TY  - JOUR
A1  - Jarius, Sven
A1  - Ruprecht, Klemens
A1  - Kleiter, Ingo
A1  - Borisow, Nadja
A1  - Asgari, Nasrin
A1  - Pitarokoili, Kalliopi
A1  - Pache, Florence
A1  - Stich, Oliver
A1  - Beume, Lena-Alexandra
A1  - Hümmert, Martin W.
A1  - Ringelstein, Marius
A1  - Trebst, Corinna
A1  - Winkelmann, Alexander
A1  - Schwarz, Alexander
A1  - Buttmann, Mathias
A1  - Zimmermann, Hanna
A1  - Kuchling, Joseph
A1  - Franciotta, Diego
A1  - Capobianco, Marco
A1  - Siebert, Eberhard
A1  - Lukas, Carsten
A1  - Korporal-Kuhnke, Mirjam
A1  - Haas, Jürgen
A1  - Fechner, Kai
A1  - Brandt, Alexander U.
A1  - Schanda, Kathrin
A1  - Aktas, Orhan
A1  - Paul, Friedemann
A1  - Reindl, Markus
A1  - Wildemann, Brigitte
T1  - MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome
T2  - Journal of Neuroinflammation
N2  - Background
A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG).

Objective
To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes.

Methods
Retrospective multicenter study.

Results
The sex ratio was 1:2.8 (m:f). Median age at onset was 31 years (range 6-70). The disease followed a multiphasic course in 80% (median time-to-first-relapse 5 months; annualized relapse rate 0.92) and resulted in significant disability in 40% (mean follow-up 75 ± 46.5 months), with severe visual impairment or functional blindness (36%) and markedly impaired ambulation due to paresis or ataxia (25%) as the most common long-term sequelae. Functional blindness in one or both eyes was noted during at least one ON attack in around 70%. Perioptic enhancement was present in several patients. Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70%). Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44%. Fourty-one percent had a history of simultaneous ON and myelitis. Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50%; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one). CSF pleocytosis (partly neutrophilic) was present in 70%, oligoclonal bands in only 13%, and blood-CSF-barrier dysfunction in 32%. Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal. Full recovery was achieved by plasma exchange in some cases, including after IVMP failure. Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids. Methotrexate was effective in 5/6 patients. Interferon-beta was associated with ongoing or increasing disease activity. Rituximab and ofatumumab were effective in some patients. However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion. Coexisting autoimmunity was rare (9%). Wingerchuk’s 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28%, 32%, 15%, 33%, respectively; MS had been suspected in 36%. Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases.

Conclusion
Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD. The predominantly relapsing and often severe disease course and the short median time to second attack support the use of prophylactic long-term treatments in patients with MOG-IgG-positive ON and/or myelitis.
KW  - Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG)
KW  - Aquaporin-4 antibodies (AQP4-IgG, NMO-IgG)
KW  - Optic neuritis
KW  - Transverse myelitis
KW  - Longitudinally extensive transverse myelitis
KW  - Magnetic resonance imaging
KW  - Autoantibodies
KW  - Neuromyelitis optica spectrum disorders (NMOSD)
KW  - Cerebrospinal fluid
KW  - Oligoclonal bands
KW  - Electrophysiology
KW  - Evoked potentials
KW  - Treatment
KW  - Therapy
KW  - Methotrexate
KW  - Azathioprine
KW  - Rituximab
KW  - Ofatumumab
KW  - Interferon beta
KW  - Glatiramer acetate
KW  - Natalizumab
KW  - Outcome
KW  - Pregnancy
KW  - Infections
KW  - Vaccination
KW  - Multiple sclerosis
KW  - Barkhof criteria
KW  - McDonald criteria
KW  - Wingerchuk criteria 2006 and 2015
KW  - IPND criteria
KW  - International consensus diagnostic criteria for neuromyelitis optica spectrum disorders
Y1  - 2016
UR  - https://opus.bibliothek.uni-wuerzburg.de/frontdoor/index/index/docId/16557
UR  - https://nbn-resolving.org/urn:nbn:de:bvb:20-opus-165570
VL  - 13
IS  - 280
ER  -