12/2/2016 Disclosures What the Ocular Fundus can tell Dr. Graves has no disclosures for the content of this presentation. us about Neurological Disease She has received grants from Genentech, Biogen, S3 Jennifer Graves, MD, PhD, MAS { group, NMSS, NIH, and Race to Erase MS. She has UCSF Departments of Neurology and Ophthalmology received compensation for non-branded resident education seminar supported by Biogen. Case 1 Case 1: Exam • 12 y/o girl presents with sub-acute onset • High contrast acuity 20/70 OD and 20/100 OS blurry vision and headache Color plates: 3/6 OD, 1/6 OS • No past history but BMI 30 • Pupils responsive, no relative afferent defect • Previously 20/20 with her glasses • Visual fields: Dense paracentral scotomas • Pain behind the eyes and at temples • Ductions full but elicit pain • Fundus Exam: 1
12/2/2016 Pearl: Case 1: Differential? Optic Nerve Head Edema • What are the most relevant features of case? • Best term for edematous optic disc • What next? • Papillitis • Ischemia • Papilledema = ONHE with elevated ICP http://az616578.vo.msecnd.net/files/2016/05/20/6359936760922213671202756498_w hite-pearl-in-oyster.jpg Case 1: Differential? Case 1: Evaluation for Suspected ON • What are the most relevant features of case? • MRI Brain and orbits – Mild optic nerve head edema • Serological evaluation – Painful vision loss – As appropriate: Lyme, Bartonella, RPR, ESR, B12, – Central acuity and color loss TSH, SPEP, anti-aquaporin-4 antibodies • If available visual evoked potential • What next? • Consider lumbar puncture 2
12/2/2016 Case 1: MRI Diagnosing Optic Neuritis • Visual evoked potentials http://stanford25blog.stanford.edu/wp-content/uploads/2013/06/pupilReflex.jpg; http://www.neurology.org/content/82/6/474/F1.large.jpg http://www.acadjournal.com/2006/v17/part1/p3/ Case 1: Evaluation Case 1: Evaluation Ending #1 Ending #2 Ending #3 Aquaporin-4 Antibody positive CSF: 50 WBC, IgG index 0.6 1 unique oligoclonal band VEP – P100 latencies 130, 145 VEP – P100 latencies 125, 135 CSF: 5 WBC, IgG index 1.2, CSF: 4 WBC, normal IgG index 0.5 5 unique oligoclonal bands No oligocloncal bands 3
12/2/2016 Neuromyelitis Optica (NMO) vs. MS: Case 1: Management Visual Outcomes Worse!! • Much more likely to go blind on first ON • All non-infectious ON treatment begins with high dose corticosteroids (methylprednisolone) • More complement, destructive process • If unresponsive to steroids consider plasma exchange • Global pallor, not temporal pattern in MS • Data less convincing for IVIg • Often need PLEX after steroids • Determine underlying etiology as treatments differ • Different long term therapies than MS!! – MS therapy if indicated – NMO treatment (mycophenylate, rituximab) – Isolated bilateral ON may just monitor, but if recurrent consider broad spectrum immunosuppressant (mycophenylate) Wingerchuk, 2006 Optic Neuritis vs Neuroretinitis Infectious ON • Lyme disease • Cat scratch (Bartonella) • Syphillis • Measles, mumps, herpes • Macular star, other atypical findings • Fever, systemic illness • Treat with antimicrobial not just blast with steroids! Liu, Volpe, Galetta, Elsevier, 2010 4
12/2/2016 Sarcoid: Fundus Exam Sarcoid Perineuritis with optic nerve swelling Liu, Volpe, Galetta, 2010 Liu, Volpe, Galetta, 2010; Frohman, L., et al. JNO 2003 Case 2 Sarcoid: Fundus Exam • 20 y/o college student had routine eye exam and had abnormal optic disc appearance • No headaches, blurred vision, double vision, or tinnitus • Weight gain at college over the last year • No steroid use or vitamin A products • Prior mild anemia as a teenager Optic Nerve Head Swelling Peripapillary Subretinal Granulomas Liu, Volpe, Galetta, 2010 5
12/2/2016 Case 2: Exam Case 2: Fundus Exam and OCT • 20/15 OD and 20/15 OS • Normal color and low contrast vision • Fields full to confrontation • Normal ductions • Gaze orthophoric in all directions Case 2: Differential? Case 2: Differential? • Most relevant findings • Most relevant findings – Mild optic nerve head edema (superior/inferior) • Next steps – Preserved central acuity and color vision • Next steps 6
12/2/2016 Case 2: Evaluation Case 2: Visual Fields • Next steps – Visual Fields – MRI/MRV head – Orbital ultrasound – If no mass lesion, then lumbar puncture • Opening pressure • Usual – cell counts, protein, glucose, culture and infectious as appropriate Keltner, et al., https://www.nordicclinicaltrials.com/nordic/secureFiles/public/IIHTT_Baseline_HVF_Findings_Published_version.pdf Case 3: MRI Case 2: Lumbar Puncture • Opening pressure: 37 mmHg • Otherwise bland tap • Diagnosis confirmed as? Elsevier 2010, Liu, Volpe, Galetta, Neuro-ophthalmology 7
12/2/2016 OCT in Idiopathic Intracranial Idiopathic intracranial hypertension Hypertension • Pseudotumor cerebri • Elevated intracranial pressures • Dilated optic nerve sheaths • Optic disc elevation and edema • Headache, peripheral vision loss, diplopia from pseudo- 6 th palsy, tinnitus Elsevier 2010, Liu, Volpe, Galetta, Neuro-ophthalmology Invest Ophthalmol Vis Sci. 2012 Jun 29;53(7):4069-75 Idiopathic intracranial hypertension (IIH): Idiopathic intracranial hypertension (IIH) Treatment • Medical • Adults, children affected – First line Acetazolamide , • Risk factors – Recent IIH trial – if tolerated can try up to 4gr – Recent weight gain – Avoid in first trimester pregnancy if possible – Second line: furosemide, topiramate – Steroids • Lifestyle – Vitamin A products – Weight loss , low salt diet – Doxycycline/antibiotics • Surgical – Anemia – CSF Diversion procedure • More females than males – Optic nerve sheath fenestration after puberty • Monitor Visual Fields Elsevier 2010, Liu, Volpe, Galetta, Neuro-ophthalmology Elsevier 2010, Liu, Volpe, Galetta, Neuro-ophthalmology 8
12/2/2016 OCT in Idiopathic Intracranial Hypertension Thank you! Jennifer.graves@ucsf.edu Invest Ophthalmol Vis Sci. 2012 Jun 29;53(7):4069-75 9
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