Brain abnormalities as an initial manifestation of neuromyelitis optica spectrum disorder

2011 ◽  
Vol 17 (9) ◽  
pp. 1107-1112 ◽  
Author(s):  
Woojun Kim ◽  
Su-Hyun Kim ◽  
Sang Hyun Lee ◽  
Xue Feng Li ◽  
Ho Jin Kim
Keyword(s):  
Neuromyelitis Optica ◽  
Inflammatory Diseases ◽  
Age At Onset ◽  
Spectrum Disorder ◽  
Cancer Center ◽  
Enzyme Linked Immunosorbent Assay ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Brain Abnormalities ◽  
Initial Manifestation ◽  
Imaging Characteristics

Background: Although brain abnormalities are being recognized more frequently in patients with neuromyelitis optica spectrum disorder (NMOSD), most brain lesions in previous reports have accompanied pre-existing NMOSD. Here, we describe clinical and imaging characteristics of patients with NMOSD who presented with brain symptoms as their first manifestation of the condition. Methods: Anti-aquaporin-4 antibody was measured using cell-based assays and/or enzyme-linked immunosorbent assay in the sera of 257 patients with inflammatory diseases of the central nervous system who attended the multiple sclerosis clinic of the National Cancer Center, Korea, between May 2005 and December 2009. Eighty-three were seropositive, and 15 of these who presented with brain symptoms were included in this study. We retrospectively reviewed these individuals’ clinical and radiological findings. Results: Patients with NMOSD were followed for a mean of 90 months. Median age at onset was 24 years (6–54 years) and there was a female preponderance (94%). The initial manifestation was classified into two groups according to clinical characteristics: encephalopathy mimicking acute disseminated encephalomyelitis or posterior reversible encephalopathy syndrome and characteristic brainstem symptoms such as intractable hiccup and vomiting. The majority of brain symptoms and lesions resolved. Intriguingly, eight patients (53%) experienced brain relapses that followed a similar pattern during the course of their disease. Conclusion: It is important to recognize that NMO or NMOSD can initially present with brain symptoms.

Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

2011 ◽  
Vol 18 (2) ◽  
pp. 244-247 ◽  
Author(s):  
Sung-Min Kim ◽  
Ji-Soo Kim ◽  
Young Eun Heo ◽  
Hye-Ran Yang ◽  
Kyung Seok Park
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Ocular Manifestation ◽  
Initial Manifestation ◽  
Ocular Symptoms ◽  
Head Tremor ◽  
Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

scholarly journals Investigating the Presence of Interattack Astrocyte Damage in Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol 8 (3) ◽  
pp. e965 ◽  
Author(s):  
Jae-Won Hyun ◽  
Yeseul Kim ◽  
So Yeon Kim ◽  
Min Young Lee ◽  
Su-Hyun Kim ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Single Molecule ◽  
Spectrum Disorder ◽  
Cancer Center ◽  
Multiple Time ◽  
Healthy Controls ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Serum Samples ◽  
Neurofilament Light Chain ◽  
Cutoff Value

ObjectivesInformation on subclinical astrocyte damage can provide further insight into neuromyelitis optica spectrum disorder (NMOSD) pathophysiology and disease-monitoring strategies. To investigate whether astrocyte and neuroaxonal damage occurs during interattack periods in individuals with NMOSD through longitudinal measurement of serum glial fibrillary acidic protein (sGFAP) and neurofilament light chain (sNfL) at multiple time points.MethodssGFAP and sNfL levels were measured in 187 serum samples from 20 participants with NMOSD treated with rituximab (median follow-up: 24 months) and 19 age-/sex-matched healthy controls using a highly sensitive single-molecule array assay. From the NMOSD cohort of National Cancer Center, Korea, 14 clinically stable participants were randomly selected for focused investigation of interattack periods, and 6 participants with clinical attacks despite treatment were enrolled for attack-related measurements.ResultsSignificant elevations of sGFAP levels were observed in all clinical attacks, and 95% (19/20) of patients showed reduction of sGFAP levels below the cutoff value (3 SDs above mean levels in age-/sex-matched healthy controls) within 3 months of their clinical attacks. The sGFAP levels were consistently low during interattack periods in 90% (17/19) of patients whose sGFAP levels returned to below the cutoff value. Changes in sNfL levels were similar to but slower than those in sGFAP levels.ConclusionsSubclinical astrocyte damage represented by increasing sGFAP levels rarely occurred during interattack periods in individuals with NMOSD; however, a certain degree of astrocyte damage did occur at the time of clinical attacks without exception, but it was not evident within 3 months of the attack.

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.

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 Deep Learning-Based Method to Differentiate Neuromyelitis Optica Spectrum Disorder From Multiple Sclerosis

