1 research outputs found
Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome
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