scholarly journals Assessing attacks and treatment response rates among adult patients with NMOSD and MOGAD: Data from a nationwide registry in Argentina

2021 ◽  
Vol 7 (3) ◽  
pp. 205521732110323
Author(s):  
Edgar Carnero Contentti ◽  
Pablo A Lopez ◽  
Juan Pablo Pettinicchi ◽  
Juan Criniti ◽  
Agustín Pappolla ◽  
...  
Keyword(s):  
Generalized Estimating Equations ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Study Data ◽  
High Dose ◽  
Radiological Findings ◽  
Treatment Interventions ◽  
Remission Status ◽  
Remission Rates ◽  
Edss Score ◽  
Spectrum Disorders

We aimed to examine treatment interventions implemented in patients experiencing neuromyelitis optica spectrum disorders (NMOSD) attacks (frequency, types, and response). Methods Retrospective study. Data on patient demographic, clinical and radiological findings, and administered treatments were collected. Remission status (complete [CR], partial [PR], no remission [NR]), based on changes in the EDSS score was evaluated before treatment, during attack, and at 6 months. CR was analyzed with a generalized estimating equations (GEEs) model. Results A total of 131 patients (120 NMOSD and 11 myelin oligodendrocyte glycoprotein-antibody-associated diseases [MOGAD]), experiencing 262 NMOSD-related attacks and receiving 270 treatments were included. High-dose steroids (81.4%) was the most frequent treatment followed by plasmapheresis (15.5%). CR from attacks was observed in 47% (105/223) of all treated patients. During the first attack, we observed CR:71.2%, PR:16.3% and NR:12.5% after the first course of treatment. For second, third, fourth, and fifth attacks, CR was observed in 31.1%, 10.7%, 27.3%, and 33.3%, respectively. Remission rates were higher for optic neuritis vs. myelitis (p < 0.001). Predictor of CR in multivariate GEE analysis was age in both NMOSD (OR = 2.27, p = 0.002) and MOGAD (OR = 1.53, p = 0.03). Conclusions This study suggests individualization of treatment according to age and attack manifestation. The outcome of attacks was generally poor.

scholarly journals The Association of Social Support and Symptomatic Remission among Community-Dwelling Schizophrenia Patients: A Cross-Sectional Study

2021 ◽  
Vol 18 (8) ◽  
pp. 3977
Author(s):  
Chi-Hsuan Fan ◽  
Shih-Chieh Hsu ◽  
Fei-Hsiu Hsiao ◽  
Chia-Ming Chang ◽  
Chia-Yih Liu ◽  
...  
Keyword(s):  
Social Support ◽  
Protective Factor ◽  
Cross Sectional Study ◽  
Community Dwelling ◽  
Psychotic Symptoms ◽  
Sectional Study ◽  
Cross Sectional ◽  
Remission Status ◽  
Remission Rates ◽  
Symptomatic Remission

Schizophrenia is a mental disease that often leads to chronicity. Social support could reduce the severity of psychotic symptoms; therefore, its influence on remission should be examined. This study investigated the remission rates in community-dwelling schizophrenia patients and examined the association between social support and remission status. A cross-sectional study was conducted in 129 schizophrenia patients in Taiwan. Remission rates were evaluated, and the level of social support, clinical characteristics, sociodemographic variables, and healthy lifestyle status were compared between the remission and nonremission groups. The association between social support and remission was analyzed after adjusting for confounding factors. The mean illness duration is 12.9 years. More than 95% of the participants lived with their families, 63% were unemployed, and 43% achieved remission. Higher social support was observed in the remission group, and a significant correlation was observed between family domain of social support and remission status. Family support was a protective factor of symptomatic remission in community-dwelling schizophrenia patients in Taiwan. The results reflect the effects of a family-centered culture on patients during illness. Consequently, reinforcing family relationships and the capacity of families to manage the symptoms of patients and providing support to families are recommended.