2020 ◽  
Vol 11 ◽  
Author(s):  
Hyunjin Kim ◽  
Youngin Lee ◽  
Yong-Hwan Kim ◽  
Young-Min Lim ◽  
Ji Sung Lee ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Deep Learning ◽  
Clinical Practice ◽  
Neuromyelitis Optica ◽  
Inflammatory Diseases ◽  
Medical Center ◽  
Characteristic Curve ◽  
Brain Magnetic Resonance Imaging ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Background: Differentiating neuromyelitis optica spectrum disorder (NMOSD) from multiple sclerosis (MS) is crucial in the field of diagnostics because, despite their similarities, the treatments for these two diseases are substantially different, and disease-modifying treatments for MS can worsen NMOSD. As brain magnetic resonance imaging (MRI) is an important tool to distinguish the two diseases, extensive research has been conducted to identify the defining characteristics of MRI images corresponding to these two diseases. However, the application of such research in clinical practice is still limited. In this study, we investigate the applicability of a deep learning-based algorithm for differentiating NMOSD from MS.Methods: In this study, we included 338 participants (213 patients with MS, 125 patients with NMOSD) who visited the Asan medical center between February 2009 and February 2020. A 3D convolutional neural network, which is a deep learning-based algorithm, was trained using fluid-attenuated inversion recovery images and clinical information of the participants. The performance of the final model in differentiating NMOSD from MS was evaluated and compared with that of two neurologists.Results: The deep learning-based model exhibited an area under the receiver operating characteristic curve of 0.82 (95% CI, 0.75–0.89). It differentiated NMOSD from MS with an accuracy of 71.1% (sensitivity = 87.8%, specificity = 61.6%), which is comparable to that exhibited by the neurologists. The intra-rater reliability of the two neurologists was moderate (κ = 0.47, 0.50), which was in contrast with the consistent classification of the deep learning-based model.Conclusion: The proposed model was verified to be capable of differentiating NMOSD from MS with accuracy comparable to that of neurologists, exhibiting the advantage of consistent classification. As a result, it can aid differential diagnosis between two important central nervous system inflammatory diseases in clinical practice.

Association of cigarette smoking with neuromyelitis opticaimmunoglobulin G sero-positivity in neuromyelitis optica spectrum disorder

2019 ◽  
Author(s):  
Sharareh Eskandarieh ◽  
Abdorreza Naser Moghadasi ◽  
Mohammad Ali Sahraiain ◽  
Amir Reza Azimi ◽  
Negar Molazadeh
Keyword(s):  
Cigarette Smoking ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Water Channel ◽  
Cross Sectional Study ◽  
Spectrum Disorder ◽  
Enzyme Linked Immunosorbent Assay ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Cross Sectional ◽  
Tertiary Referral Center

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a neuroinflammatory demyelinating disease caused by the presence of a highly specific serum autoantibody marker, NMO-immunoglobulin G (NMO-IgG), that reacts against the water channel aquaporin-4 (AQP4). The present study examined the association between NMO-IgG sero-positivity and environmental factors such as cigarette smoking. Methods: A cross-sectional study was conducted in Sina Hospital, a tertiary referral center in Tehran, Iran. All the patients with a definite diagnosis of NMOSD were involved in this study. The enzyme-linked immunosorbent assay (ELISA) was used to examine the AQP4-IgG status. To assess the association between NMO-IgG sero-positivity and cigarette smoking, a researcher-made questionnaire covering patients’ lifestyle information on smoking habits was designed and administered using the structured face-to-face interviews with the patients. Results: The positive and negative NMO-IgG results were found in 44 (46.8%) and 50 (53.2%) patients, respectively. The increased NMO-IgG sero-positivity odds were observed among the lifetime smokers [odds ratio (OR) = 3.24, 95% confidence interval (CI): 1.16-9.08], current smokers (OR = 6.08, 95% CI: 1.26-29.39), and passive smokers (OR = 2.22, 95% CI: 1.10-4.50). Conclusion: Lifetime and current smoking as well as passive smoking can be regarded as risk factors for NMO-IgG sero-positivity. Smoking with its immunological effects can lead to the production of autoantibodies such as NMO-IgG. 

scholarly journals Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

2014 ◽  
Vol 72 (8) ◽  
pp. 619-624 ◽  
Author(s):  
Diogo C. Carvalho ◽  
Tauana S. Tironi ◽  
Denise S. Freitas ◽  
Rodrigo Kleinpaul ◽  
Natalia C. Talim ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Neuromyelitis Optica ◽  
Age At Onset ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Autoimmune Response ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Organ Specific ◽  
The Relationship ◽  
Receptor Antibodies

The relationship between Sjögren’s syndrome (SS) and neuromyelitis optica spectrum disorder (NMOSD) is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74). Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.

scholarly journals Gender effect on neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G

2016 ◽  
Vol 23 (8) ◽  
pp. 1104-1111 ◽  
Author(s):  
Sung-Min Kim ◽  
Patrick Waters ◽  
Mark Woodhall ◽  
Yoo-Jin Kim ◽  
Jin-Ah Kim ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Spectrum Disorder ◽  
Pattern Reversal ◽  
Neuromyelitis Optica Spectrum Disorder

Background: Neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G (NMOSD-AQP4) is an inflammatory disease characterised by a high female predominance. However, the effect of gender in patients with NMOSD-AQP4 has not been fully evaluated. Objective: The aim of this study was to determine the effect of gender in clinical manifestations and prognosis of patients with NMOSD-AQP4. Methods: The demographics, clinical and radiological characteristics, pattern reversal visual evoked potential (VEP) test results, and prognosis of 102 patients (18 males) with NMOSD-AQP4 were assessed. Results: Male patients had a higher age at onset (48.7 vs 41 years, p = 0.037) and less optic neuritis as the onset attack (17% vs 44%, p = 0.026), higher tendency to manifest as isolated myelitis over the follow-up period (67% vs 28%, p = 0.005), fewer optic neuritis attacks per year (0.08 vs 0.27, p < 0.001), and shorter relative P100 latency on VEP testing (97.1% vs 108.3%, p = 0.001). Moreover, male gender was significantly associated with the absence of optic neuritis attacks over the follow-up period independent of their age of onset. Conclusion: In NMOSD-AQP4 patients, gender impacts on disease onset age and site of attack. This may be an important clue in identifying NMOSD-AQP4 patients with limited manifestations as well as in predicting their clinical courses.

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