Late‐onset Neuromyelitis Optica Spectrum Disorder with Anti‐AQP4 and Anti‐MOG Antibodies

ObjectiveTo describe the clinical features of late-onset (≥50 years) neuromyelitis optica spectrum disorder (LO-NMOSD), to compare the outcome with that of early-onset (EO-NMOSD), and to identify predictors of disability.MethodsA retrospective, multicenter study of 238 patients with NMOSD identified by the 2015 criteria. Clinical and immunologic features of patients with LO-NMOSD were compared with those with EO-NMOSD. All patients were evaluated for aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) antibodies.ResultsSixty-nine (29%) patients had LO-NMOSD. Demographic features, initial disease presentation, annualized relapse rate, and frequency of AQP4-IgG and MOG-IgG did not differ between patients with LO-NMOSD and EO-NMOSD. Among patients with AQP4-IgG or double seronegativity, those with LO-NMOSD had a higher risk to require a cane to walk (hazard ratio [HR], 2.10, 95% CI 1.3–3.54, p = 0.003 for AQP4-IgG, and HR, 13.0, 95% CI 2.8–59.7, p = 0.001, for double seronegative). No differences in outcome were observed between patients with MOG-IgG and LO-NMOSD or EO-NMOSD. Older age at onset (for every 10-year increase, HR 1.63, 95% CI 1.35–1.92 p < 0.001) in NMOSD, and higher disability after the first attack (HR 1.68, 95% CI 1.32–2.14, p < 0.001), and double seronegativity (HR 3.74, 95% CI 1.03–13.6, p = 0.045) in LO-NMOSD were the main independent predictors of worse outcome.ConclusionsPatients with LO-NMOSD have similar clinical presentation but worse outcome than EO-NMOSD when they are double seronegative or AQP4-IgG positive. Serostatus and residual disability after first attack are the main predictors of LO-NMOSD outcome.

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Two Cases of Very-Late-Onset Neuromyelitis Optica Spectrum Disorder (NMOSD) in Patients over the Age of 80

Case Reports in Neurology ◽

10.1159/000505231 ◽

2020 ◽

Vol 12 (1) ◽

pp. 13-17 ◽

Cited By ~ 1

Author(s):

Shunya Fujiwara ◽

Yasuhiro Manabe ◽

Ryuta Morihara ◽

Taijun Yunoki ◽

Syoichiro Kono ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Clinical Course ◽

Late Onset ◽

Transverse Myelitis ◽

Spectrum Disorder ◽

High Dose ◽

Neuromyelitis Optica Spectrum Disorder ◽

Initial Manifestation ◽

High Dose Methylprednisolone ◽

Aqp4 Antibody

We report two cases of very-late-onset neuromyelitis optica spectrum disorder (NMOSD) in patients over the age of 80 with transverse myelopathy as the initial manifestation. In both cases, the patients presented with paraplegia and sensory, bladder, and rectal disturbances. Thoracic magnetic resonance imaging showed longitudinal high-intensity signals on a T2-weighted image. The patients received high-dose methylprednisolone. Their serum was positive for anti-AQP4 antibody (cell-based assay) during the clinical course. They were diagnosed with NMOSD and treated with immunoadsorption, plasmapheresis, and followed up with daily prednisolone. Very-late-onset NMOSD in patients over the age of 80 has only rarely been reported. The present cases suggest that NMOSD should be considered for elderly patients presenting with transverse myelitis. Early diagnosis and treatment are important.

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Diagnostic Challenges in Pediatric Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder

Journal of Pediatric Neurology ◽

10.1055/s-0037-1604421 ◽

2017 ◽

Vol 16 (03) ◽

pp. 185-191

Author(s):

Brenda Banwell ◽

Anusha Yeshokumar

Keyword(s):

Multiple Sclerosis ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

First Episode ◽

Neuromyelitis Optica Spectrum Disorder ◽

Pediatric Multiple Sclerosis ◽

Mri Features ◽

The Central Nervous System ◽

Mog Antibodies ◽

Magnetic Resonance Imaging Mri

AbstractThis review highlights the most common presentations of demyelination of the central nervous system (CNS, termed acquired demyelinating syndrome) in children, the difficulty in determining whether the first episode represents a monophasic/transient illness or relapsing disease, and the potential underlying etiologies that must be considered, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and disorders associated with antibodies to myelin oligodendrocyte glycoprotein (MOG) antibodies. The initial clinical and magnetic resonance imaging (MRI) features, as well as those observed over time, are highlighted, emphasizing the distinct and overlapping features of each of these disorders.

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Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75

Journal of Neurology ◽

10.1007/s00415-015-7766-8 ◽

2015 ◽

Vol 262 (5) ◽

pp. 1379-1384 ◽

Cited By ~ 24

Author(s):

Markus Krumbholz ◽

Ulrich Hofstadt-van Oy ◽

Klemens Angstwurm ◽

Ingo Kleiter ◽

Sven Jarius ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Late Onset ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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Neuromyelitis Optica Spectrum Disorder and Anti-Aquaporin 4 Channel Immunoglobulin in an Australian Pediatric Demyelination Cohort

Journal of Child Neurology ◽

10.1177/0883073819895191 ◽

2020 ◽

Vol 35 (4) ◽

pp. 291-296 ◽

Cited By ~ 1

Author(s):

Ariel Dahan ◽

Fabienne Brilot ◽

Richard Leventer ◽

Andrew J. Kornberg ◽

Russell C. Dale ◽

...

Keyword(s):

Retrospective Study ◽

Neuromyelitis Optica ◽

Immunoglobulin G ◽

Axonal Neuropathy ◽

Myelin Oligodendrocyte Glycoprotein ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

Disease Course ◽

Neuromyelitis Optica Spectrum Disorder ◽

Mog Antibodies

Neuromyelitis optica spectrum disorder is uncommon in children, and often seronegative for aquaporin-4 immunoglobulin G (AQP4-IgG). We conducted a retrospective study of 67 children presenting to a single Australian center with acquired demyelinating syndromes over a 7-year period. All patients were tested for AQP4-IgG. Five children (7.5%) had neuromyelitis optica spectrum disorder. One child was seropositive for AQP4-IgG (1.5%) and had a relapsing disease course with mild residual deficits. She also had a concomitant motor axonal neuropathy that improved with immunosuppressive therapy. Of the remaining 4 children, 3 had a monophasic course and 1 a relapsing course. Two were tested for anti–myelin oligodendrocyte glycoprotein (anti-MOG) antibody and both were seropositive. This study confirms that neuromyelitis optica spectrum disorder is uncommon in children, and that AQP4-IgG seropositivity is rare. Anti-MOG antibodies should be tested in children with neuromyelitis optica spectrum disorder.

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Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: A multicenter retrospective study in Korea

Multiple Sclerosis Journal ◽

10.1177/1352458516685416 ◽

2017 ◽

Vol 23 (13) ◽

pp. 1748-1756 ◽

Cited By ~ 19

Author(s):

Jin Myoung Seok ◽

Hye-Jin Cho ◽

Suk-Won Ahn ◽

Eun Bin Cho ◽

Min Su Park ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Clinical Laboratory ◽

Age Of Onset ◽

Late Onset ◽

Brain Mri ◽

Age At Onset ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Tertiary Hospitals ◽

Characteristic Features

Background: There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). Objective: We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. Methods: Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated. Results: Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). Conclusion: Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.

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