Short Term Effect of Additional Apheresis on Visual Acuity

Changes in Patients With Steroid Resistant Optic Neuritis in

Neuromyelitis Optica Spectrum Disorders

Presented by : dr. Jehan Fauzi Rakhmandani

Supervised by : dr. Tatang Talka Gani, Sp.M (K)., M. Kes

Japanese Journal Of Ophtalmology, 2018.

Background
• Recent studies find that Plasma Exchange is effective in
suppressing acute attacks in patients with NMO and other CNS
demyelinating diseases that are unresponsive to HIMP.
Background
Plasma Exchange and Immunoabsorption
are metode of apharesis.
Investigated the temporal course of
Found that half of The patients
improvement of Expanded Disability
who were unresponsive to HIMP
Status Scale with acute CNS
improved immediately after
demyelinating diseases before and
initiation of PE (Kim et al,2013)
after PE at 1, 6, and 12 months.
Objective
• To evaluate temporal changes in visual acuity in patients with
steroid-resistant optic neuritis (ON) in neuromyelitis optica
spectrum disorders (NMOSD) after apheresis.
Material and Method
Retrospective review the Medical Record ( Kobe University Hospital )

15 eyes of 9 consecutive patients

seropositive for anti-AQP4 antibody

March 2010-September 2017

1 g of methylprednisolone per day for 3 days before apheresis

The patients received a median of 5.1 simple PEs (range, three to seven).

Excluded : intravenous immunoglobulin (IVIG) for the treatment of

ON

For analysis, visual acuity values were converted to the log of the
minimum angle of resolution (logMAR).
After Apheresis
3 of 9 patients were administered
a combined therapy of a low
6 of 9 patients were administered
dose of prednisolone with 50 mg
a low dose of prednisolone (10–
azathioprine (2 patients) or 5 mg
15 mg)
tacrolimus hydrate (1 patient) to
prevent a recurrence.

However, recurrence of ON was

noted in 2 of 13 eyes (15%)
within 3 months.
Discussion

Compared the visual outcome

Additional apheresis effectively
Half of the Patients responsive of ON relapses associated with
improved the visual acuity of
to apheresis show NMO in 16 patients treated
patients who had AQ4 antibody
improvement > 0,3 early ( 1 with apheresis plus HIMP and
positive and were to resistant
week ) 36 patients treated with HIMP
to HIMP.
alone. ( Merle, et al, 2012 )
Conclusion
• Twelve eyes (80%) showed improvement with logMAR > 0.3 at
1 month after apheresis.
• There after visual acuity became stable for the 11 eyes, until 12
months. However, two eyes (12%) showed recurrence of visual
acuity reduction 3 months .
• Additional Apheresis therapy rapidly improvements the visual
acquity of Steroid-resitant seropsotive AQ 4 ON.
 Thank You
Advices and suggestions are very welcome
Diagnostic criteria for NMOSD without AQP4-IgG or antibody status unknown
1. At least 2 core clinical characteristics meeting all of the following characteristics

a. At least 1 core clinical c. Fulfillment of additional MRI

characteristic must be optic
neuritis, acute myelitis with
longitudinally extensive
transverse myelitis (LETM), or
area postrema syndrome
b. Dissemination in space (2 or
more different core clinical
characteristics)
requirements :
i. Acute optic neuritis: brain MRI
shows normal findings or
nonspecific white matter
lesions; OR optic nerve MRI
demonstrates T2-hyperintense
lesion or T1-weighted
gadolinium enhancing lesion
extending over at least half the
optic nerve length or
involving optic chiasm
ii. Acute myelitis: spine MRI lesion
extending over at least 3 contiguous
segments
(LETM)
iii. Area postrema syndrome:
associated dorsal medulla lesions
iv. Acute brainstem syndrome:
associated periependymal
brainstem lesions

2. Negative test for AQP4-IgG, or testing unavailable

3. Exclusion of alternative diagnoses

 Patofisiologi

• AQP4-IgG masuk via BBB 

selective binding AQP4  ggn
homeostasis air pd astrosit 
aktivasi komplemen  
permeabilitas BBB dan infiltrasi
leukosit,sebagian eosinofil dan
neutrofil  kematian astrosit,
oligodendrosit,neuronal dan
demielinisasi
Core Clinical Typical Presentation Seropositive Seronegative Additional Imaging Requirements
Presentation Criteria Criteria (Seronegative Cases Only)
Optic neuritis Bilateral At least one ON with (1) normal MR imaging of
(ON) VA ,Severe core brain or non spesific white matter
Poor recovery At least one presentation lessions or (2) T2 or T1 GAD-
painless core enhancement of optic chiasma or at
presentatio least ½ optic nerve length on MRI
orbit
n -
Acute myelitis Bilateral Longitudinal ly extensive spinal cord
Motoris, Sensoris lesion (or atrophy) ≥ 3 vertebrae
At least one
Spincter involvement segments on MRI
- others core
Persistent vomiting
Area posttrema presentation Area posttrema lesion on MRI imaging
syndrome Nausea, hiccoughs of brain
Acute brainstem Cranial palsies - Periependymal brainstem lesion on
syndrome Ataxia Positive MR imaging of brain
Limb weakness AQP4 Additional
Dienchepalic Narcolepsy , antibodies imaging
syndrome Hypothermia requirement
DIAGNOSTIC
Daytime somnolence
obesity
s as listed CRITERIA NMOSD
Cerebral syndrome Encephalopathy IPND 2015
seizure