Early predictors of disability of paediatric-onset AQP4-IgG-seropositive neuromyelitis optica spectrum disorders

2021 ◽  
pp. jnnp-2021-327206
Author(s):  
Valentina Camera ◽  
Silvia Messina ◽  
Kariem Tarek Elhadd ◽  
Julia Sanpera-Iglesias ◽  
Romina Mariano ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Neuromyelitis Optica ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Onset Age ◽  
Visual Disability ◽  
Therapeutic Decisions ◽  
First Relapse ◽  
Edss Score ◽  
Spectrum Disorders

ObjectiveTo describe onset clinical features predicting time to first relapse and time to long-term visual, motor and cognitive disabilities in paediatric-onset aquaporin-4 antibody (AQP4-IgG) neuromyelitis optica spectrum disorders (NMOSDs).MethodsIn this retrospective UK multicentre cohort study, we recorded clinical data of paediatric-onset AQP4-IgG NMOSD. Univariate and exploratory multivariable Cox proportional hazard models were used to identify long-term predictors of permanent visual disability, Expanded Disability Status Scale (EDSS) score of 4 and cognitive impairment.ResultsWe included 49 paediatric-onset AQP4-IgG patients (38.8% white, 34.7% black, 20.4% Asians and 6.1% mixed), mean onset age of 12±4.1 years, and 87.7% were female. Multifocal onset presentation occurred in 26.5% of patients, and optic nerve (47%), area postrema/brainstem (48.9%) and encephalon (28.6%) were the most involved areas. Overall, 52.3% of children had their first relapse within 1 year from disease onset. Children with onset age <12 years were more likely to have an earlier first relapse (p=0.030), despite showing no difference in time to immunosuppression compared with those aged 12–18 years at onset. At the cohort median disease duration of 79 months, 34.3% had developed permanent visual disability, 20.7% EDSS score 4 and 25.8% cognitive impairment. Visual disability was associated with white race (p=0.032) and optic neuritis presentations (p=0.002). Cognitive impairment was predicted by cerebral syndrome presentations (p=0.048), particularly if resistant to steroids (p=0.034).ConclusionsAge at onset, race, onset symptoms and resistance to acute therapy at onset attack predict first relapse and long-term disabilities. The recognition of these predictors may help to power future paediatric clinical trials and to direct early therapeutic decisions in AQP4-IgG NMOSD.

scholarly journals Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

2019 ◽  
Vol 32 (11) ◽  
pp. 727
Author(s):  
Raquel Gil-Gouveia ◽  
Natália Marto ◽  
Pedro Vilela ◽  
Ana Catarino
Keyword(s):  
Hearing Loss ◽  
Fungal Infection ◽  
Infectious Agent ◽  
Brain Biopsy ◽  
High Dose ◽  
Radiological Findings ◽  
Susac Syndrome ◽  
Infection Treatment ◽  
Immune Mediated

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease: practical considerations

2018 ◽  
Vol 19 (3) ◽  
pp. 187-195 ◽  
Author(s):  
Maciej Juryńczyk ◽  
Anu Jacob ◽  
Kazuo Fujihara ◽  
Jacqueline Palace
Keyword(s):  
Neuromyelitis Optica ◽  
Inflammatory Diseases ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Lesion Volume ◽  
Imaging Features ◽  
Visual Disability ◽  
Permanent Disability ◽  
Spectrum Disorders ◽  
Aqp4 Antibody

The field of central nervous system (CNS) inflammatory diseases has recently broadened to include a new condition associated with pathogenic serum antibodies against myelin oligodendrocyte glycoprotein (MOG). This is distinct from multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody neuromyelitis optica spectrum disorders (NMOSD). MOG antibody-associated disease phenotypes are varied and range from classical neuromyelitis optica to acute demyelinating encephalomyelitis and cortical encephalitis. The diagnosis depends on using a reliable, specific and sensitive assay of the antibody. Clinical and imaging features of MOG-associated syndromes overlap with AQP4 antibody NMOSD but can be usually distinguished from MS: in particular, the silent lesions typical of MS that progressively increase lesion volume are rare in MOG antibody disease. The disease can relapse but medium-term immunosuppression appears to be protective. Permanent disability, particularly severe ambulatory and visual disability, is less frequent than in AQP4 antibody NMOSD and usually results from the onset attack. However, sphincter and sexual dysfunction after a transverse myelitis is common. Here we review the practical aspects of diagnosing and managing a patient with MOG antibody-associated disease.

scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Recurrent and Migrating Focal Cortical Encephalitis

2020 ◽  
Vol 7 ◽  
pp. 2329048X2096617
Author(s):  
Xinran Maria Xiang ◽  
Rachel Evans ◽  
Jesus Lovera ◽  
Rashmi Rao
Keyword(s):  
Brain Mri ◽  
Occipital Cortex ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
New Right ◽  
High Dose ◽  
Antibody Testing ◽  
Leptomeningeal Enhancement ◽  
The Right ◽  
Work Up ◽  
New Onset

Although pediatric myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is increasingly well-recognized, its full clinical spectrum is still being defined. Cortical encephalitis is emerging as a distinct clinico-radiologic syndrome of adult MOG antibody-associated disease. We describe a 12-year-old girl who presented with new onset seizures and left-sided hemiparesis. Brain MRI showed edema of the right temporal-parietal-occipital cortex with associated focal leptomeningeal enhancement. Patient received high-dose corticosteroids and 21 days of acyclovir despite negative infectious work-up due to the focal nature of encephalitis. Patient remained seizure-free for 20 months before presenting with new right hemiclonic seizures with right-sided hemiparesis and edema of the left temporal-parietal cortex with associated leptomeningeal enhancement. Patient’s MOG antibody titer was 1:40. She completed high-dose corticosteroids and intravenous immunoglobulin. Our patient highlights the importance of MOG antibody testing in pediatric focal cortical encephalitis to avoid unnecessary anti-viral agents and provide more appropriate immunotherapy and a more informed prognosis.

scholarly journals High-dose biotin in progressive multiple sclerosis: A prospective study of 178 patients in routine clinical practice

2019 ◽  
Vol 26 (14) ◽  
pp. 1898-1906 ◽  
Author(s):  
Laura Couloume ◽  
Laetitia Barbin ◽  
Emmanuelle Leray ◽  
Sandrine Wiertlewski ◽  
Emmanuelle Le Page ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Clinical Practice ◽  
Prospective Study ◽  
Outcome Measures ◽  
Progressive Multiple Sclerosis ◽  
Routine Clinical Practice ◽  
High Dose ◽  
Disability Progression ◽  
Edss Score ◽  
Multiple Sclerosis Patients

Background: A recent controlled trial suggested that high-dose biotin supplementation reverses disability progression in patients with progressive multiple sclerosis. Objective: To analyze the impact of high-dose biotin in routine clinical practice on disability progression at 12 months. Methods: Progressive multiple sclerosis patients who started high-dose biotin at Nantes or Rennes Hospital between 3 June 2015 and 15 September 2017 were included in this prospective study. Disability outcome measures, patient-reported outcome measures, relapses, magnetic resonance imaging (MRI) data, and adverse events were collected at baseline, 6, and 12 months. Results: A total of 178 patients were included. At baseline, patients were 52.0 ± 9.4 years old, mean Expanded Disability Status Scale (EDSS) score was 6.1 ± 1.3, mean disease duration was 16.9 ± 9.5 years. At 12 months, 3.8% of the patients had an improved EDSS score. Regarding the other disability scales, scores either remained stable or increased significantly. In total, 47.4% of the patients described stability, 27.6% felt an improvement, and 25% described a worsening. Four patients (2.2%) had a relapse. Of the 74 patients (41.6%) who underwent an MRI, 20 (27.0%) had new T2 lesions, 8 (10.8%) had gadolinium-enhancing lesions. Twenty-five (14%) reported adverse event. Conclusion: In this study, high-dose biotin did not seem to be associated with a clear improvement in disability.

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