Ancillary Testing in Neuro-ophthalmology : OCT et al. Bonnie M. Keung, MD Neuro-ophthalmology Clinic 1 Objectives • Review of retinal and optic nerve anatomy • To understand the role of OCT technology in neuro-ophthalmology and neurology – Demyelinating disease – Optic nerve edema vs pseudopapilledema – Compression of the optic nerve • Basic interpretation of OCT RNFL and GCC 2 OCT • Ancillary test in neuro-ophthalmology clinic • OCT is a quick, non-contact, non-contrast technique for imaging tissues at a 3D micron- level resolution • O = Optical = Light (infrared light) • C = Coherence = monochromatic light • T = Tomography = slices 3 4 Resolution of OCT http://obel.ee.uwa.edu.au/research/fundamentals/introduction-oct/ 5 OCT • There are four generations – 3rd generation: Time domain – 4th generation: Spectral domain • Routinely used in ophthalmology – Glaucoma – Retinal pathology 6 Kimbrel EA, Lanza R. Current status of pluripotent stem cells: moving the first therapies to the clinic. Nat Rev Drug Discov. 2015 Oct;14(10):681-92 7 http://www.siumed.edu/~dking2/ssb/EE020b.htm 8 Duker JS, et al. Handbook of Retinal OCT. 2014 9 OCT: imaging the macula Case • 50 yo neurosurgeon presents with central vision loss OS x 2 weeks, metamorphopsia • PMH: seasonal allergies • Meds: intranasal steroid spray 10 11 OCT: imaging the macula Case • Exam – VA 20/20, 20/60 – No RAPD 12 OCT: imaging the macula 13 OCT: imaging the macula Duker JS, et al. Handbook of Retinal OCT. 2014 14 OCT: imaging the macula Central Serous Chorioretinopathy (CSCR) • Build up of sub-retinal fluid in macula • Related to increased corticosteroid exposure, stress, type A personality • Resolves spontaneously in a few months 15 OCT: macula to nerve 16 OCT: RNFL Retinal Nerve Fiber Layer 17 OCT: RNFL Retinal Nerve Fiber Layer Lamirel C,et al. Optical coherence tomography (OCT) in optic neuritis and multiple sclerosis. Rev Neurol (Paris). 2010 Dec;166(12):978-86. 18 OCT: print out 19 OCT: RNFL 20 OCT: GCC Ganglion Cell Complex 21 CASE • 23 yo with right sided headache x 10 days, followed by 24 hours of progressive vision loss OD. Eye pain OD with movement. • EXAM – VA hand motion OD – large RAPD OD – Fundus exam: swelling of the optic nerve 22 CASE Goldmann Visual Field OD 23 Case Goldmann Visual Field OS 24 OCT RNFL 25 OCT GCC 26 OCT Macula 27 CASE 28 CASE – Optic neuritis Anterior and retrobulbar • Received IV solumedrol: 1 g daily x 5 days • 1 month follow up – No pain – VA 20/20 OD and 20/20 OS • Was OCT really necessary? 29 OCT- Optic Neuritis with Disc swelling - Acute 30 OCT- Optic Neuritis with Disc swelling 1 month later… 31 OCT & Optic neuritis • Loss of up to 20 microns per optic neuritis • RNFL thinning occurs later – 3-6 months Costello F et al. Quantifying axonal loss after optic neuritis with optical coherence tomography. Ann Neurol 2006 59:963-969 32 OCT & Optic Neuritis • Optic neuritis – RNFL 75 u = threshold value for visual recovery* Costello F, et al. Tracking retinal nerve fiber layer loss after optic neuritis: a prospective study using optical coherence tomography. Mult Scler. 2008 Aug;14(7):893-905 33 OCT & Optic Neuritis • Case of using GCL, in optic neuritis MS • Not affected by swelling of the nerve • Earlier loss 34 OCT and Multiple Sclerosis (MS) • OCT in non-ON eyes showed thinning in RNFL compared to controls • OCT predicts MS disability in patient without ON – RNFL < 88 u • 2x risk of disability worsening in 1-3 years • 4x risk of disability worsening in 3-5 years • OCT of GCC thinning reliably mirrors brain degeneration – More strongly associated with progressive MS Martinez-Lapiscina EH, et al. Retinal thickness measured with optical coherence tomography and risk of disability worsening in multiple sclerosis: a cohort study. Lancet Neurol. 