Pitié-Salpêtriere

Author Of 2 Presentations

Symptom Management Oral Presentation

FC04.04 - Respiratory disorders in severe MS patients  : an innovative study with evaluation of respiratory muscles during sleep.

Speakers
Presentation Number
FC04.04
Presentation Topic
Symptom Management
Lecture Time
13:36 - 13:48

Abstract

Background

Respiratory disorders (RD) remain incompletely described and understood in multiple sclerosis (MS), although they might play an important role in the burden of MS. RD are the first cause of mortality in MS patients, and could suddenly worsen with acute respiratory failure, for example during an infectious pneumopathy. However, they are underestimated especially due to the motor disability and cognitive disorders.

Objectives

The primary objective was to assess the categories of RD in MS patients:

i) isolated respiratory muscles impairment (decrease of inspiratory maximal pressure or sniff nasal inspiratory pressure < 60%)

ii) diaphragmatic dysfunction (upright vital capacity (VC) - supine VC > 20% of upright VC and/or phasic activation of respiratory muscles during sleep and/or opposition of the thoracic and abdominal respiratory movements during sleep and/or orthopnea and/or respiratory muscles impairment )

iii) nocturne alveolar hypoventilation (PaCO2 > 45 mmHg and/or during the sleep : > 10 min of sleep with PtcCO2 > 55 mmHg or PaCO2 > 50 mmHg if increasing of PaCO2 > 10 mmHg between awake and the sleep).

The secondary objectives were to evaluate the correlation between RD and i) disability scores, including fatigue and cognitive evaluation, and ii) MRI encephalic and spinal lesion load.

Methods

Patients with severe MS (EDSS ≥ 6.5), with or without respiratory complaint, were included in this prospective monocentric study. Comprehensive pulmonary function tests, polysomnography with specific electromyography of accessory respiratory muscles, cognitive tests, brain and cervical spinal cord MRI were performed within 24 hours.

Results

71 patients (39 F/32 M) were included: median age 53,9 years (IQR: 48.40-60.95), median EDSS 7.5 (IQR: 6.5 - 8), median disease duration 21.4 years (IQR: 16-31.35). 46 patients (65%) had diaphragmatic dysfunction, including 36 patients (50%) with isolated respiratory muscles impairment. 9 patients (13%) had nocturnal alveolar hypoventilation. 21 (30 %) patients had no RD. Correlation studies with disability scores and MRI lesion load are on going.

Conclusions

Using specific technics of polysomnography and respiratory muscle testing, this study highlights the frequency of respiratory disorders in MS patients with EDSS ≥ 6.5, but also provides innovative insight into the different types of RD. These findings should lead to specific multidisciplinary care, such as non-invasive ventilation or preventive measures (vaccination against pulmonary infections for patients and families).

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Neuromyelitis Optica and Anti-MOG Disease Oral Presentation

YI02.04 - Comparison of clinical characterization, risk of relapses and antibody dynamics between children and adults with MOGAD

Abstract

Background

To predict the clinical course of myelin oligodendrocyte glycoprotein (MOG)-antibody (Ab)-associated disease (MOGAD) is essential to guide treatment recommendations.

Objectives

We aimed to 1) compare clinical features and disease course, and 2) to evaluate the association of MOG-Ab dynamics and relapses, between children and adults with MOGAD.

Methods

Retrospective study evaluating clinical features of 98 children and 266 adults with MOGAD, between January 2014 and September 2019. To analyse relapses over the whole disease course, a Cox regression analysis for recurrent time-to-event data was performed, introducing treatment as time-dependent covariate. To evaluate dynamics, delta mean fluorescence intensity ratio signal (ΔMFIratio) of MOG-Ab was measured in patients with a minimum time elapsed between two samples of 4 months.

Results

Median age at onset of symptoms was 10.9 (interquartile range 5.4-14.3) years in children and 36.2 (27.7-47.6) in adults. Isolated optic neuritis was the most frequent clinical presentation both in children (40.8%) and adults (55.9%), p=0.013, and acute disseminated encephalomyelitis syndrome was more frequent in children (36.7% vs. 5.6%; p<0.001). Compared to adults, children displayed a better recovery (EDSS ≥3.0 at last follow-up reached only by 10 of 97 [10.3%] vs. 66/247 [26.7%], p<0.001).

