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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
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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 • DiltiazemqAM • Losartan 100mg qHS • hydrocodoneprn • 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 • Not ruled out by normal visual acuity • Dyschromatopsia proportional to reduction in visual acuity • Afferent pupillary defect • Fundoscopic Exam: • 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, FRCOphthhttp://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-arteriticischaemic 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