scholarly journals CNS Demyelinating Attacks Requiring Ventilatory Support With Myelin Oligodendrocyte Glycoprotein or Aquaporin-4 Antibodies

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012599
Author(s):  
Hannah H. Zhao-Fleming ◽  
Cristina Valencia Sanchez ◽  
Elia Sechi ◽  
Jery Inbarasu ◽  
Eelco F. Wijdicks ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Ventilatory Support ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Respiratory Support ◽  
Mri Findings ◽  
Black Race ◽  
Non Invasive ◽  
Aqp4 Antibody

Background and Objective:Severe attacks of myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disorder (MOGAD) and aquaporin-4 (AQP4)-antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) may require ventilatory support but data on episodes is limited, particularly for MOGAD. We sought to compare the frequency, characteristics, and outcomes of MOGAD and AQP4-NMOSD attacks requiring ventilatory support.Methods:This retrospective descriptive study identified Mayo Clinic patients (1/1/1996-12/1/2020) with MOGAD or AQP4-NMOSD and an attack requiring non-invasive or invasive ventilation at Mayo Clinic or an outside facility by searching for relevant terms in their electronic medical record. Inclusion criteria were: 1) Attack-related requirement for non-invasive (BiPAP or CPAP) or invasive respiratory support (mechanical ventilation); 2) MOG or AQP4 antibody positivity with fulfillment of MOGAD and AQP4-NMOSD clinical diagnostic criteria, respectively; 3) Sufficient clinical details. We collected data on demographics, co-morbidities, indication for and duration of respiratory support, MRI findings, treatments, and outcomes. The race of those with attacks requiring respiratory support were compared to those without such attacks in MOGAD and AQP4-NMOSD.Results:Attacks requiring ventilatory support were similarly rare in MOGAD (8/279, 2.9%) and AQP4-NMOSD patients (11/503, 2.2%) (p=0.63). The age at attack (median years [range]) (MOGAD, 31.5[5-47] vs AQP4-NMOSD, 43[14-65]; p=0.01) and percentage of female sex (MOGAD, 3/8[38%] vs AQP4-NMOSD, 10/11[91%]; p=0.04) differed. The reasons for ventilation differed between MOGAD (inability to protect airway from seizure, encephalitis or encephalomyelitis with attacks of: acute disseminated encephalomyelitis, 5[62.5%]; or unilateral cortical encephalitis, 3[37.5%]) and AQP4-NMOSD (inability to protect airway from cervical myelitis, 9[82%]; rhombencephalitis, 1[9%]; or combinations of both, 1[9%]). Median ventilation duration for MOGAD was 2 days (range, 1-7) versus 19 days (range, 6-330) for AQP4-NMOSD (p=0.01). All MOGAD patients recovered, but 2/11 (18%) of AQP4-NMOSD died from the attack. For AQP4-NMOSD, Black race was over-represented with attacks requiring ventilatory support versus those without these episodes (5/11[45%] versus 88/457[19%]; p=0.045).Discussion:Ventilatory support is rarely required for MOGAD and AQP4-NMOSD attacks and the indications differ. When compared to MOGAD, these attacks in AQP4-NMOSD may have higher morbidity and mortality and those of Black race were more predisposed, which we suspect may relate to socially mediated health inequality.

scholarly journals Acute Disseminated Encephalomyelitis: Current Perspectives

Children ◽  
2020 ◽  
Vol 7 (11) ◽  
pp. 210
Author(s):  
Renata Barbosa Paolilo ◽  
Kumaran Deiva ◽  
Rinze Neuteboom ◽  
Kevin Rostásy ◽  
Ming Lim
Keyword(s):  
Magnetic Resonance Imaging ◽  
Central Nervous System ◽  
Nervous System ◽  
Magnetic Resonance ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Immune Mediated ◽  
Magnetic Resonance Imaging Mri

