EDEN

Devic’s neuromyelitis optica (NMO) is a rare demyelinating and inflammatory disease of the central nervous system with a high rate of morbidity and mortality when not diagnosed promptly and treated appropriately.There were still many issues and unmet needs in NMO research and care: the rarity of the condition making it difficult to gather robust epidemiological data and biosamples at a single centre level; heterogeneity in the diagnosis and treatment at the national and European level; lack of standardisation of the biomarker assay; lack of interaction through academic research centres in Europe; and absence of information for patients, caregivers and clinicians. Thus, the Eugene Devic European Network (EDEN) emerged from a common will to collaborate on NMO and related disorders across Europe. The main objective was to progress beyond the state of the art in diagnosis, and to explore treatment and pathophysiology of NMO. More specifically, the project aimed to:
–    set up a European database for NMO to describe the demographic, clinical, radiological and biological features and the clinical course with and without specific treatments;
–    create standardised biobanks for NMO for biomarkers and pathophysiology studies;
–    set up European gold standard for AQP4 Ab detection, develop prognostic tests and research for new biomarkers;
–    develop and validate models for NMO;
–    propose standardised therapeutic procedures to improve management of patients;
–    disseminate procedures to improve communication to patients and clinicians

The main results of this interdisciplinary project are as follows:
–    The EDEN database – composed of data on demographics, clinical, biological and imaging characteristics and treatment – has been launched and is used in 4 countries.
–    Procedures for storage of blood and cerebrospinal fluid have been validated.
–    Better knowledge of NMO physiopathology: Significant differences in the pathogenicity of NMO whichs is possibly related to the properties of antibodies have been demonstated
–    Greater understanding of the heterogeneity of NMO tisue lesion show that at least six different lesion types can be identified, providing clear evidence for a pathogenic role of aquaporin 4 antibodies in the disease
–    New animal and cell models to study the role of T-cells and the intrinsic effect of auto-antibodies were developed
–    New biomarkers were assessed and validated : AQP1-antibodies, MOG-antibodies
–    New diagnostic tools were set up and validated : AQP4-IgG detection methods were compared in more than 15 centers; a prototypic cell-based assay for the determination of MOG-specific antibodies is under development
–    Texts providing information for NMO patients and caregivers were prepared. These texts include anatomic, pathological, clinical, imaging, laboratory and treatment aspects of DNMO. Leaflets about the most frequent symptoms, usual diagnostic tests, therapeutic regimes, side effects, complications, prognosis and potential disability of DNMO are prepared.
–    The Turkish MS Society plans to form a NMO/NMOSD sub-group for patients and their family
Overall this work has helped to increase the knowledge and awareness of NMO over the last years. The concept of NMO has been clarified and now includes a broader range of clinical phenotypes than previously appreciated, the NMO spectrum disorder (NMOSD). We have a greater understanding of the disease mechanism from animal models, of the heterogeneity in lesions from biopsy and autopsy tissue from AQP4-Ab positive patients, and more biomarkers to help patient diagnosis and prognosis. These major achievements have opened new challenges and avenues in the field that we propose to address in the next years.