case study of optic neuritis rule out multiple sclerosis, neuromyelitis optica interesting presentation of the disease

1. INTERDEPARTMENT
Ophthalmology & Neuro-Medicine

2. Case
• A 18-year-old female
• History of optic neuritis RE (1 yr)

3. Chief Complaint
• Left eye pain 1 day

4. present illness
1 yr PTA : She was admitted to PMK hospital
(10-16 Aug 2016) with acute visual loss RE (RE
VA FC'2), no clinical eye pain/pain on eye
movement, no floater, no flashing, no Hx of
head/eye trauma, No Hx ocular Sx , she had
paresthesia both legs, no
respiratory/bowel/bladder symptoms

5. OD OS
VA FC'2 PH not improved 20/80-1 PH 20/40-1
EOM full, no pain on eye movement full, no pain on eye movement
IOP 12 16
Lid Not swelling Not swelling
Conjunctiva No injected, No discharge Not injected, no discharge
Cornea Clear Clear
A/C Deep/Quiet Deep/Quiet
Iris and Pupil Round, 3 mm RTL Round, 3 mm RTL
RAPD Positive RE
Lens Clear Clear
Vitreos No AV cell /scatter vitreous
clumping
No AV cell / scatter vitreous
clumping
Fundus C:D 0.3, blurred disc margin, A:V
2:3, macular normal foveal reflex,
scatter whitish retinal infiltration,
sheathing vessels
C:D 0.3, A:V 2:3, macular normal
foveal reflex, scatter whitish retinal
infiltration, sheathing vessels
10 Aug 2016
Ocular examination

6. Fundus photography
10 Aug 2016

7. Disc photography
10 Aug 2016

8. Impaired color vision RE
10 Aug 2016
Color vision

9. Diffuse visual field defect RE
10 Aug 2016
CT VF 24-2
10 Aug 2016

10. MRI brain c
orbit
Right orbit seen enhancing optic
10 Aug 201610 Aug 2016

11. MRI spine
ill-defined multifocal intramedullary lesion C3 to
T10
15 Aug 2016 15 Aug 2016

12. OCT disc
• Poor signal strength BE
10 Aug 2016

13. Lab
WBC Hb Hct PLT N L
CBC 7800 13.5 38.5 168000 68.3 28
BUN Cr K Na Cl CO2 hs-CRP
9.9 0.7 3.88 142 103.2 22.8 0.36
pH WBC RBC epi
UA 5.5 0-1 2-3 0-1
10 Aug 2016

14. Lab
Anti nuclear antibody Positive
Homogeneous1:1280
Speckle1:1280
Nuclear Dot 1:80
Anti-Toxoplasma gondi (IGM)
(ELISA)
Negative
Anti-Toxoplasma gondi (IGM)
(ELISA)
0.1
Anti-dsDNA-NcX(Quantitative) 3.2
Anti-dsDNA-NcX Negative
C3 1
C4 0.2
Anti- HIV Non-Reaction
VDRL Non-Reaction
TPHA Negative
10 Aug 2016

15. CSF protein sugar WBC RBC PMN Mono
CBC 38 64 6 - - -
NMO igG (CSF,serum) :negative
Oligoclonal band in CSF : positive
Quantiferon-TB : Negative
VEP : Slightly abnormal RE
Lab10 Aug 2016

16. Diagnosis
• Optic neuritis RE (Ddx NMO or MS)
• Intermediate uveitis BE (phlebitis R/O pars
planitis)
• R/O Autoimmune disease

17. Treatment
•Methylprednisolone 750 mg IV OD * 3
days(11-13 Aug 20/6)
-clinical improved 3 days(VA RE20/125) and
color vision normal
•pred (5) 7*1 oral(11days)

18. Progress note
Date VA RAPD
24 Aug 2016 RE : Fc' CC 20/200
LE : 20/40 PH not
positive RE
-Pred(5) 7*1
CTVF 24-2 : normal BE
26 Aug 2016 RE : 20/125 PH20/40 positive RE
23 Sep 2016 RE : 20/200 PH20/50 positive RE
28 Sep 2016 RE : 20/200 PH20/40 Negative
27/10/2016 -MRI brain c orbit c spine : slightly
enhancing of optic nerve RE
-MRI spine : No significant change of
lesion
1 Feb 2017 RE 20/125 PH not Negative
CTVF 24-2 : normal BE
color vision : normal BE
14 June 2017 RE 20/125 PH 20/63 Negative

19. MRI spine
• No significant change of lesion
27 Oct 2016
27 Oct201627 Oct2016

20. Present illness
1 day PTA : She had left eye pain and pain on eye
movement and suddenly blurred vision, no floater, no
flashing, no history of head/eye trauma, no history of
ocular surgery, no paresthesia/paralysis, no respiratory
symptom, no bowel/bladder symptom

