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Neurology Case Presentation MARCH 23, 2012 LORI NOOROLLAH Chief Complaint Double Vision HPI: Middle aged woman who reports that she woke up with blurry vision and pain in her right eye Two week later– woke up with double vision Binocular, vertical and horizontal Worse on right gaze Three months later– woke up with blurry vision in left eye and left orbital pain More History PMH: HTN, Anxiety, chronic pain, GI bleed due to diverticulosis Meds: Clonidine 0.2mg qHS Metoprolol 50mg BID Diazepam prn Diltiazem qAM Losartan 100mg qHS hydrocodone prn SH: Smokes 3-4 cigarettes daily for 25 years No EtOH or illicit drug use General Exam Alert, oriented, no acute distress CV: RRR, no carotid bruit Chest: CTAB Visual Acuity: OD: 20/60 OS: 20/25 +relative APD on right red-green dyschromatopsia on right Neurological Exam Mental status and speech normal CN: PERRL APD on right Visual Fields – Inferior arcuate defect on Right Enlarged blind spot on Left normal facial sensation and movement, symmetric palate elevation, tongue midline EOM:Limited abduction and slightly limited upgaze bilaterally Motor, Sensory, Reflexes, Coordination – within normal limits Visual Fields Inferior arcuate defect in right eye Enlarged blind spot in left eye ?Where? ?What? Differential Diagnosis Anterior Ischemic Optic Neuropathy (AION) + cranial nerve infarcts AAION vs. NAION Optic Neuritis Ocular Myasthenia gravis Acetylcholine receptor antibodies negative NAION Non-arteritic Anterior Ischemic Optic Neuropathy is an “idiopathic” ischemic insult of the optic nerve head Most common optic neuropathy Annual incidence for people > age 50 is 2.3 – 10.2 /100,000 95% of cases occur in Caucasian population NAION Clinical presentation: Sudden monocular visual loss Blurring or cloudiness Often noticed upon awakening (73%) Most often painless 12% have ocular pain or headache A lot of pain more suggestive of optic neuritis or AION Exam: Reduced visual acuity to varying degrees Dyschromatopsia proportional to reduction in visual acuity Afferent pupillary defect Fundoscopic Exam: Not ruled out by normal visual acuity Optic disc swelling Disc hyperemia with splinter or flame hemorrhages Small optic cup (nerve fiber crowding) in unaffected eye Visual field defect – relative inferior altitudinal defect and absolute inferior nasal defect NAION – Fundoscopic Exam Hayreh SS (2009) Ischemic optic neuropathy. Progress in retinal and eye research 28: 34-62 NAION Vascular supply to optic nerve head 15-20 short posterior ciliary arteries, supplied by ophthalmic artery NAION Pathogenesis: Different than Ischemic CVA No clear relationship with HTN, HLD, smoking Not associated with embolism or large vessel occlusion Transient hypoperfusion of posterior ciliary arteries Vasospasm vs. nocturnal hypotension vs. impaired autoregulation of microvasculature vs. vasculopathic occlusion vs. venous insufficiency Hypoxia/Ischemia optic disc swelling (in setting of physiologically crowded optic nerve head) infarction Treatment = Modify risk factors, vision therapy Early therapy shown to have better recovery Questionable role for steroids NAION and OSA Nocturnal Hypotension Normal physiologic occurrence Autoregulation OSA Loss of autoregulation Non-dipping status Hypoxic-ischemic insult to optic nerve head Anti-hypertensive medications at night may also disrupt autoregulation OSA and NAION Stein, 2011 – American Journal of Ophthalmology Retrospective cohort study Review from managed care database looking at patients > 40 with at least 1 eye-care visit N=2,259,061 Compared incidence of NAION in population with and without OSA Compared NAION in treated vs. untreated OSA OSA and NAION Results: After adjusting for confouding variables: Untreated OSA patients had 16% increased hazard of experiencing NAION (HR 1.16, CI 1.01-1.33) compared with non-OSA patietns Treated OSA patients had no difference in hazard (HR 1.38, CI 0.76-2.5) compared with non-OSA patients NAION – Future Studies Implications: Do patients with NAION need screening for OSA? Do patients with OSA need evaluation? Consider avoiding anti-hypertensive medications at night, especially in patients “at risk” for NAION Future Studies: Treatment options/Intervention/Prevention Further investigation into the pathophysiology of NAION References Anterior Ischemic Optic Neuropathy:Part II: a discussion for physicians. Sohan Singh Hayreh, MD, MS, PhD, DSc, FRCS, FRCOphth http://webeye.ophth.uiowa.edu/component/content/article/118-aion-part2 Atkins, EJ Nonarteritic Anterior Ischemic Optic Neuropathy. Current Treatment Options in Neurology. 2011; 13: 92-100 Hayreh SS (2009) Ischemic optic neuropathy. Progress in retinal and eye research 28: 34-62 Kerr NM, Etal. Non-arteritic ischaemic optic neuropathy: A review and update. Journal of Clinical Neuroscience. 2009; 16: 994-1000. Stein JD, Etal. The Association between Glaucomatous and other causes of Optic Neuropathy and Sleep Apnea. Am J Ophthalmol. 2011; 152: 989-998. Up To Date Online