual field defect OS! SL WNL, TA 14 OU! See DFE photos! Visual Fields What is Your Diagnosis?! Anterior Ischemic Optic Neuropathy! Optic Neuritis! Toxic Optic Neuropathy

6 ! Compressive Optic Neuropathy! Papilledema! Pseudotumor Cerebri! Optic Nerve Drusen What Test Would You Like to do to Confirm the Diagnosis?! MRI of the head and orbits! Lumbar puncture! Fluorescein angiography! Ultrasonography! Something else! Computerized Tomography! Autofluorescence Optic Nerve Head Drusen! Hyalin bodies! Elevated disc (little or no cupping)! Anomalous disc vessels! Familial! May develop vision or field loss! NFL dropout! Vascular anomalies CASE LH! 71 YOWM! POH MVA 1997 PMH: HTN! C/O: Reduced vision in his left eye BVA 20/20 OD 20/40 OS! Left APD SLE Iridodialysis OS TA 16 OD 18 OS! Gonio: Two clock hrs angle recession OS See optic nerve and visual fields What Is Your Diagnosis? What Is Your Management Plan?! Start anti-glaucoma treatment! Do diurnal curve! Order MRI of brain and orbits! Take disc photos and observe

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