Swollen Optic Disc Presentation

Northeastern University10/31/12

• 58yo WM with type II DM and HTN is an established patient with one swollen optic disc and spots in his vision when he woke up. There are no other significant abnormal findings.

Proceed by:1. GCA and Increased Intracranial Pressure

questions (HA, Jaw/scalp/NECK, Tinnitus, N/V, TVO)

2. Cranial Nerve Exam (Dr. Castillo)-cover test in multiple positions of gaze (Keane)

3. Vital Signs4. Image posterior pole5. schedule the VF and F/U appt6. Educate “Swollen Optic Disc”/ER visit possible 7. Get release of information for PCP’s note/etc8. ESR/CRP within a few hours

Valerie Biousse’s Neuro-OphthalmologyAnterior Optic Neuropathy Papilledema

OCULAR SIGNS: decrease in VA

decrease in color

Central/Arcuate/Altitudinal

Disc edema more often unilateral

____________________________SYSTEMIC SIGNS:Often isolated (or associated with symptoms/signs related to underlying disease – like GCA symptoms)

OCULAR SIGNS:Normal VA’s til late

Normal color

Enlarged blindspot, nasal defect, constrictionDisc edema almost always bilateral

____________________________

SYSTEMIC SIGNS:Other symptoms or signs of increased ICP, HA, Nausea, Vomiting, Diplopia, 6th nerve palsy, Pulsatile Tinnitus, TVO’s,(Fever,Seizure,Stiffness)

(OR >1 CN DAMAGED)

Grant Liu’s NeuroOphthamologyTable 6–1 Differential diagnosis

of a swollen optic disc: causes according to frequency

Most common Papilledema BILATERALOptic neuritis PAINMRIAnterior ischemic optic

neuropathy (GCAPAIN)PseudopapilledemaCommonCentral retinal vein occlusion? Diabetic papillopathy Uncommon Ocular hypotony

Intraocular inflammation (uveitis) Malignant hypertension Optic perineuritis PAIN MRI Papillitis Intrinsic optic disc tumors Leber’s hereditary optic

neuropathy -YOUNGOptic nerve infiltration by sarcoidosis PAIN MRI lymphoma leukemia plasma cell dyscrasia

ADDRESSED BY HISTORY

Grant Liu’s NeuroOphthamologyTable 6–1 Differential diagnosis of

a swollen optic disc: causes according to frequency

Most common Papilledema Optic neuritis Anterior ischemic optic

neuropathy Pseudopapilledema CHARACTCommon FINDINGSCentral retinal vein occl-RET Diabetic papillopathy-RET Uncommon Ocular hypotony-IOP

Intraocular inflammation (uveitis) - CELLS

Malignant hypertension BPOptic perineuritis Papillitis BILATERAL Intrinsic optic disc tumors Leber’s hereditary optic

neuropathy Optic nerve infiltration by sarcoidosis lymphoma ? CELLS (Kanski)leukemia ? RET (Kanski)plasma cell dyscrasia RETINAL

ADDRESSED BY EXAM

Grant Liu’s NeuroOphthamologyTable 6–1 Differential diagnosis

of a swollen optic disc: causes according to frequency

Most common Papilledema Optic neuritis Anterior ischemic optic

neuropathy Pseudopapilledema Common Central retinal vein occlusion? Diabetic papillopathy Uncommon Ocular hypotony

Intraocular inflammation (uveitis)

Malignant hypertension Optic perineuritis Papillitis Int. optic D. tum. Fast;NO IMP.Leber’s hereditary optic

neuropathy Optic nerve infiltration sarcoidosis lymphoma leukemia Meningioma—Slow ; NO IMP.Paraneoplastic –Slow; NO IMP.

Differential Diagnosis• AION – Most Common• In order search for NEOPLASIA IMAGING WHICH YOU MUST PURSUE YOURSELF

-------------------------------------------------------

LOOKING AT AION: 1. GCA2. NAION

1. GCA • is the most common form of

systemic vasculitis in adults • its most feared complication

is irreversible loss of vision (like Pseudo. Cerebri)

three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.

Vasculitis PLUS any 3 of 5 gets Dx of GCA

1. 50yrs or older 2. New onset or new type of localized pain in the head

3. ESR ≥50 mm/hr by the Westergren method

4. Temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries 5. Biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells

three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.

Vasculitis PLUS any 3 of 5 gets Dx of GCA

1. 50yrs or older 2. New onset or new type of localized pain in the head

3. ESR ≥50 mm/hr by the Westergren method

4. Temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries 5. Biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells when ESR is normal, systemic symptoms are almost always present.

three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.