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated central nervous system (CNS) disorder, characterized by polyfocal symptoms, encephalopathy and typical magnetic resonance imaging (MRI) findings, that especially affects young children. Advances in understanding CNS neuroimmune disorders as well as the association of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) with both monophasic and recurrent forms of ADEM have led to new insights into its definition, management and outcome. In this review, we aim to provide an update based on current epidemiologic, clinical, radiological and immunopathological aspects and clinical outcome of ADEM.

scholarly journals P.045 Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibody Testing in Calgary: A Quality Improvement Review

2021 ◽  
Vol 48 (s3) ◽  
pp. S32-S32
Author(s):  
JD Krett ◽  
H Hou ◽  
K Alikhani ◽  
MJ Fritzler ◽  
JM Burton
Keyword(s):  
Quality Improvement ◽  
Mayo Clinic ◽  
Chart Review ◽  
False Negative ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Antibody Testing ◽  
Improvement Project ◽  
Confirmatory Testing ◽  
Mog Antibodies

Background: Despite the availability of cell-based assays for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies provincially, outside confirmatory testing is often performed (typically Mayo Clinic Laboratories, USA) when results deviate from expected. It is unknown how often this costly undertaking (upwards of $1,200 CAN) alters diagnosis and management. Methods: We undertook a quality improvement project evaluating the concordance/discordance rate with select chart review in all patients who had cell-based AQP4 or MOG IgG antibody testing at Mitogen Diagnostics (MitogenDx; Calgary, Alberta) and subsequent testing at Mayo Clinic Laboratories from as early as 2010 to July 2020. Results: Preliminary review of data from January 2016 to July 2020 retrieved 145 paired tests; 10 of which were discordant (concordance rate: 93.1%). Chart review confirmed 9 truly discordant cases, often associated with AQP4 or MOG weak-positive results (7/9 cases) or presumed false negative AQP4 results in prototypical neuromyelitis optica spectrum disorder (2/9 cases). Conclusions: Discordant results were rare when comparing MitogenDx local AQP4/MOG antibody test results to those referred out to Mayo Clinic Laboratories, impacting diagnosis and treatment in only 3 patients out of the total. Our results suggest costly outside confirmatory testing of AQP4/MOG antibodies could be reduced.

scholarly journals Case Report: Postvaccination Anti–Myelin Oligodendrocyte Glycoprotein Neuromyelitis Optica Spectrum Disorder

2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
First Trimester ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Future Research ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.

scholarly journals Prolonged non-invasive respiratory support in a COVID-19 patient with severe acute hypoxemic respiratory failure

2021 ◽  
Author(s):  
Andrea Lanza ◽  
Maurizio Sommariva ◽  
Sara Mariani ◽  
Gabriela Ferreyra ◽  
Giuliana Enrica Stagni ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Ventilatory Support ◽  
Respiratory Support ◽  
Low Flow ◽  
Hypoxemic Respiratory Failure ◽  
Acute Hypoxemic Respiratory Failure ◽  
Non Invasive ◽  
High Dependency ◽  
Non Invasive Ventilation ◽  
Experienced Team

A pandemic caused by the Severe Acute Respiratory Syndrome Coronavirus 2 was declared in 2020. Severe cases were characterized by the development of acute hypoxemic respiratory failure (AHRF) requiring advanced respiratory support. However, intensive care units (ICU) were saturated, and many patients had to be treated out of ICU. This case describes a 75-year-old man affected by AHRF due to Coronavirus Disease 2019 (COVID-19), hospitalized in a high-dependency unit, with PaO2/FiO2 <100 for 28 consecutive days. An experienced team with respiratory physiotherapists was in charge of the noninvasive ventilatory support (NIVS). The patient required permanent NIVS with continuous positive airway pressure, non-invasive ventilation, high flow nasal oxygen and body positioning. He was weaned from NIVS after 37 days and started exercise training afterwards. The patient was discharged at home with low-flow oxygen therapy. This case represents an example of a successful treatment of AHRF with the still controversial noninvasive respiratory support in one patient with COVID-19.

scholarly journals Failure of non-invasive respiratory support after 6 hours from initiation is associated with ICU mortality

