This document describes the case of a 46-year-old man who presented with vision loss. He was initially diagnosed with viral illness but his vision continued to deteriorate. He was ultimately diagnosed with neuromyelitis optica (NMO) based on clinical features and a positive NMO antibody test. He received treatment including steroids and plasma exchange which improved his vision. His condition was well-controlled with immunosuppression medications.

1. Dr Clare Fraser

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

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

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

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

30.  21 March: (+ 6 weeks)
 VAR 6/18VAL 6/18
 2 May: (+ 3 months)
 VAR 6/9VAL 6/9
 14 Nov: (+ 9 months)
 VAR 6/7.5VAL 6/7.5

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

33. Neuromyelitis optica

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