In the multivariate analysis, adults were at higher risk of relapse than children (Hazard ratio 1.41, 95%Confidence interval [CI] 1.12-1.78; p=0.003). Among the 124 participants evaluated for MOG-Ab dynamics, 36.3% became seronegative, 60.5% decrease and 3.2% increase the ΔMFIratio. At two years, 64.2% (95%CI 40.9-86.5) of non-relapsing children became MOG-Ab negative compared to 14.1% (95%CI 4.7-38.3) of relapsing ones, log-rank p<0.001, with no differences observed between non-relapsing and relapsing adults, log-rank p=0.280.

Conclusions

MOGAD differs in its clinical presentation at onset, showing a progressive shift in the clinical features across age-groups. Compared to children, adults have a higher risk of relapses and a worse functional recovery. Finally, children with monophasic disease became MOG-Ab negative earlier than relapsing ones, but not in adults. Considering these differences, management and treatment guidelines should be considered independently in children and adults.

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Presenter Of 1 Presentation

Symptom Management Oral Presentation

FC04.04 - Respiratory disorders in severe MS patients  : an innovative study with evaluation of respiratory muscles during sleep.

Speakers
Presentation Number
FC04.04
Presentation Topic
Symptom Management
Lecture Time
13:36 - 13:48

Abstract

Background

Respiratory disorders (RD) remain incompletely described and understood in multiple sclerosis (MS), although they might play an important role in the burden of MS. RD are the first cause of mortality in MS patients, and could suddenly worsen with acute respiratory failure, for example during an infectious pneumopathy. However, they are underestimated especially due to the motor disability and cognitive disorders.

Objectives

The primary objective was to assess the categories of RD in MS patients:

i) isolated respiratory muscles impairment (decrease of inspiratory maximal pressure or sniff nasal inspiratory pressure < 60%)

ii) diaphragmatic dysfunction (upright vital capacity (VC) - supine VC > 20% of upright VC and/or phasic activation of respiratory muscles during sleep and/or opposition of the thoracic and abdominal respiratory movements during sleep and/or orthopnea and/or respiratory muscles impairment )

iii) nocturne alveolar hypoventilation (PaCO2 > 45 mmHg and/or during the sleep : > 10 min of sleep with PtcCO2 > 55 mmHg or PaCO2 > 50 mmHg if increasing of PaCO2 > 10 mmHg between awake and the sleep).

The secondary objectives were to evaluate the correlation between RD and i) disability scores, including fatigue and cognitive evaluation, and ii) MRI encephalic and spinal lesion load.

Methods

Patients with severe MS (EDSS ≥ 6.5), with or without respiratory complaint, were included in this prospective monocentric study. Comprehensive pulmonary function tests, polysomnography with specific electromyography of accessory respiratory muscles, cognitive tests, brain and cervical spinal cord MRI were performed within 24 hours.

Results

71 patients (39 F/32 M) were included: median age 53,9 years (IQR: 48.40-60.95), median EDSS 7.5 (IQR: 6.5 - 8), median disease duration 21.4 years (IQR: 16-31.35). 46 patients (65%) had diaphragmatic dysfunction, including 36 patients (50%) with isolated respiratory muscles impairment. 9 patients (13%) had nocturnal alveolar hypoventilation. 21 (30 %) patients had no RD. Correlation studies with disability scores and MRI lesion load are on going.

Conclusions

Using specific technics of polysomnography and respiratory muscle testing, this study highlights the frequency of respiratory disorders in MS patients with EDSS ≥ 6.5, but also provides innovative insight into the different types of RD. These findings should lead to specific multidisciplinary care, such as non-invasive ventilation or preventive measures (vaccination against pulmonary infections for patients and families).

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Author Of 2 Presentations

COVID-19 Late Breaking Abstracts

LB1222 - Outcomes of coronavirus disease 2019 in patients with neuromyelitis optica and associated disorders (ID 2095)

Abstract

Background

Outcomes of coronavirus disease 2019 (COVID-19) in patients with neuromyelitis optica spectrum disorders (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), often treated with immunosuppressive therapies, are still unknown.

