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Michael Rubin, MD The University of Chicago
Who are these folks?
• 78 y/o female presents with decreased vision in the right eye since yesterday.
• PMH: HTN. Lascol. Fosomax • POH: Macular Degeneration. Ocuvite. Hypercholesterolemia • ALL: Iodine—itching • Meds: Cozaar. Acute visual loss left eye three years ago • Oc Meds: Ocuvite . Zantac. GERD. Calcium.
Vision • • • • Right: 20/100 (No improvement w/ refraction) Left: Counting Fingers at 2 feet (No improvement w/ refraction) .
13 Ant seg: PCIOL OU Fundus: See photos .Exam • • • • • • APD Left eye Ishihara: 6/11 Right Eye EOM: FULL IOP: 12.
Right Eye .
Right Eye .
Left Eye .
Blood Pressure BP: 148/67 Pulse 79 .
MRI • 1. or abnormal enhancement . mass effect. Cerebral and cerebellar atrophy • 3. No evidence of intracranial hemorrhage. Chronic lacunar infarcts involving cerebral white matter bilaterally • 2.
3 Plts: 272 ESR: 29 (1-53) CRP: <3 (<5) .6 Hct: 40.2 Hg: 13.Labs • • • • • WBC: 7.
return 1 week .Treatment? • Alphagan Bid OD.
These two… .
This test is named after me… .
Follow up 1 week • Vision right eye: HM peripherally .
Treatment • • • • Prednisone: 60 mg x 5 days 40 mg x 5 days 20 mg x 5 days .
Follow up 2 weeks • VA right: HM peripherally .
Follow up 5 weeks • VA right eye: Hand motions peripherally .
" • In 1924. • Miller and Smith first used the term "ischemic optic neuropathy" in 1966. Uhthoff first described severe visual loss.History • Field defects typical of ischemic optic neuropathy were probably first described by Knapp in 1875. . with field defects and swollen optic discs. and Hayreh later added the term "anterior.
. Gilmour suggested the term "giant cell arteritis" in 1941. in 1932. temporal arteritis probably was first described very early by Ali Ibn Isa (AD 940-1010). • In modern times (1890). Jonathan Hutchinson described a disease of this nature. Horton and colleagues at the Mayo Clinic used the term "temporal arteritis. and.More history • According to Rucker." • To better describe the histologic features. • Treatment with steroids was started at the Mayo Clinic in 1949.
. the latter being associated with giant cell arteritis. • It can be nonarteritic (nonarteritic anterior ischemic optic neuropathy [NAION]) or arteritic.Types • Anterior ischemic optic neuropathy (AION) is the most common cause of acute optic neuropathy in older age groups.
• Optic atrophy of varying degrees ensues within the next few weeks. and it usually is generalized but may be sectorial (NAION).Characteristics • It is characterized by visual loss associated with optic disc swelling of a pallid nature. or over a few days at most. sometimes with flame hemorrhages on the swollen disc or nearby neuroretinal layer. . • Visual loss usually is sudden. and sometimes with nearby cotton-wool exudates. and it usually is permanent. with some recovery possibly occurring within the first weeks or months.
Pathophysiology • AION is thought to be an ischemic process affecting the posterior circulation of the globe. in which the entire ophthalmic arterial circulation to the eye and orbit may be compromised. the swelling compromises circulation. The ischemic spiral is less implicated in the arteritic type of AION. • As an ischemic episode evolves. • Early observations of optic disc photographs suggested that patients with small discs having smaller or nonexistent cups have an anatomic predisposition for NAION. . with a spiral of ischemia resulting in further neuronal damage.
000 in the US. • The incidence of the nonarteritic type is 2.36 per 100.3-10. and for the arteritic type 0.000. .Frequency • Patients with both arteritic and nonarteritic forms of AION are usually older than 50 years. • The literature seems to support the notion that whites are affected more commonly than blacks in the nonarteritic group. with females predominating in the arteritic group. and people of Scandinavian or European ancestry are the most commonly affected ethnic group in the arteritic type.3 per 100.
hypertension. myocardial infarction.Morbidity • NAION is not commonly associated with life-threatening conditions. 23. 46. and usually occurs sequentially instead of simultaneously.9%. although the presence of other vascular conditions is frequent (eg.9%. . 11%). diabetes. • Bilateral visual loss may be seen in 1219% of NAION.
