This document discusses the evaluation and differential diagnosis of a patient presenting with a swollen optic disc. The 58-year-old male patient woke up with spots in his vision in the left eye and was found to have unilateral disc swelling upon examination. Key points addressed include ruling out giant cell arteritis with urgent bloodwork and evaluating for signs of increased intracranial pressure. The differential diagnosis discussion centers on anterior ischemic optic neuropathy versus papilledema. Factors supporting NAION include the patient's risk factors of diabetes and hypertension as well as the clinical features matching NAION such as unilateral involvement and visual field defects. Close monitoring over subsequent months tracked improvement consistent with NAION.

1. Swollen Optic Disc Presentation
Northeastern University
10/31/12

2. • 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.

3. 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 Signs
4. Image posterior pole
5. schedule the VF and F/U appt
6. Educate “Swollen Optic Disc”/ER visit possible
7. Get release of information for PCP’s note/etc
8. ESR/CRP within a few hours

4. Valerie Biousse’s Neuro-Ophthalmology
Anterior 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, constriction
Disc edema almost
always bilateral
____________________________
SYSTEMIC SIGNS:
Other symptoms or signs of
increased
ICP, HA, Nausea, Vomiting, Dip
lopia, 6th nerve palsy, Pulsatile
Tinnitus, TVO’s,(Fever,Seizure,
Stiffness)
(OR >1 CN DAMAGED)

5. Grant Liu’s NeuroOphthamology
Table 6–1 Differential
diagnosis of a swollen optic
disc: causes according to
frequency
Most common
Papilledema BILATERAL
Optic neuritis PAINMRI
Anterior ischemic optic
neuropathy (GCAPAIN)
Pseudopapilledema
Common
Central 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 -YOUNG
Optic nerve infiltration by
sarcoidosis PAIN MRI
lymphoma
leukemia
plasma cell dyscrasia
ADDRESSED BY HISTORY

6. Grant Liu’s NeuroOphthamology
Table 6–1 Differential
diagnosis of a swollen optic
disc: causes according to
frequency
Most common
Papilledema
Optic neuritis
Anterior ischemic optic
neuropathy
Pseudopapilledema CHARACT
Common FINDINGS
Central retinal vein occl-RET
Diabetic papillopathy-RET
Uncommon
Ocular hypotony-IOP
Intraocular inflammation
(uveitis) - CELLS
Malignant hypertension BP
Optic 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

7. Grant Liu’s NeuroOphthamology
Table 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.

8. Differential Diagnosis
• AION – Most Common
• In order search for NEOPLASIA  IMAGING
WHICH YOU MUST PURSUE YOURSELF
-------------------------------------------------------
LOOKING AT AION:
1. GCA
2. NAION

9. 1. GCA
• is the most common form of
systemic vasculitis in adults
• its most feared complication
is irreversible loss of vision
(like Pseudo. Cerebri)

10. three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.
Vasculitis PLUS any 3of 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

11. three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.
Vasculitis PLUS any 3of 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.

12. three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.
Vasculitis PLUS any 3of 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

13. 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

14. 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
Artery
Disease)
FULL SPECTRUM OF GCA’s
VISION FINDINGS
(NAION)

15. 2. NAION

16. NAION
• Pathogenesis: unknown
• majority 60-70yo but could be any age
• Caucasian>African American or Hispanic
American
• Increased Risk in DM, high Cholesterol, HTN

17. 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

18. 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?

19. 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.

20. 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

21. 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

22. 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

23. Brief Mention about…
…VA’s
20/20 OU throughout
…Macula’s:
No macular edema throughout
…IOP’s:
IOP was unremarkable throughout

24. 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 spots
No HA, scalp tenderness, jaw
claudication, or new onset neurological
deficit

25. -18
-16
-14
-12
-10
-8
-6
-4
-2
0
0 1 2 3 4 5 6 7 8 9 10
RIGHT EYE
LEFT EYE
MONTHS in 2012
MD
Of
VF
~Altitudinal defects
W/ CENTRAL SPARING

26. 0
50
100
150
200
250
300
350
400
450
0 1 2 3 4 5 6 7 8 9 10
OCT thickness measures of RIGHT EYE
um
Inferior rim
Superior rim
SECTORAL DISC INVOLVEMENT
HYPEREMIC SWELLING (HEMES)

27. 0
50
100
150
200
250
300
350
400
450
0 1 2 3 4 5 6 7 8 9 10
OCT thickness measures of LEFT EYE
um
Inferior rim
Superior rim
SECTORAL DISC INVOLVEMENT
HYPEREMIC SWELLING/HEMES

28. Superior rim
right eye
0
50
100
150
200
250
300
350
400
450
0 1 2 3 4 5 6 7 8 9 10
SUSPECTED
Inferior rim
of left eye
4-5 mos

29. 0
50
100
150
200
250
300
350
400
450
0 1 2 3 4 5 6 7 8 9 10
Inferior rim
of left eye
Superior rim
right eye
-18
-16
-14
-12
-10
-8
-6
-4
-2
0
0 1 2 3 4 5 6 7 8 9 10
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)

30. NAION NAION Kanski’sNAION
Kanski’s P’edema Kanski’s Arteritic AION

31. NAION NAION K’sAcuteEst.P’edema
Kanski’s Bur. Drusen Kanski’s Hypoplastic

33. 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