Grand Rounds

Michael Rubin, MD

The University of Chicago

Who are these folks?

Chief Complaint

• 78 y/o female presents with decreased vision in the right eye since yesterday.

I am…

• PMH: HTN, GERD, Hypercholesterolemia

• ALL: Iodine—itching

• Meds: Cozaar, Zantac, Lascol, Ocuvite, Calcium, Fosomax

• POH: Macular Degeneration, Acute visual loss left eye three years ago

• Oc Meds: Ocuvite

Vision

• Right: 20/100

• (No improvement w/ refraction)

• Left: Counting Fingers at 2 feet

• (No improvement w/ refraction)

Exam

• APD Left eye

• Ishihara: 6/11 Right Eye

• EOM: FULL

• IOP: 12, 13

• Ant seg: PCIOL OU

• Fundus: See photos

Right Eye

Right Eye

Left Eye

Blood Pressure

BP: 148/67 Pulse 79

Right

Left

MRI

• 1. Chronic lacunar infarcts involving cerebral white matter bilaterally

• 2. Cerebral and cerebellar atrophy

• 3. No evidence of intracranial hemorrhage, mass effect, or abnormal enhancement

Labs

• WBC: 7.2

• Hg: 13.6 Hct: 40.3

• Plts: 272

• ESR: 29 (1-53)

• CRP: <3 (<5)

Treatment?

• Alphagan Bid OD, return 1 week

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

History

• Field defects typical of ischemic optic neuropathy were probably first described by Knapp in 1875.

• Miller and Smith first used the term "ischemic optic neuropathy" in 1966, and Hayreh later added the term "anterior."

• In 1924, Uhthoff first described severe visual loss, with field defects and swollen optic discs.

More history

• According to Rucker, 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, and, in 1932, Horton and colleagues at the Mayo Clinic used the term "temporal arteritis."

• To better describe the histologic features, Gilmour suggested the term "giant cell arteritis" in 1941.

• Treatment with steroids was started at the Mayo Clinic in 1949.

Types

• Anterior ischemic optic neuropathy (AION) is the most common cause of acute optic neuropathy in older age groups.

• It can be nonarteritic (nonarteritic anterior ischemic optic neuropathy [NAION]) or arteritic, the latter being associated with giant cell arteritis.

Characteristics

• It is characterized by visual loss associated with optic disc swelling of a pallid nature, sometimes with flame hemorrhages on the swollen disc or nearby neuroretinal layer, and sometimes with nearby cotton-wool exudates.

• Visual loss usually is sudden, or over a few days at most, and it usually is permanent, with some recovery possibly occurring within the first weeks or months.

• Optic atrophy of varying degrees ensues within the next few weeks, and it usually is generalized but may be sectorial (NAION).

Pathophysiology

• AION is thought to be an ischemic process affecting the posterior circulation of the globe.

• Early observations of optic disc photographs suggested that patients with small discs having smaller or nonexistent cups have an anatomic predisposition for NAION.

• As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage. The ischemic spiral is less implicated in the arteritic type of AION, in which the entire ophthalmic arterial circulation to the eye and orbit may be compromised.

Frequency

• Patients with both arteritic and nonarteritic forms of AION are usually older than 50 years, with females predominating in the arteritic group.

• The incidence of the nonarteritic type is 2.3-10.3 per 100,000 in the US, and for the arteritic type 0.36 per 100,000.

• The literature seems to support the notion that whites are affected more commonly than blacks in the nonarteritic group, and people of Scandinavian or European ancestry are the most commonly affected ethnic group in the arteritic type.

Morbidity

• NAION is not commonly associated with life-threatening conditions, although the presence of other vascular conditions is frequent (eg, hypertension, 46.9%; diabetes, 23.9%; myocardial infarction, 11%).

• Bilateral visual loss may be seen in 12-19% of NAION, and usually occurs sequentially instead of simultaneously.

Race and Sex

• NAION is most common in Caucasians (95%); it is less common in African Americans (2%), Asians (3%), and Hispanics (1%).

• Females dominate the incidence in both forms of AION, but only slightly in the nonarteritic form (1.2:1), as compared to the arteritic type (2:1).

You see these corneal findings in my disease…

Painful…

History

• Visual loss is painless in at least 90% of patients with NAION. The vision loss is noticed upon awakening, perhaps due to nocturnal hypotension.

– NAION has typical findings of visual loss and field loss in an otherwise asymptomatic individual.

– A small cup disc ratio usually is noted. Initially, the optic disc is swollen and pale, often in a generalized or diffuse manner.

– Sectorial disc edema, especially of the superior disc, is classic.– Visual loss with NAION usually is not as severe as with arteritic

AION, but vision loss as severe as no light perception has been described.

Associations with AION– Vasculitides

• Giant cell arteritis

• Polyarteritis nodosum

• Systemic lupus

• Buerger disease

• Allergic vasculitis

• Postviral vasculitis

• Postimmunization

• Syphilis

• Radiation necrosis– Systemic vasculopathies

• Hypertension

• Atherosclerosis

• Diabetes mellitus

• Migraine

• Takayasu disease

• Carotid occlusive disease – Hematologic

• Polycythemia vera

• Sickle cell disease (trait)

• Acute hypotension (shock)

• Glucose-6-phosphate dehydrogenase deficiency (G-6-PD)

Differential

• Diabetic papillitisOcular hypotony with disc edemaImpending central retinal vein occlusionPseudopapilledemaPapilledema

Labs

• The most important test is ESR.• A hematology group is useful. Mild anemia may

be present.• Other blood tests, such as the C-reactive protein

(CRP), have been found useful in diagnosing giant cell arteritis.

