Aquaporin 4 Antibody
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Life ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 19
Author(s):  
Michela Ada Noris Ferilli ◽  
Roberto Paparella ◽  
Ilaria Morandini ◽  
Laura Papetti ◽  
Lorenzo Figà Talamanca ◽  
...  

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.


2021 ◽  
pp. 135245852110603
Author(s):  
Jae-Won Hyun ◽  
Hye Lim Lee ◽  
Jaehong Park ◽  
Jiah Kim ◽  
Ju-Hong Min ◽  
...  

In a large acute myelitis cohort, we aimed to determine whether brighter spotty lesions (BSLs)—using the refined terminology—on spinal magnetic resonance imaging (MRI) help distinguish aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) from myelin oligodendrocyte glycoprotein antibody disease (MOGAD). An experienced neuro-radiologist and two neurologists independently analyzed 133 spinal MRI scans (65 from MOGAD and 68 from AQP4-NMOSD) acquired within 1 month of attacks. BSLs were observed in 18 of 61 (30%) participants with AQP4-NMOSD, while none of 49 participants with MOGAD showed BSL ( p < 0.001). BSL during the acute phase would be useful to differentiate AQP4-NMOSD from MOGAD.


2021 ◽  
Author(s):  
Thiago Gonçalves Fukuda ◽  
Ivã Taiuan Fialho Silva ◽  
Tayla Samanta Silva dos Santos ◽  
Marcos Baruch Portela Filho ◽  
Fernanda Ferreira de Abreu ◽  
...  

Abstract Introduction: Neuromyelitis optic spectrum disorders (NMOSD) is a rare inflammatory and demyelinating disease of the central nervous system (CNS) more frequent in women and Afro-descendants. No previous epidemiological or prognostic study has been conducted in the region of the state of Bahia, Brazilian Northeast. Objective: To evaluate clinical and prognostic aspects in patients with NMOSD from a cohort in northeastern Brazil. Material and Methods: A single-center retrospective study was conducted with consecutive patients diagnosed with NMOSD. Clinical and epidemiological characteristics were described. The degree of disability was expressed by the Expanded Disability Status Scale (EDSS). Worsening disability were analyzed through negative binomial regression adjusted for disease duration. Results: Ninety-one patients were included, 72 (79.1%) female and 67 (73.6%) afro descendants. Mean age at onset was 36 (± 14) years and 73.3% were anti-aquaporin-4 antibody positive. Isolated transverse myelitis (32.9%) and isolated optic neuritis (22.4%) were the most frequent initial clinical syndromes. After multivariate analysis, optic neuritis (RR = 0.15; 95% CI=0.03 – 0.59; p = 0.008) and dyslipidemia (RR = 0.07; 95% CI=0.04 – 0.40; p < 0.001) were associated with slower disease progression. Area postrema involvement (RR = 29.69; 95% CI=3.40 – 226.07; p = 0.002) and age at onset (RR = 1.05; 95% CI=1.00 – 1.10; p = 0.037) were associated with faster disease progression. Conclusions: In the first clinical and prognostic study in northeastern Brazil, we identified area postrema involvement, age at onset, optic neuritis at fist syndrome and dyslipidemia as the main prognostic factors associated with disease progression.


Author(s):  
Tetsuya Akaishi ◽  
Tatsuro Misu ◽  
Kazuo Fujihara ◽  
Toshiyuki Takahashi ◽  
Yoshiki Takai ◽  
...  