• Females dominate the incidence in both forms of AION. as compared to the arteritic type (2:1).2:1). but only slightly in the nonarteritic form (1.Race and Sex • NAION is most common in Caucasians (95%). . and Hispanics (1%). it is less common in African Americans (2%). Asians (3%).
You see these corneal findings in my disease… .
especially of the superior disc. – A small cup disc ratio usually is noted. is classic. – Sectorial disc edema. . the optic disc is swollen and pale. Initially. but vision loss as severe as no light perception has been described. – NAION has typical findings of visual loss and field loss in an otherwise asymptomatic individual. The vision loss is noticed upon awakening. often in a generalized or diffuse manner. perhaps due to nocturnal hypotension. – Visual loss with NAION usually is not as severe as with arteritic AION.History • Visual loss is painless in at least 90% of patients with NAION.
Associations with AION – Vasculitides • • • • • • • Giant cell arteritis Polyarteritis nodosum Systemic lupus Buerger disease Allergic vasculitis Postviral vasculitis Postimmunization • • Syphilis Radiation necrosis Hypertension Atherosclerosis Diabetes mellitus Migraine Takayasu disease Carotid occlusive disease Polycythemia vera Sickle cell disease (trait) Acute hypotension (shock) Glucose-6-phosphate dehydrogenase deficiency (G-6-PD) – Systemic vasculopathies • • • • • • – Hematologic • • • • .
Differential • Diabetic papillitis Ocular hypotony with disc edema Impending central retinal vein occlusion Pseudopapilledema Papilledema .
have been found useful in diagnosing giant cell arteritis. • Other blood tests. . a markedly prolonged choroidal filling time usually is present. • Fluorescein angiography has been suggested as a possible method of distinguishing arteritic AION from NAION. With arteritic AION.Labs • The most important test is ESR. such as the C-reactive protein (CRP). Mild anemia may be present. • A hematology group is useful.
It is not useful in older age groups. showing segmental stenosis or even occlusion of the extracranial vessels. this invasive study has fallen into less frequent use. either in the arteritic or nonarteritic form of AION. However. .Imaging • Magnetic resonance imaging is useful in younger individuals who may have demyelinating disease. • Angiography of the cerebral circulation has been useful in giant cell arteritis.
.Histology • The idiopathic form of ischemic optic neuropathy has no characteristic pathology other than obliterative occlusion of the cilioretinal arteries and ischemic necrosis of the optic nerve head in variable degree.
Anterior Segment… .
In those cases where the diagnosis is in question. a short-term trial is warranted. . but data do not support its use.Steroids? • Steroid treatment for the nonarteritic type of AION has its advocates.
Prognosis • Prognosis for visual recovery generally is poor. . • If there is any good news about the nonarteritic form of ischemic optic neuropathy. it is that a second attack has never been documented in an eye that has already suffered one attack.
Patients receiving surgery had a significantly greater risk of losing three or more lines of vision at 6 months: 23.9% in the surgery group worsened compared with 12. and should be abandoned.7% of the careful follow-up group. RESULTS: Patients assigned to surgery did no better when compared with patients assigned to careful follow-up regarding improved visual acuity of three or more lines at 6 months: 32. The spontaneous improvement rate is better than previously reported. multicenter trial. single-masked. CONCLUSION: Results from the IONDT indicate that optic nerve decompression surgery for NAION is not effective.6% of the surgery group improved compared with 42. may be harmful.Optic Nerve Decompression Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful.4% in the careful follow-up group. . The Ischemic Optic Neuropathy Decompression Trial Research Group. DESIGN: The Ischemic Optic Neuropathy Decompression Trial (IONDT) is a randomized.
Who are we? .
My eye findings are due to this bugger… .
