Despite improved recognition and diagnosis of neuromyelitis spectrum disorder (NMOSD),
management remains a challenge. Patients of African/Asian ethnicity and those with
a clinical phenotype involving the brainstem/medulla probably are most at risk for
disease progression and/or death. Factors that determine mortality risk include age
at diagnosis, relapse rates and poor recovery from attacks (
Kitley et al., 2012
). No data exists regarding the prognosis or treatment of poorly responsive seronegative
NMOSD. Plasma exchange (PLEX) therapy and IV steroids are used to treat relapses regardless
of antibody status, but chronic management of seronegative NMOSD remains undefined.
Rituxan is likely more efficacious in NMOSD patients compared to mycophenolate mofetil
(MMF) in both seropositive or negative NMOSD (
- Kitley J.
- Leite M.I.
- Nakashima I.
- Waters P.
- McNeillis B.
- Brown R.
- Takai Y.
- Takahashi T.
- Misu T.
- Elsone L.
- Woodhall M.
- George J.
- Boggild M.
- Vincent A.
- Jacob A.
- Fujihara K.
- Palace J
Prognostic factors and disease course in aquaporin-4 antibody-positive patients with
neuromyelitis optica spectrum disorder from the United Kingdom and Japan.
Brain. 2012; 135: 1834-1849
Poupart et al., 2020
) but the approval of Eculizumab (Solaris, Alexion) for AQP4 seropositive patients
adds complexity to treatment options. The recently approved CD19-directed humanized
monoclonal antibody, inebilizumab (Uplinza) is only indicated for patients with APQP4
Ab seropositive status although it was also tried in seronegative patients with inconclusive
results.- Poupart J.
- Giovannelli J.
- Deschamps R.
- Audoin B.
- Ciron J.
- Maillart E.
- Papeix C.
- Collongues N.
- Bourre B.
- Cohen M.
- Wiertlewski S.
- Outteryck O.
- Laplaud D.
- Vukusic S.
- Marignier R.
Zephir H on behalf of the NOMADMUS study group. Evaluation of efficacy and tolerability
of first-line therapies in NMOSD.
Neurology. 2020; 94: e1645-e1656
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References
- Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan.Brain. 2012; 135: 1834-1849
- Zephir H on behalf of the NOMADMUS study group. Evaluation of efficacy and tolerability of first-line therapies in NMOSD.Neurology. 2020; 94: e1645-e1656
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Article info
Publication history
Published online: July 03, 2020
Accepted:
July 1,
2020
Received in revised form:
June 30,
2020
Received:
June 27,
2020
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
