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

2017 ◽  
Vol 23 (13) ◽  
pp. 1748-1756 ◽  
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.

scholarly journals Late-onset neuromyelitis optica spectrum disorder

2019 ◽  
Vol 6 (6) ◽  
pp. e607 ◽  
Author(s):  
Maria Sepulveda ◽  
Guillermo Delgado-García ◽  
Yolanda Blanco ◽  
Nuria Sola-Valls ◽  
Elena H. Martinez-Lapiscina ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Clinical Presentation ◽  
Late Onset ◽  
Age At Onset ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Igg Antibodies ◽  
Retrospective Multicenter Study ◽  
Demographic Features

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.

AQP4-IgG-positive neuromyelitis optica spectrum disorder with late onset in Mexico.

2020 ◽  
Vol 43 ◽  
pp. 102221 ◽  
Author(s):  
Guillermo Delgado-García ◽  
Emmanuel Antonio-Luna ◽  
Diego López-Mena ◽  
Verónica Rivas-Alonso ◽  
José Flores-Rivera ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Late Onset ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Frequency of brain MRI abnormalities in neuromyelitis optica spectrum disorder at presentation: A cohort of Latin American patients

2018 ◽  
Vol 19 ◽  
pp. 73-78 ◽  
Author(s):  
Edgar Carnero Contentti ◽  
Vanessa Daccach Marques ◽  
Ibis Soto de Castillo ◽  
Veronica Tkachuk ◽  
Amilton Antunes Barreira ◽  
...  
Keyword(s):  
Latin American ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

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

2020 ◽  
Vol 12 (1) ◽  
pp. 13-17 ◽  
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.

scholarly journals Seronegative Neuromyelitis Optica Spectrum Disorder following Exposure to Hepatitis B Vaccination

2015 ◽  
Vol 7 (1) ◽  
pp. 78-83 ◽  
Author(s):  
Richard Heekin ◽  
Chetan Gandhy ◽  
Derrick Robertson
Keyword(s):  
Hepatitis B ◽  
Plasma Exchange ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Oligoclonal Bands ◽  
Hepatitis B Vaccination ◽  
Neuromyelitis Optica Spectrum Disorder

Controversy exists regarding a potential link between exposure to recombinant hepatitis B vaccine (HBV) and central nervous system demyelinating diseases. Here, we present a case of seronegative neuromyelitis optica spectrum disorder (NMOSD) following exposure to HBV. A 28-year-old man developed painful eye movements 11 days after exposure to HBV. Within 24 h, he experienced vision loss, ascending numbness, and ataxia. T-spine MRI showed a cord lesion spanning T6-T9. Brain MRI showed bilateral optic nerve contrast enhancement and a right-sided internal capsule lesion. Cerebrospinal fluid analysis was normal, including negative oligoclonal bands and normal IgG index. AQP4-IgG serology was negative. The patient's visual symptoms improved after treatment with steroids and plasma exchange. He received plasma exchange weekly for 4 weeks with decreased numbness and tingling as well as improved coordination. Treatment with mycophenolate mofetil was started, and the patient remains clinically stable with near resolution of his prior symptoms. Neuromyelitis optica is characterized by optic neuritis and/or longitudinally extensive transverse myelitis. While our patient tested seronegative for AQP4-IgG (which remains negative in 10-50% of NMOSD cases, despite testing with the most sensitive assays available), he did meet NMOSD diagnostic criteria. In a literature review, we found 7 cases of NMOSD onset or relapse associated with exposure to various vaccines, but to our knowledge this represents the first published report of NMOSD onset following exposure to HBV. While causality between vaccination and CNS demyelinating disease remains elusive, it is important to report these cases to help develop safer vaccinations and provoke further inquiry into the pathogenesis of NMOSD.

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