Objectives

The objective was to describe the clinical characteristics and outcomes of COVID-19 in patients with neuromyelitis optica and associated disorders and to identify the factors associated with COVID-19 severity.

Methods

We conducted a multi-center, retrospective, observational cohort study among all French expert centers for neuromyelitis optica and related disorders. Patients with NMOSD or MOGAD included in the study received a confirmed or highly suspected diagnosis of COVID-19 between March 1, 2020 and June 30th, 2020. Main outcome was COVID-19 severity score assessed on a 7-point ordinal scale ranging from 1 (not hospitalized with no limitations on activities) to 7 (death).

Results

Fifteen cases (mean [SD] age: 39.3 [14.3] years, 11 female) were included. Five patients (33.3%) were hospitalized, all receiving rituximab. A 24-year-old patient with positive aquaporine-4 antibody, with obesity as comorbidity, needed mechanical ventilation. Outpatients were receiving anti-CD20 (5), mycophenolate mofetil (3) or azathioprine (3). They were younger (mean [SD] age: 37.0 [13.4] years), with a longer disease duration (mean [SD]: 8.3 [6.3] years) and had a lower EDSS score (median [range] EDSS: 2.5 [0-4]) relative to patients requiring hospitalization (mean [SD] age: 44.0 [16.4] years, mean [SD] disease duration: 5.8 [5.5] years, median [range] EDSS: 4 [0-6.5]).

Conclusions

COVID-19 outcome was overall favorable in this cohort. Larger international studies are needed to identify risk factors of severe COVID-19, however we recommend to maintain preventive measures to limit the risk of contamination with SARS-CoV-2 in this immunocompromised population.

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Epidemiology Poster Presentation

P0480 - Multiple Sclerosis: Is there a risk of worsening after yellow fever vaccination? (ID 299)

Speakers
Presentation Number
P0480
Presentation Topic
Epidemiology

Abstract

Background

Yellow fever vaccine (YFV) is mandatory for travel in areas where yellow fever is endemic, but is not authorized for multiple sclerosis (MS) patients because of the potential risk of post-vaccine relapses. However, this recommendation is only based on a single study including 7 patients.

Objectives

The aim of the study is to assess the risk of worsening in relapsing remitting (RR) MS after YFV.

The primary objective was to compare the risk of relapse, during the 12 months after the YFV between exposed and non-exposed subjects. The secondary objectives were: (i) to assess the time to first relapse after YFV, using Kaplan-Meier curves. Hazard Ratio (HR) was estimated by an adjusted Cox model for EDSS score and for DMT at the time of YFV (ii) to compare the disability progression and the disease form 12 months after YFV, and at the end of the follow-up.

Methods

This is a non-interventional observational retrospective, exposed/non-exposed cohort study, nested in the French national cohort including MS patients. Exposed RR-MS patients received one subcutaneous dose of YFV. Each exposed subject was matched to 3 RR-MS non-exposed to YFV. The matching criteria were: age, sex and annualized relapse rate (ARR) for the year before vaccination. The risk of relapse during the 12 months after the YFV was compared between exposed and non-exposed subjects. The time to first relapse after YFV was assessed using Kaplan Meier curves; Hazard Ratio (HR) was estimated by an adjusted Cox model for EDSS score and for disease modifying therapy at time of YFV. The disability progression 12 months after the YFV was compared between exposed and non-exposed subjects.

Results

128 RR MS according to McDonald Criteria 2017 (32 exposed/96 non-exposed) were included. The ARR the year after YFV did not differ between exposed: 0.233 (0.430) and non-exposed subjects: 0.213 (0.511) (p=0.84). Time to first relapse was not different between the 2 survival curves (adjusted HR, 1.33; 95% CI 0.53-3.30, p=0.54). The disability progression over the year following YFV did not differ between exposed and non-exposed subjects (p=0.83).

Conclusions

YF vaccine, a live attenuated vaccine which is very effective, is required to enter the territory of endemic areas. French and US recommendations for immunization in MS concluded that « There is insufficient data in the literature to conclude on the potential risks related to yellow fever vaccine because studies are either lacking or insufficiently powered ».These results show that YFV doesn’t worsen RR-MS, and suggest that non-immunosuppressed RR-MS patients travelling to endemic areas for professional or personal reasons could be vaccinated against YF.

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