Abstract Objective The patterns of relapse and relapse-prevention strategies for anti-myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are not completely investigated. We compared the patterns of relapse in later stages of MOGAD with those of anti-aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD). Methods In this observational, comparative cohort study, 66 patients with MOGAD and 90 with AQP4-Ab-positive NMOSD were enrolled. We compared the patterns of relapse and annualized relapse rates (ARRs) in the first 10 years from disease onset, stratified by relapse-prevention treatments. Results Approximately 50% of the patients with MOGAD experienced relapses in the first 10 years. Among those not undergoing relapse-prevention treatments, ARRs in the first 5 years were slightly lower in MOGAD patients than in AQP4-Ab-positive NMOSD patients (MOGAD vs. AQP4-Ab NMOSD: 0.19 vs. 0.30; p = 0.0753). After 5 years, the ARR decreased in MOGAD patients (MOGAD vs. AQP4-Ab NMOSD: 0.05 vs. 0.34; p = 0.0001), with a 72% reduction from the first 5 years (p = 0.0090). Eight (61.5%) of the 13 MOGAD patients with more than 10-year follow-up from disease onset showed relapse 10 years after onset. Clustering in the timing and phenotype of attacks was observed in both disease patients. The effectiveness of long-term low-dose oral PSL for relapse prevention in patients with MOGAD has not been determined. Conclusions The relapse risk in patients with MOGAD is generally lower than that in patients with AQP4-Ab-positive NMOSD, especially 5 years after onset. Meanwhile, relapses later than 10 years from onset are not rare in both diseases.


2021 ◽  
Author(s):  
Yajun Yao ◽  
Xindi Li ◽  
Yun Xu ◽  
Xiaofang Liang ◽  
Liu Yang ◽  
...  

Abstract Background Antibodies against myelin-oligodendrocyte-glycoprotein (MOG-Abs) associated disorders (MOGAD) have been recognized as a disease entity in their own right. Optic neuritis (ON) is the most common symptom in MOGAD. Objective To demonstrate the differences in retinal microvascular characteristics between patients with MOGAD-ON and aquaporin-4 antibody (AQP4-Ab) positive ON. Methods A prospective study in which optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) were used to measure retinal and microvascular parameters. Results 22 MOGAD-ON eyes, 32 AQP4-ON eyes and 60 control eyes were included in the study. The thickness of RNFL and GCC in MOGAD-ON eyes is significantly lower than that of HC (p <0.001, respectively), but comparable to AQP4-ON eyes. The vessel density in retina capillary plexus (RCP) reduced significantly in MOGAD-ON than that in AQP4-ON (p < 0.05, respectively). Unlike AQP4-ON, the visual accuracy in MOGAD-ON was positivitly correlated with vessel density of superficial RCP (P = 0.010) and ON relaspe times (P = 0.038). Conclusion The retinal neuro-axonal damages between MOGAD-ON and AQP4-ON were comparable. Unlike AQP4-ON eyes, microvascular densities were significantly reduced in MOGAD-ON, and was positively correlated with the deterioration of visual acuity in MOGAD-ON.


2021 ◽  
Vol 9 (10) ◽  
pp. 656-659
Author(s):  
Maria Nina Grace Q. Bastinen , MD

Neuromyelitis Optica (previously called Devics disease) is an autoimmune-induced demyelinating disorder of the central nervous system that primarily affects the optic nerves and spinal cord. This manuscript presents a case of a 28-yearold female patient who clinically presented with acute lower extremity weakness and numbness associated with unilateral impairment of vision. Later in the course of the disease, magnetic resonance imaging of the cervico-thoracic spine showed multi-level abnormal non-enhancing and enhancing lesions from levels C3 to T8 levels which were identified to be combined transverse myelitis and cord syrinx. An anti-aquaporin-4 receptor antibody was obtained and yielded to be seropositive. Given the patients clinical manifestations, combined with imaging and laboratory examinations, led to a diagnosis of Neuromyelitis Optica (NMO). The patient was managed with high-dose steroids. The significant discovery of anti-aquaporin-4 antibodies served as a key factor in the NMO immunopathogenesis. It is currently regarded as a specific biomarker of the diagnostic process, making it distinguishable from multiple sclerosis. This case highlights the mechanism of formation of a fluid-filled syrinx-like cavity in the cord in the setting of Neuromyelitis Optica.


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