Vasculitis PLUS any 3 of 5 gets Dx of GCA

1. 50yrs or older 2. New onset or new type of localized pain in the head

3. ESR ≥50 mm/hr by the Westergren method

4. Temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries 5. Biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells

In the 16-26% WITHOUT systemic symptoms the ESR is almost always elevated

1. GCA–this pt in this case had no GCA symptoms and

the ESR/CRP were not elevated – so GCA not suspected in this case

–MUST RULE OUT GCA WITH STAT ESR AND CRP

GCA (Purvin) BOTH OIS(Glaser-Mendrinos)

● Ischemic optic neuropathy

● Homonymous hemianopia

● Cortical blindness

(NECK PAIN)

● Retinal ischemia● Anterior segment ischemia● Eye pain● Transient visual loss● Abnormal ocular Motility – diplopia

● Retinal Embolus(IF you see it in a GCA suspect, look for Carotid ArteryDisease)

FULL SPECTRUM OF GCA’s VISION FINDINGS

(NAION)

2. NAION

NAION

• Pathogenesis: unknown• majority 60-70yo but could be any age• Caucasian>African American or Hispanic

American• Increased Risk in DM, high Cholesterol, HTN

Hypertensive THERAPY as a POSSIBLE PRECIPITATING Risk factor for NAION

• Nocturnal Hypotension–vision loss noticed in the morning in

NAION –as well as progressive vision loss in

NAION

Other possible risk factors

• Disc at Risk / crowded disc–If you look at the fellow eye and it is

cupped – question NAION as the dx• Sleep Apnea?• Smoking?• Viagra?

Symptoms of NAION

• IONDT: 40% noticed monocular vision loss upon awakening

• Maximal when noted and usually does not progress

• Not other ocular or systemic symptoms

•Pain is rare.

Signs of NAION• IONDT:

50% see better than 20/64 67% see better than 20/200

• +APD; +red cap test• Any VF Defect including inferior altitudinal

• Classically Sectoral or Diffuse Hyperemic or Pale Disc Edema with hemes

Education of NAION pt• Can improve or worsen in 1st month

• IONDT: 43% IMPROVE with no tx• IONDT: 14.7% is the risk of fellow eye

involvement within 5 years• Take Evening dose of BP meds earlier• Avoid Viagra

The Case

03/16/12 – As previously stated the pt woke up with bunch of black spots in left eye’s vision…

History of microvascular CN 6 palsy ‘07 that resolved within two months

Brief Mention about……VA’s

20/20 OU throughout…Macula’s:No macular edema throughout…IOP’s:IOP was unremarkable throughout

Brief Mention about……Optic Nerve:No pallor or APD or red desat was noted

throughout…motilities:After initial CN 6 palsy resolved; No diplopia; no

restriction in eye movement

…overall changes in health:No symptoms other than black spotsNo HA, scalp tenderness, jaw claudication, or

new onset neurological deficit

3 4 5 6 7 8 9 10

-18

-16

-14

-12

-10

-8

-6

-4

-2

0

RIGHT EYE

LEFT EYE

MONTHS in 2012

MDOfVF

~Altitudinal defectsW/ CENTRAL SPARING

3 4 5 6 7 8 9 100

50

100

150

200

250

300

350

400

450

OCT thickness measures of RIGHT EYE

umInferior rim

Superior rim

SECTORAL DISC INVOLVEMENT

HYPEREMIC SWELLING (HEMES)

3 4 5 6 7 8 9 100

50

100

150

200

250

300

350

400

450

OCT thickness measures of LEFT EYE

um

Inferior rim

Superior rim

SECTORAL DISC INVOLVEMENT

HYPEREMIC SWELLING/HEMES

Superior rim right eye

3 4 5 6 7 8 9 100

50

100

150

200

250

300

350

400

450

SUS-PECTEDInferior rim of left eye

4-5 mos

3 4 5 6 7 8 9 100

50

100

150

200

250

300

350

400

450

Inferior rim of left eye

Superior rim right eye

3 4 5 6 7 8 9 10

-18

-16

-14

-12

-10

-8

-6

-4

-2

0

Mean Deviation of VF RIGHT

SUDDEN (NOT COMPLETE) LOSS OF VISION WITH IMPROVEMENT

A PROLONGED/POOR COURSE WOULD NOT BE CONSISTENT WITH NAION (THINK IMAGING)

NAION NAION Kanski’sNAION

Kanski’s P’edema Kanski’s Arteritic AION

NAION NAION K’sAcuteEst.P’edema

Kanski’s Bur. Drusen Kanski’s Hypoplastic

References• Liu: NeuroOphthalmology• Biousse: NeuroOphthalmology Illustrated• Dr. Richard Castillo Northeastern State University• Kanski: Illustrated Tutorials in Clinical Ophthalmology• Walsh and Hoyt: the Essentials• Daroff: Bradley’s Neurology in clinical practice• Firestein: Kelley's Textbook of Rheumatology• Keane: “Multiple Cranial Nerve Palsies” 2005• Purvin: “Neuro-Ophthalmic Emergencies for the Neurologist” 2005• Glaser in Duane’s: “Topical Diagnosis: Prechiasmal Visual Pathways

Mendrinos: “Ocular Ischemic Syndrome” 2010