Pearls, Pitfalls and Advances in Neuro-Ophthalmology Nancy J. Newman, MD Emory University Atlanta, GA Consultant for Gensight Biologics, Santhera Data Safety Monitoring Board for Quark AION Study Medical-legal consultant (IIH and peri-op vision loss) Where ? What? Now What? 1
CRAO BRAO Vascular TMVL Acute retinal ischemia Different visual outcomes Same systemic implications Study MRI Results Correlation Boston DWI+ in 31/129 (24%) Neuro sx+ 2012 Same vascular territory as Permanent VL > TMVL visual loss in 28/31 Identified cause Small, multiple infarctions Embolic cause Am J Ophthalmol 2014 Korea DWI+ in 8/33 (24.2%) Neuro sx+ 2014 Same vascular territory as CRAO > BRAO visual loss in 8/8 Identified cause “TIA” + = STROKE Small, multiple infarctions Embolic cause Germany DWI+ in 49/213 (23%) Neuro sx+ 2015 Same vascular territory as Identified cause visual loss in 55% Embolic cause Small, multiple infarctions 2
( The Neurologist 2012;18:350–355) Non-mydriatic fundus cameras • Easy for non-ophthalmic trained individuals to use Arch Ophthalmol 2012; 130: 939-940 • No pupillary dilation • Able to take quality photographs of the posterior pole • Reveals unrecognized findings in ED (Bruce et al. NEJM 2011; 364:387-9 ) 3
Optic Neuropathy Classic Features • Decreased visual acuity • Abnormal visual field • Relative afferent pupillary defect • Can see through to the nerve • Swollen or pale optic nerve Optic Neuropathy Optic Neuropathy Disc Alternatives Causes • Inflammatory • Vascular • Compressive/Infiltrative • Toxic/Nutritional • Hereditary • Traumatic • Elevated intracranial pressure • Elevated intraocular pressure 4
Optic Neuropathy Optic Neuropathy Papilledema Causes • Inflammatory • Disc swelling from ↑ • Vascular intracranial pressure • Compressive/Infiltrative • Any age • Toxic/Nutritional • Painless • Hereditary • Bilateral • Spares visual acuity • Traumatic • Constriction of • Elevated intracranial pressure visual field • Elevated intraocular pressure Papilledema Idiopathic intracranial Causes hypertension • Papilledema • Intracranial mass • Headaches lesions • No localizing neurologic • Hydrocephalus symptoms/signs except for VIth • Meningeal processes • No intracranial process, no venous • Cerebral venous sinus thrombosis thrombosis • Normal CSF contents • Idiopathic • CSF opening pressure ≥25cm H 2 O (pseudotumor cerebri) 5
IIH imaging • Elevated ICP measured in the lateral decubitus position: neonates: >76 mm H2O, age 1–18 years: >280 mm H2O • Normal CSF composition except in neonates who may have up to 19 WBC/mm3 if 0–28 days and up to 9 WBC/mm3 if between 29 and 56 days old; the protein may be as high as 150 mg/dl IIH: Poor visual prognosis � Patient’s characteristics � Not just a diagnosis of exclusion • Black race. Neurology 2008; 70: 861-7 • Male. Neurology 2009; 72:304-9 � New diagnostic criteria • Severe obesity. J Neuro-Ophthalmol 2013; 33: 4-8 � Papilledema • Anemia / sleep apnea syndrome / HTN � Measure of intracranial pressure � Rapid onset (fulminant IIH ). Neurology 2007; 68: 229-232 � Neuroimaging findings 6
� IIH is everywhere there are obese people 7
Optic Neuropathy Causes ScientificWorldJournal. 2015; 2015: 140408. • Inflammatory • Vascular • Compressive/Infiltrative • Toxic/Nutritional TS stenosis • Hereditary • Traumatic After stenting • Elevated intracranial pressure Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis • Elevated intraocular pressure Neurology 2013;80:289-95 8
Optic Neuropathy Typical Optic Neuritis • Inflammation of the optic nerve • F:M 3:1 • Age: 15-45 • Pain on eye movement • Normal or swollen disc • Spontaneous improvement • Associated with multiple sclerosis ONTT ONTT: MRI predicts the risk of MS 70 Total 50% ≥ 1 lesion 72% at 15 yrs • No difference in visual acuity 60 between steroid and placebo 50 groups at 6 months. 40 • I.V. steroids may accelerate 30 recovery by 2 to 3 weeks. 20 No lesion 25% • P.O. steroids doubled the risk of 10 recurrence in either eye. 0 Years 1 2 3 4 5 6 7 8 9 10 11 12 (NEJM 326:581, 1992) 9
OCT: Retinal Nerve Fiber Clinical Features of Optic Neuritis with Low Risk of CDMS in Patients with No Brain MRI Lesions Layer (RNFL) Thickness No cases of CDMS have developed when any one of • Correlates with axonal the following clinical features* was present: loss • Severe Disc Swelling (21 patients) • Correlates with visual dysfunction • Hemorrhage, disk or peripapillary (16 patients) • Correlates with: • Macular Exudates (8 patients) – Brain atrophy in MS • Painless (19 patients) – Disability • No Light Perception (7 patients) – Quality of life From: Relationships Between Retinal Axonal and Neuronal Measures and Global Central Nervous System Pathology in Multiple Sclerosis JAMA Neurol. 2013;70(1):34-43. doi:10.1001/jamaneurol.2013.573 Cellular composition of the retinal layers : ILM: inner limiting membrane RNFL: retinal nerve fiber layer GCL: ganglion cell layer IPL: inner plexiform layer INL: inner nuclear layer OPL: outer plexiform layer ONL: outer nuclear layer ELM: external limiting membrane IPS: inner photoreceptor segments OPS: outer photoreceptor segments PR: photoreceptors RPE: retinal pigment epithelium -Healthy subject. 3-dimensional macular volume cube generated by Cirrus HD-OCT from the macular region -The individual layers of the retina are readily discernible, except for GCL and IPL, which are difficult to distinguish. -During the segmentation process (performed in 3-dimension), the segmentation software identifies the outer boundaries of the macular RNFL, IPL, and OPL, as well as the inner boundary of the RPE, which is identified by the conventional Cirrus HD-OCT algorithm. The identification of these boundaries facilitates OCT segmentation, enabling determination of the thicknesses of the macular RNFL, GCL + IPL, the INL + OPL, and the ONL including the inner and outer photoreceptor segments 10
Fingolimod and Macular Edema 1. Incidence of macular edema is low (~1%); (uveitis, DM increase risk). Ophthalmology 2013; 120: 1432-1439 2. Screening evaluation for uveitis, macular or retinal vascular disease prior to starting, or within the first few weeks of starting fingolimod 3. Re-evaluation (complete eye exam +/- macular OCT) at 3-4 months of therapy (most reported cases of macular edema occurred within 3-4 months) Optic Neuropathy Causes • Inflammatory • Vascular • Compressive/Infiltrative • Toxic/Nutritional • Hereditary • Traumatic • Elevated intracranial pressure • Elevated intraocular pressure 11
Optic Neuritis and NMO Abs • Severe • Bilateral • Bilateral • Poor recovery • Severe • Recurrent Optic Neuritis and NMO Abs • Poor recovery • Recurrent 12
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