2. 46 yo man, Fijian-Indian heritage
6 Feb: family doctor with 2 week history
myalgia
nausea and loss of appetite
blurred vision
Diagnosed as viral illness
Told to increase his thyroxine dose
3. Presents to Eye Emergency Department
blurred vision when reading
vision “fading in and out” in his left eye
denies recent viral illness
denies current systemic symptoms
no headache
no pain on eye movement
4. Past ocular history
myopic
colour blind
Past medical history
Hashimotos thyroiditis
thalassaemia trait
lowVit D
Medications
thyroxine
5. Family history
thalassaemia trait
no vision loss
Social history
works in office, lots of computer work
no cigarettes or alcohol
normal balanced diet
6. RIGHT
6/6
full eye movements
Anterior segment
normal
Dilated fundus exam
normal
LEFT
6/18
50% red desaturation
no RAPD
Anterior segment
normal
Dilated fundus exam
normal
HR 69 reg, BP 100/68, temp 36.8C
7.
8. Resident review
no obvious ocular cause
? bitemporal inferior quadrant field defect
CT scan
normal
Referred for routine clinic follow-up
“left optic neuritis”
9. returns to Eye Emergency
no longer feels safe to drive
myalgia, arthralgia and nausea returned
no neurological symptoms
no headache
no pain on eye movement
10. RIGHT
6/21
full eye movements
Anterior segment
normal
Dilated fundus exam
normal
LEFT
6/120
no red desaturation
no RAPD
Anterior segment
normal
Dilated fundus exam
normal
HR 72 reg, BP 105/70, temp 36.7C
11. Patient didn’t want to wait in emergency for
repeat work-up
very busy at work
Advised not to drive!
Booked for neuro-ophthalmology clinic 2
days later
12. Failed to attend neuro-ophthalmology clinic
patient contacted
really very busy at work
13. Comes back to Eye Emergency
vision is so blurred he can’t work anymore
gradual progression of vision loss
intermittent mild headache
loss of appetite
difficulty sleeping
14. RIGHT
6/120
no RAPD
Anterior segment
normal
Dilated fundus exam
normal
LEFT
Count Fingers at 30cm
no red desaturation
full EOMS, pain in left
Anterior segment
normal
Dilated fundus exam
? slight disc hyperaemia
HR 70 reg, BP 100/70, temp 36.7C
15.
16. Call for a neuro-ophthalmology consult
Admit
Blood tests – inflammatory, infective work-up
Chest X-ray
Lumbar puncture
1g IV methyprednisolone daily
MRI brain/orbits with gadolinium
ordered - outside institution
17. Vision 1/60 right, count fingers left
Fields to confrontation
left central scotoma
right hemi-field red desaturation
Poor pupil response to light OU
0.3 log-unit left RAPD
Cranial nerves normal
Upper and lower limb exam – normal
Anterior and posterior segments - normal
18. full blood count – normal
renal function – normal
liver function – normal
ESR 2, CRP 0.5
chest X-ray – no evidence ofTB or sarcoid
Lumbar puncture - normal opening pressure
and basic constituents
19. “Atypical optic neuritis”
46 year old man
Fijian-Indian
nausea, sleep disturbance
Long lesion suspected
left optic nerve intra-cranially
extending to left optic tract (right hemifield)
neuromyelitis optica until proven otherwise
Ddx: sarcoid
20. anti-AQP4 (NMO) results = 2 weeks
MRI scan can only be done next week
Do you:
continue IVMP?
move rapidly to plasma exchange?
▪ based on clinical diagnosis of NMO
▪ PLEX started within 15-20 days = best outcome
Magana S et al. Beneficial plasma exchange response in central nervous system inflammatory
demyelination. Arch Neurol 2011; 68(7): 870-8
21. 2 days IVMP – no change in vision
Transferred to general hospital
5 days plasma exchange
1g IV methylprednisolone continued 5 days
22.
23.
24.
25.
26.
27.
28. RIGHT
6/60
full EOMs
Anterior segment
normal
Dilated fundus exam
normal
LEFT
6/90
0.3 log-unit RAPD
Anterior segment
normal
Dilated fundus exam
normal
Oral prednisolone 60mg taper, azathoprine increasing to 150mg
29. 1 week after discharge
repeat MRI brain – reduction in lesion size and
intensity
NMO antibody positive
32. 18 months with no relapses
Back at work full-time
Able to return to driving
Final medication:
mycophenolate 500mg BD
thyroxine
Vit D supplement
34. Patient subgroups MS is rare: African, Asian
30s+
Bilateral – simultaneous or sequential loss
Progresses for > 2 weeks
Severe pain > 2 weeks since onset
Require steroids to induce recovery
Cannot apply the findings of the Optic
NeuritisTreatmentTrial
35. Useful after first episode of transverse
myelitis, severe or recurrent ON
Serum autoantibody Aquaporin-4 (AQP4)
One of the major water channel proteins
Sensitivity 50-80% (cell-based higher than ELISA)
Specificity 90-100%
TrebstC et al. Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the
meuromyelitis optica study group (NEMOS). J Neurol 2013; 261: 1-16.
36. Optic neuritis
Transverse myelitis
Within 2 years of each other
Plus 2 of:
Brain MRI non diagnostic for MS
Spinal cord lesion > 3 vertebral segments
Seropositive for NMO-ab (anti-AQP4)
Wingerchuk D et al. Revised diagnostic criteria for neuromyelitis optica. Neurology 2006; 66(10): 1485-9
37. AQP4-IgG positive longitudinally extensive
transverse myelitis
AQP4-IgG positive recurrent or bilateral optic
neuritis
Wingerchuk D et al.The spectrum of neuromyelitis optica. Lancet Neurol 2007; 6(9):805-15
38. Poor prognosis without treatment
= medical emergency
5 days IV methylprednisolone
Plasmapheresis
immediate improvement 50%
improvement in 6 months in 78%
1mg/kg oral prednisolone – slow taper
Immunosuppression (azathioprine)
Kim S et al. Clinical efficacy of plasmapheresis in patients with neuromyelitis optica spectrum
disorder and effects on circulating anti-aquaporin 4 antibody levels. J Clin Neurol 2013;9(1): 36-42
39. Multiple sclerosis ON Neuromyelitis optica ON
Race Caucasians Asians,Africans
Eye Unilateral Bilateral or sequential
Recovery Spontaneous Poor without steroids
Course of optic neuritis Less severe Profound vision loss
Neurological Varied CNS signs Transverse myelitis
Overall prognosis Good recovery Poor recovery without Rx