21. Systemic review
• No fever
• No headache / severe dizziness / seizure /
unconsciousness / depression or mental illness
• No tinnitus
• No oral ulcer/severe sore
throat/tonsilitis/sinusitis/running nose/ abscessed teeth
• No UTI/proteinurea/hematurea/genital herpes
• No muscular pain/arthritis/stiffness/severe back pain
• No alopecia/poliosis/vitilligo/rash

22. Personal History
• No Smoking
• No ever eaten raw meat / unpasteurized milk / dirty
fruit or veggies
• No contact cat / dog / soil
• No insect bite
• No Hx of contact TB/chronic cough

23. Ocular examination
OD OS
VA 20/160 PH 20/100 20/160 PH 20/50
EOM full, no pain on eye movement full, pain on eye movement
IOP 19 17
Lid Not swelling Not swelling
Conjunctiva Mild injected, no discharge Not injected, no discharge
Cornea Clear Clear
A/C Deep/Quiet Deep/Quiet
Iris and Pupil Round, 3 mm RTL Round, 3 mm RTL
RAPD Positive LE
Lens Clear Clear
Vitreos AV cell trace/scatter snowball AV cell trace / scatter snowball
Fundus C:D 0.3 pale disc (temporal), A:V 2:3,
macular dull reflex, no cws , no
hemorrhage, no sheathing vessles
C: C:D 0.3 hyperemia,blurred discA:V
2:3, macular dull reflex, no cws , no
hemorrhage, no sheathing vessles
1 Sep 2017

24. Fundus photography
1 Sep 2017

25. Disc photography
1 Sep 2017

26. Problem Lists
• Young Thai female
• Acute painful visual loss
What is your DDX?

27. CTVF 24-2
• Left superior altitudinal VF defect
1 Sep 20171 Sep 2017

28. Color vision
• Normal color vision BE
1 Sep 2017

29. MRI brain c orbit
LE orbit seen enhancing optic nerve
No abnormal brain parenchyma
7 Sep 20177 Sep 2017

30. MRI spine
• No spine lesion
7 Sep 2017

31. Lab
WBC Hb Hct PLT N L
CBC 7600 12.6 38.3 238000 69.7 25.4
BUN Cr K Na Cl CO2 hs-CRP
10.2 0.66 3.38 139 101.6 26.1 1.3
pH WBC RBC epi
UA 6 0-1 0-1 0-1
1 Sep 2017

32. CSF protein sugar WBC RBC PMN Mono
CBC 38 71 6 43 - -
CSF c/s: No growth
NMO igG (CSF,serum) :negative
Oligoclonal band in CSF : positive
VEP : Abnormal RE
Lab
1 Sep 2017

33. Lab
ENA Profile plus 1 Positive
Anti-Jo-1 Negative
Anti-Ro-52 Negative
Anti-SCL70 Negative
Anti-RNP/Sm Positive 1+
Anti-SSA Negative
Anti-SSB Borderline
Anti-Sm Negative
Anti- HIV Non-Reaction
VDRL Non-Reaction
TPHA Negative
1 Sep 2017

34. Impression
• Recurrent optic neuritis LE (Ddx MS or
NMO)
• Intermediate uveitis BE (phlebitis R/O pars
planitis)
• R/O Autoimmune disease (SLE)

35. Treatment
•Methylprednisolone 2 gm IV OD * 3 days(1-
3Sep 2017)
-clinical improved 3 days
•Prednisolone (5) 7*1 oral(11days)

36. Progress note
Date BCVA EOM
Eye pain/ Pain
on eye
movement
RAPD Tx
4 Sep 2017 RE : 20/63
LE : 20/32
full No positive LE -Pred(5) 7*1
- Disc Photo : No change
-CTVF 24-2 : Left
Superotemporal VF
defect( Compare
1/9/2017)
- OCT disc : poor signal
strength
13 Sep 2017 RE : 20/50
LE : 20/25
full No positive RE -Pred(5) 7*1
-Disc Photo and
-OCT disc : No change

37. Disc photography
4 Sep 2017

38. Disc photography
13 Sep 2017

39. Left Superotemporal VF defect
( Compare 1/9/2017)
4 Sep 2017
CT VF 24-2
4 Sep 2017

40. OCT disc
4 Sep 2017

41. OCT discOCT disc
13 Sep 2017

42. DISCUSSION

43. Female
Young
History of optic neuritis RE
History of limb weakness
VF defect
Positive RAPD in each attack of effected eye
Color vision defect
Rapid VA gain after an attack
Positive finding on MRI
Positive result of oligoclonal band test
Negative result of NMO IgG
Presence of intermediate uveitis BE
Positive finding