Ann Intern Med 1986 Sep. 212-226. 14: 38-49. Hattenhauer MG. and age. race. Varon J: Bilateral anterior ischemic optic neuropathy after liposuction. Hodge DO. JAMA 1995 Feb 22. 1: 323. Ophthalmology 2004 Sep. Am J Ophthalmol 1997 Jan. Kuprjanowicz L. Eur J Ophthalmol 2004 May-Jun. In: Walsh and Hoyt's Clinical Neuro-Ophthalmology. Ophthalmology 2004 Jan. Arch Ophthalmol 2004 Jan. Langenberg P. Wang P. Scherer R. Brown GE: An undescribed form of arteritis of the temporal vessels. 4(3): 157-73[Medline]. Kawasaki A: Anterior ischemic optic neuropathy in eyes with optic disc drusen. Feke GT. 14(3): 245-57[Medline]. Love DC. 143(3): 184-92[Medline]. Lehaci C: Acute anterior ischemic optic neuropathy in association with optic nerve drusen. Ophthalmic Epidemiol 1997 Sep. Munteanu M. Magath TB. Miller NR: Anterior ischemic optic neuropathy. Arnold AC: Incidence of nonarteritic anterior ischemic optic neuritis (AION. Collignon-Robe NJ. Rizzo JF: Optic nerve head circulation in nonarteritic anterior ischemic optic neuropathy and optic neuritis. Vol 1. Johns LN. 105(3): 387-9[Medline]. et al: Temporal arteritis in blacks. Hutchinson J: Diseases of the arteries. J Neuroophthalmol 2004 Sep. Ogunkoya A. Podhajsky PA: Role of thrombocytosis in diagnosis of giant cell arteritis and differentiation of arteritic from non-arteritic anterior ischemic optic neuropathy. 273(8): 625-32[Medline]. 86(6 Pt 1): 707-8[Medline]. Rosenberg N. Costello F. 7: 700. • • . Leavitt JA. 111(1): 184-8[Medline]. 111(9): 1663-72[Medline]. 24(3): 211-3[Medline]. Goslawski W. Foroozan R. Purvin V. 122(1): 48-53[Medline]. 48(3): 16-9[Medline]. The Ischemic Optic Neuropathy Decompression Trial Research Group: Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. Salomon O. Zimmerman MB. Am J Med 1989 Jun. 123(1): 1037[Medline]. Rapkin J. Bielory L. 114(11): 1366-74[Medline]. Karczewicz D: Retinal nerve fiber analysis in patients with anterior ischemic optic neuropathy by scanning laser polarimetry. 1936. Uhtoff W: Zu den entzundlichen sehnerven:Affectionen bei arteriosklerose. Glueck CJ. J Neuroophthalmol 1994. population based study). Proc Staff Meet Mayo Clinic. Lesser GR.Bibliography • • • • • • • • • • • • • • • • • • Ali Ibn Isa: Memorandum Book of a Tenth-Century Oculist. Ber Dtsch Ophthalmol Gesampte 1924. Oftalmologia 2004. 1932. Steinberg DM: Nonarteritic anterior ischemic optic neuropathy is associated with a specific platelet polymorphism located on the glycoprotein Ibalpha gene. Arch Ophthalmol 1996 Nov. et al: Incidence of nonarteritic anterior ischemic optic neuropathy. Ischemic Optic Neuropathy Decompression Trial: Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the Ischemic Optic Neuropathy Decompression Trial. Crawley B. Arch Surg (London) 1890. Frohman LP: Temporal arteritis in blacks. King R. Klin Oczna 2004. Chicago: Northwestern University. Horton BT. 106(3 Suppl): 440-2[Medline]. J Lab Clin Med 2004 Mar. 1982. (Translated by CA Wood). Bell H: Nonarteritic anterior ischemic optic neuropathy: associations with homozygosity for the C677T methylenetetrahydrofolate reductase mutation. 44: 196-198. Dickersin K: Participation in the Ischemic Optic Neuropathy Decompression Trial: sex.