PLoS ONE ◽  
2021 ◽  
Vol 16 (4) ◽  
pp. e0251030
Author(s):  
Mitsuaki Nishikimi ◽  
Kazuki Nishida ◽  
Yuichiro Shindo ◽  
Muhammad Shoaib ◽  
Daisuke Kasugai ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Acute Respiratory Failure ◽  
Lung Diseases ◽  
Ventilatory Support ◽  
Respiratory Support ◽  
Positive Pressure ◽  
Icu Mortality ◽  
Failure Group ◽  
Non Invasive ◽  
Significant Difference

A previous study has shown that late failure (> 48 hours) of high-flow nasal cannula (HFNC) was associated with intensive care unit (ICU) mortality. The aim of this study was to investigate whether failure of non-invasive respiratory support, including HFNC and non-invasive positive pressure ventilation (NPPV), was also associated with the risk of mortality even if it occurs in the earlier phase. We retrospectively analyzed 59 intubated patients for acute respiratory failure due to lung diseases between April 2014 and June 2018. We divided the patients into 2 groups according to the time from starting non-invasive ventilatory support until their intubation: ≤ 6 hours failure and > 6 hours failure group. We evaluated the differences in the ICU mortality between these two groups. The multivariate logistic regression analysis showed the highest mortality in the > 6 hours failure group as compared to the ≤ 6 hours failure group, with a statistically significant difference (p < 0.01). It was also associated with a statistically significant increased 30-day mortality and decreased ventilator weaning rate. The ICU mortality in patients with acute respiratory failure caused by lung diseases was increased if the time until failure of HFNC and NPPV was more than 6 hours.

scholarly journals Myelin oligodendrocyte glycoprotein-antibody (MOG-IgG) associated disease with centrally located long spinal cord lesion in a 14-month old child

2020 ◽  
Vol 12 ◽  
pp. 117957352095500
Author(s):  
Mohamad Syafeeq Faeez Md Noh
Keyword(s):  
Spinal Cord ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spinal Cord Lesion ◽  
Igg Antibodies ◽  
Mri Findings ◽  
Radiological Features ◽  
Spinal Mri ◽  
Children And Young Adults ◽  
Cord Lesion

Myelin oligodendrocyte glycoprotein-antibodies (MOG-IgG) are associated with acquired inflammatory demyelinating syndromes, seen predominantly in children and young adults. The overlapping clinical and radiological features of the heterogenous spectrum of demyelinating central nervous system (CNS) diseases makes the detection of MOG-IgG antibodies important for prognosis and treatment decisions. Herein, we describe the occurrence of MOG-IgG associated disease presenting as acute disseminated encephalomyelitis (ADEM), with spinal MRI findings of centrally located long cord lesion in a 14-month old child.

scholarly journals Negative pressure ventilation as a bridge to lung transplant

2020 ◽  
Vol 2020 (8) ◽  
Author(s):  
María D P Martínez ◽  
Francisco G Alvarez ◽  
Jorge M Mallea ◽  
Sadia Z Shah ◽  
Si M Pham ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Negative Pressure ◽  
Lung Transplant ◽  
Ventilatory Support ◽  
Respiratory Support ◽  
Mechanical Ventilatory Support ◽  
Negative Pressure Ventilation ◽  
Less Invasive ◽  
Non Invasive ◽  
Allocation Strategies

ABSTRACT Recent years have witnessed evolution of lung allocation strategies to prioritize sicker recipients. In the pre-transplant period, this has translated into increased utilization of invasive extracorporeal or mechanical ventilatory support as a bridge to lung transplantation. The morbidity associated with these strategies warrants consideration to less invasive respiratory support modalities. Herein, we present a case highlighting successful bridge to lung transplantation with a relatively non-invasive negative pressure ventilator.

Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica

2012 ◽  
Vol 19 (8) ◽  
pp. 1052-1059 ◽  
Author(s):  
K Rostásy ◽  
S Mader ◽  
EM Hennes ◽  
K Schanda ◽  
V Gredler ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Pediatric Patients ◽  
Temporal Dynamics ◽  
Clinical Symptoms ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Aquaporin 4 ◽  
Igg Antibodies ◽  
Mri Findings ◽  
Distinct Subgroup

Background: Recently we showed that antibodies to myelin oligodendrocyte glycoprotein (MOG) can be found in aquaporin-4 (AQP4)-immunoglobulin (IgG) seronegative pediatric and adult patients with definite and high-risk neuromyelitis optica (NMO). Objective: The purpose of this study was to describe the clinical characteristics and temporal dynamics of MOG-IgG in AQP4-IgG seronegative pediatric patients presenting with definite NMO. Methods: Children with definite NMO who were referred for further testing of serum antibodies for AQP4 and MOG with a cell-based assay were included in this study. Clinical disease course, cerebrospinal fluid and magnetic resonance imaging (MRI) studies of these patients were reviewed. Results: Between 2008 and 2012 eight children who fulfilled the diagnostic criteria of definite NMO were recruited. Two children with definite NMO tested positive for AQP4-IgG but were negative for MOG-IgG antibodies. Three children had an absence of AQP4-IgG and MOG-IgG antibodies. Three children with definite NMO had high titers of serum MOG-IgG antibodies (≥1: 160), but no AQP4-directed humoral immune response. Longitudinal analysis of serum samples of the latter three children showed persisting high MOG-IgG titers over time. Conclusion: Pediatric patients presenting with clinical symptoms and MRI findings highly suggestive of NMO but with high and persisting MOG-IgG antibody titers are most likely to represent a distinct subgroup of acute demyelinating diseases with important clinical and therapeutic implications.

Comparison of MOG and AQP4 antibody seroprevalence in Korean adults with inflammatory demyelinating CNS diseases

2020 ◽  
pp. 135245852094821
Author(s):  
Jae-Won Hyun ◽  
Hye Lim Lee ◽  
Woo Kyo Jeong ◽  
Hye Jung Lee ◽  
Jong Hwa Shin ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Aquaporin 4 ◽  
Cns Diseases ◽  
Korean Adults ◽  
The Central Nervous System ◽  
Mog Antibodies ◽  
Aqp4 Antibody

We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell–based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.

Utilisation of RAM cannula for non-invasive respiratory support for infants in the cardiac ICU

2021 ◽  
pp. 1-7
Author(s):  
Ashish Saini ◽  
Kevin O. Maher ◽  
Shriprasad R. Deshpande
Keyword(s):  
Ventilatory Support ◽  
Respiratory Support ◽  
Pulmonary Insufficiency ◽  
Invasive Ventilation ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Additional Burden ◽  
Chest X Ray ◽  
One Year

Abstract Background: Children with CHD carry an additional burden of pulmonary insufficiency, often necessitating prolonged ventilatory support, especially in the peri-operative phase. There has been an increase in the utilisation of non-invasive ventilatory support for these children. The objective of this study was to evaluate the utilisation, safety, and outcomes of RAM cannula as a tool for escalation and de-escalation of respiratory support in paediatric cardiac patients less than one year of age. Methods: A single-centre retrospective cohort study of patients supported with RAM cannula. Results: A total of 275 instances of RAM use were included in the study, 81.1% being post-operative. Patients were stratified into escalation and de-escalation cohorts based on the indication of non-invasive ventilation. The success rate of using RAM cannula was 69.5% overall, 66.1% in the escalation group, and 72.8% in the de-escalation group. At baseline, age at cardiac ICU admission >30 days, FiO2 ≤ 40%, PaCO2 ≤ 50 mmHg; and after 12 hours of non-invasive ventilation support respiratory rate ≤ 60/min, PaO2 ≥ 50 mmHg, PaCO2 ≤ 50 mmHg; and absence of worsening on follow-up chest X-ray predicted the success with a sensitivity of 95% in the logistic regression model. Successful support was associated with a significantly shorter unit stay. Conclusions: RAM cannula can be safely used to provide non-invasive support to infants in the cardiac ICU for escalation and de-escalation of respiratory support. Factors associated with success can be used to make decisions about candidacy and appropriate timing of non-invasive ventilation use to maximise effectiveness.

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