44. Is it just an ordinary Optic neuritis?
Or Is it NMO!?
or Is it Optic neuritis with MS!?

45. OPTICNEURITIS
Optic Neuritis is a clinical diagnosis that is made when
a patient presents with
CLINICAL PRESENTATION PRESENC
E
pain with eye movement and/or periorbital discomfort
RAPD
visual field defect
optic nerve swelling (35% anterior disc edema, 65%
retrobulbar)
unilateral decreased visual acuity

46. OPTICNEURITIS
1.optic neuritis is a clinical diagnosis,
2.typical (unilateral vision decrease, pain with eye movement) optic neuritis will
recover with good visual prognosis by 6-12 months,
3.the final amount of vision recovery is independent of treatment with or without IV
IV steroids
4.oral prednisone alone is CONTRAINDICATED
5.MRI of the brain is a must after an initial episode of optic neuritis

47. OPTICNEURITIS
Up to 75% of MS patients have at least 1 episode of optic neuritis
The ONTT showed that even without any lesions present
on MRI, there still was a 16% chance of developing MS in
5 years, 22% in 10 years and 25% in 15 years.
At 10 years, the overall risk of MS was 38% after an initial episode of
optic neuritis. That risk is 56% if the MRI had 1 or more lesions.

48. OPTICNEURITIS
At 15 years, the overall risk of MS was 50% after an initial episode of
optic neuritis and strongly related to presence of lesions on a
baseline non-contrast-enhanced MRI of the brain.
No lesion on MRI brain = 25% chance of
developing MS
1 or more lesions on MRI brain = 72% chance of
developing MS

49. Talking about first attack
NMO
NMOSD without AQP4-IgG or NMOSD with unknown AQP4-IgG
status
1. At least 2 core clinical characteristics : Optic neuritis +LETM
acute myelitis (disseminated in space)
2. Negative test for AQP4-IgG from best detection method
3. Exclusion of alternative diagnosis

50. NMO Talking about first attack
Red flags (clinical/laboratory)
• Presence of CSF oligoclonal bands (occur in
<20% of NMO vs >80% of MS)

51. NMO Talking about first attack

52. MS
SPACE TIME
Talking about first attack

53. MS Talking about first attack
SPACE TIME
She had negative MRI finding after follow up
approximately 30 days from attack

54. Talking about second attack
• This time she only has left optic nerve enhancement on
MRI
• No spine lesion on MRI
• Negative NMO IgG in serum and CSF
• Positive oligoclonal band
NMO

55. MS
SPACE TIME
Talking about second attack

56. • Uveitis is 10 times more common in MS patients
than in general population
• 30% of MS patients have uveitis
• Intermediate uveitis is the most common
manifestation of MS-associated uveitis
• 95% are bilateral
• Most patients develop mild vitritis with
periphlebitis
Chen L, GordonLK. Ocularn manifestations of multiple sclerosis. Curr Opin Ophthalmol.2005;16(5):315-320.
Zein G, Berta A, Foster CS. Multiplesclerosis-associated uveitis. OculImmunol Inflamm. 2004;12(2):137-142.
Evidence supporting MS theory

57. • Immunological abnormalities in the CSF are common in patients with
optic neuritis
• Frederiksen et al : abnormal CSF were present in 79% of 45 patients
with isolated optic neuritis which 69% were oligoclonal bands
• Soderstrom : oligoclonal bands were present in 69% of patients with
optic neuritis
• Predictive value of CSF oligoclonal bands for the development of CDMS
within 5 years after optic neuritis event : the presence of
oligoclonal bands was associated with the development of
CDMS (P=0.02) which was useful when MRI brain was
normal
Optic neuritis + oligoclonal bands from
ONTT

58. • CDMS developed in 42% of patients with optic neuritis+positive
oligoclonal bands test
• CDMS developed in 16% of patients with optic neuritis+negative test
result
• (Odds ratio 3.88 CI= 1.18,13.86 P=0.02)
Optic neuritis + oligoclonal bands from
ONTT

59. • Among 39 patients with normal MRI brain, CDMS developed in 3 of 11
(27%) patients with oligoclonal bands present but in only 1 of 28% (4%)
without oligoclonal bands
• Among 37 patients with abnormal MRI brain, CDMS developed in 13 of
27 (48%) with oligoclonal bands and in 5 of 10 (50%) without oligoclonal
bands
Optic neuritis + oligoclonal bands from
ONTT

60. THANK
YOU