2016 May;15(6):574-84. Saidha , S., et al (2015), Optical coherence tomography reflects brain atrophy in multiple sclerosis: A four-year study. Ann Neurol., 78: 801–813 35 OCT and NMOSD • Average RNFL loss after MS-ON= 20 u • Average RNFL loss after NMO-ON = 55-83 u • Fellow eye in NMO less affected 36 OCT? Papilledema vs Pseudopapilledema CASE • 20 yo female with history of migraine with aura, 3 months of worsened headache. • Seen by optometry, referred urgently for papilledema with VA 20/20 OD and OS. Mother requesting that MRI be done right now. 37 OCT? Papilledema vs Pseudopapilledema EXAM VA: 20/20 OD and OS, no dyschromatopsia Motility: full, orthophoric 38 OCT? Papilledema vs Pseudopapilledema +HVF testing = normal +OCT = +CT scan (old) = 39 Optic Disc Drusen (ODD) • Autosomally dominant inherited • Intracellular and extracellular deposits that become calcified over time. • Scalloped disc margins 40 Optic Disc Drusen (ODD) • 0.3%-2% of population • Usually asymptomatic, or some visual field defects 41 OCT? Papilledema vs Pseudopapilledema 42 43 Papilledema vs Pseudopapilledema Drusen • ? OCT • Fundus EXAM! • B scan • CT scan • Fluorescein angiogram (FANG) • Fundus auto-fluorescence (FAF) • Lumbar puncture • Enhanced depth OCT (EDI-OCT) 44 The Fundus EXAM Papilledema Pseudopapilledema • Overt superficial drusen • Disc vessels are obscured • Disc vessels clear • Elevation goes beyond disc • Elevation confined to disc • Hemorrhages, exudates • Usually no hemorrhages • Not familial • Drusen- autosomal dominant 45 The Fundus EXAM 46 B-scan 47 CT scan https://radiologykey.com/the-orbit/ 48 Fluorescein Angiogram 49 Fundus Autofluorescence (FAF) 50 Enhanced depth OCT (EDI-OCT) Silverman AL, Tatham AJ, Medeiros FA, Weinreb RN. Assessment of optic nerve head drusen using enhanced depth imaging and swept source optical coherence tomography. J Neuroophthalmol. 2014 Jun;34(2):198-205 51 OCT: Papilledema CASE • 23 yo obese female • 2 weeks of headache, shoulder pain, double vision • Mason General – CT (-), MRV (-), LP = 49 cm H20 – Acetazolamide 1500 mg daily • Optometrist calls – VA 20/80 OD, 20/70 OS 52 OCT: Papilledema 53 OCT: Papilledema EXAM • 250# • Esotropia • Stage 4 disc swelling https://clinicalgate.com/use-of-the-hand-held-ophthalmoscope/ 54 Goldmann Visual Field OD 55 Goldmann Visual Field OS 56 OCT: Papilledema DIAGNOSIS: • IIH, visual dysfunction 57 IIH: Idiopathic Intracranial Hypertension 58 OCT: Papilledema DIAGNOSIS: • IIH, visual dysfunction PLAN: • Acetazolamide 3500 mg daily • Weight loss • Close follow up – Serial visual fields – Serial OCTs 59 OCT: Papilledema 60 Follow-up Goldmann Visual Field 61 OCT: Pseudopapilledema vs Papilledema • OCT can be used to aid in the differentiation between pseudopapilledema and papilledema – Still lean on fundus exam, HPI – Buried Drusen • B scan, CT scan, FANG, FAF • In cases of IIH, OCT can objectively track RNFL elevation and help explain progress to patient 62 OCT: Pituitary Adenoma CASE • 56 y/o veteran with frontal and retro-orbital headache, many weeks • First received anti-biotics for presumed sinusitis • Three days later, “blurred vision OS > OD” • VA: 20/25 OD, 20/60 OS • Normal ophthalmologic examination – Ophthalmology attending: “He did have a trace RAPD OS” 63 64 65 66 Junctional Scotoma 67 OCT: Pituitary Adenoma • Significant improvement in visual field if baseline OCT – RNFL was normal – if > 75-80 microns Danesh-Meyer HV, et al. In vivo retinal nerve fiber layer thickness measured by optical coherence tomography predicts visual recovery after surgery for parachiasmal tumors. Invest Ophthalmol Vis Sci. 2008 May;49(5):1879-85 68 69 .