Acute disseminated encephalomyelitis (ADEM) is a neuroinflammatory disorder of the central nervous system, manifesting itself as impaired consciousness, even to the point of coma, and multifocal neurological deficits. ADEM is the most common encephalitis in children. Moreover, 50-65% of ADEM in children is associated with the presence of anti-MOG antibodies (MOGAD). In fact, ADEM is the most frequent clinical presentation of MOGAD in children, 50-75% before the age of 10. The risk of recurrence is higher in pediatric MOGAD of ADEM manifestation, up to 30%, compared to myelitis or optic neuritis. Multiphasic MOGAD are more frequently associated with sequelae in 50-69% of cases, versus 4-32% for monophasic forms. In ADEM, cognitive and epileptic sequelae predominate. The 2020 European consortium and the 2022 national diagnosis and care protocol recommend the introduction of disease-modifying therapies as early as the second attack of the disease, or in the event of distant sequelae, in order to limit relapses and sequelae. However, these treatments take several months to take effect. There is currently no reliable predictive factor for MOGAD recurrence other than the persistence of an elevated blood anti-MOG antibody level (≥1:1280) at 1 year. The aim of this study is therefore to identify biomarkers associated with MOGAD recurrence from the first attack. To this end, we will study the transcriptome of circulating blood mononuclear cells by single-cell next-generation RNA sequencing in children with anti-MOGAD neuroinflammatory relapses. Anticipating the multiphasic trajectory of the disease would enable the introduction of early disease-modifying therapy to prevent recurrences and long-term sequelae. Furthermore, the discovery of a molecular and/or cellular signature would provide a better understanding of the pathophysiology of ADEM and MOGAD.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
SUPPORTIVE_CARE
Masking
NONE
Enrollment
20
Drawing blood to realize biomarkers of disease course of MOGAD-ADEM and pathophysiology of ADEM (and MOGAD) : cellular and molecular signatures, inflammatory signaling.
CHU d'Angers
Angers, France
RECRUITINGUnivesity Hostipal of Brest
Brest, France
NOT_YET_RECRUITINGUnivesity Hostipal of APHP
Le Kremlin-Bicêtre, France
NOT_YET_RECRUITINGUnivesity Hostipal of Nantes
Nantes, France
NOT_YET_RECRUITINGUnivesity Hostipal of Rennes
Rennes, France
NOT_YET_RECRUITINGUnivesity Hostipal of Tours
Tours, France
NOT_YET_RECRUITINGBlood sample
1 tube (approx. 5 mL) for children under 12 kg, 2 tubes (approx. 10 mL) for children between 12 kg and 20 kg, 4 tubes (approx. 20 mL) for children over 20 kg. Collected tubes are transferred to the investigator's Biological Resource Center (BRC) for sample preparation of the biocollection, if accepted in the consent form, and preparation of circulating blood mononuclear cells (PBMC).
Time frame: Inclusion, 6 months, 24 months
Age
Time frame: Inclusion
Personal or family history of inflammatory or dysimmune disease
Time frame: Inclusion
Weight
Weight (kg)
Time frame: Inclusion, 6 months, 24 months
EDSS (Expanded Disability Status Scale)
The neurological examination is divided into eight functional systems, 4 major (pyramidal, cerebellar, sensory, brainstem), 4 minor (sphincter, vision, mental and other). A numerical score of increasing severity (0 to 6 or 7) is given to each functional system (FS). The overall scale score is measured on a 20-level scale (0 to 10 per half-point). Up to level 3.5, the score obtained in each FS (Functional System) and the number of FS reached automatically determine the EDSS score. From 4 to 7, the definition of each level is also given by the walking disability (ability to walk without stopping, need for assistance).
Time frame: Inclusion, 6 months, 24 months
Neurological episodes consistent with demyelinating relapse since previous visit
Number, date, neurological symptoms including chronic fatigue unusual for an individual of the same age, diagnosis of demyelinating relapse, type, hospitalization, treatment with intravenous corticosteroid bolus.
Time frame: 6 months, 24 months
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