• Fluorescein angiography has been suggested as a possible method of distinguishing arteritic AION from NAION. With arteritic AION, a markedly prolonged choroidal filling time usually is present.

Imaging

• Magnetic resonance imaging is useful in younger individuals who may have demyelinating disease. It is not useful in older age groups, either in the arteritic or nonarteritic form of AION.

• Angiography of the cerebral circulation has been useful in giant cell arteritis, showing segmental stenosis or even occlusion of the extracranial vessels. However, this invasive study has fallen into less frequent use.

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.

Dr…

Anterior Segment…

Steroids?

• Steroid treatment for the nonarteritic type of AION has its advocates, but data do not support its use. In those cases where the diagnosis is in question, a short-term trial is warranted.

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.

Optic Nerve DecompressionOptic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. The Ischemic Optic Neuropathy Decompression Trial Research Group.

DESIGN:The Ischemic Optic Neuropathy Decompression Trial (IONDT) is a randomized, single-masked, multicenter trial. 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: 32.6% of the surgery group improved compared with 42.7% of the careful follow-up group. 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 12.4% in the careful follow-up group. CONCLUSION:Results from the IONDT indicate that optic nerve decompression surgery for NAION is not effective, may be harmful, and should be abandoned. The spontaneous improvement rate is better than previously reported.

Who are we?

My eye findings are due to this bugger…

Bibliography• Ali Ibn Isa: Memorandum Book of a Tenth-Century Oculist. (Translated by CA Wood). Chicago: Northwestern University; 1936. • Bielory L, Ogunkoya A, Frohman LP: Temporal arteritis in blacks. Am J Med 1989 Jun; 86(6 Pt 1): 707-8[Medline]. • Collignon-Robe NJ, Feke GT, Rizzo JF: Optic nerve head circulation in nonarteritic anterior ischemic optic neuropathy and optic neuritis.

Ophthalmology 2004 Sep; 111(9): 1663-72[Medline]. • Costello F, Zimmerman MB, Podhajsky PA: Role of thrombocytosis in diagnosis of giant cell arteritis and differentiation of arteritic from non-arteritic

anterior ischemic optic neuropathy. Eur J Ophthalmol 2004 May-Jun; 14(3): 245-57[Medline]. • Crawley B, Scherer R, Langenberg P, Dickersin K: Participation in the Ischemic Optic Neuropathy Decompression Trial: sex, race, and age. Ophthalmic

Epidemiol 1997 Sep; 4(3): 157-73[Medline]. • Foroozan R, Varon J: Bilateral anterior ischemic optic neuropathy after liposuction. J Neuroophthalmol 2004 Sep; 24(3): 211-3[Medline]. • Glueck CJ, Wang P, Bell H: Nonarteritic anterior ischemic optic neuropathy: associations with homozygosity for the C677T methylenetetrahydrofolate

reductase mutation. J Lab Clin Med 2004 Mar; 143(3): 184-92[Medline]. • Hattenhauer MG, Leavitt JA, Hodge DO, et al: Incidence of nonarteritic anterior ischemic optic neuropathy. Am J Ophthalmol 1997 Jan; 123(1): 103-7

[Medline]. • Horton BT, Magath TB, Brown GE: An undescribed form of arteritis of the temporal vessels. Proc Staff Meet Mayo Clinic. 1932; 7: 700. • Hutchinson J: Diseases of the arteries. Arch Surg (London) 1890; 1: 323. • Ischemic Optic Neuropathy Decompression Trial: Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the

Ischemic Optic Neuropathy Decompression Trial. Arch Ophthalmol 1996 Nov; 114(11): 1366-74[Medline]. • Johns LN, Arnold AC: Incidence of nonarteritic anterior ischemic optic neuritis (AION. population based study). J Neuroophthalmol 1994; 14: 38-49. • Kuprjanowicz L, Goslawski W, Karczewicz D: Retinal nerve fiber analysis in patients with anterior ischemic optic neuropathy by scanning laser

polarimetry. Klin Oczna 2004; 106(3 Suppl): 440-2[Medline]. • Love DC, Rapkin J, Lesser GR, et al: Temporal arteritis in blacks. Ann Intern Med 1986 Sep; 105(3): 387-9[Medline]. • Miller NR: Anterior ischemic optic neuropathy. In: Walsh and Hoyt's Clinical Neuro-Ophthalmology. Vol 1. 1982; 212-226. • Munteanu M, Lehaci C: Acute anterior ischemic optic neuropathy in association with optic nerve drusen. Oftalmologia 2004; 48(3): 16-9[Medline]. • Purvin V, King R, Kawasaki A: Anterior ischemic optic neuropathy in eyes with optic disc drusen. Arch Ophthalmol 2004 Jan; 122(1): 48-53[Medline]. • Salomon O, Rosenberg N, Steinberg DM: Nonarteritic anterior ischemic optic neuropathy is associated with a specific platelet polymorphism located on

the glycoprotein Ibalpha gene. Ophthalmology 2004 Jan; 111(1): 184-8[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. JAMA 1995 Feb 22; 273(8): 625-32[Medline]. • Uhtoff W: Zu den entzundlichen sehnerven:Affectionen bei arteriosklerose. Ber Dtsch Ophthalmol Gesampte 1924; 44: 196-198.