Disease course and immunotherapies responses in children with relapsing myelin oligodendrocyte glycoprotein antibodies (MOG-Ab)-associated disease
ECTRIMS Online Library. Hacohen Y. 10/26/17; 199968; P313
Yael Hacohen
Yael Hacohen
Contributions
Abstract

Abstract: P313

Type: Poster

Abstract Category: Clinical aspects of MS - 3 Paediatric MS

Background: Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are associated with a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center-specific, with no formal consensus guidelines. We therefore conducted this multicenter study to describe the disease course, and response to different treatment strategies in children with relapsing MOG-Ab-associated disease.
Methods: We retrospectively collected demographic, clinical, and radiological data of 102 patients with MOG-Ab associated relapsing disease, from 8 countries that are part of the EU Paediatric Demyelinating Disease Consortium. Patients were treated according to local protocols. Annual relapse rate (ARR) and Expanded Disability Status Scale (EDSS) before and on treatment were compared in patients receiving immunotherapy for over 1 year.
Results: 102 children were identified whose original diagnoses were neuromyelitis optica spectrum disorder (NMOSD, 43.1%), multiphasic disseminated encephalomyelitis (MDEM, 19.6%), and relapsing optic neuritis (RON, 17.6%). A total of 464 demyelinating events were reported in the cohort. Clinical events under the age of 9yrs were more likely to affect the brain, whereas events over the age of 9yrs were more likely to affect the optic nerve (p< 0.0001). Brain MRI abnormalities were also more common in the younger group (p< 0.0001). Disease modifying drugs (DMDs) were given in 52 (51%) children: 28 (53.8%) patients were treated with 1 DMD, 16 (30.7%) with two and 7 (13.5%) with 3 or more sequential DMDs. The treated group had a more severe phenotype than the untreated group, with higher total number of relapses and EDSS at last follow-up (p=0.0094 and P< 0.0001 respectively). No changes in relapses frequency and EDSS were observed between the pre- and post-initiation of Interferon-beta and Glatiramer acetate, n=11). ARR was reduced by 0.8 with Azathioprine (n=20, p=0.0004), 1.3 with Mycophenolate mofetil (n=15, p=0.0028), and 1.6 with Rituximab (n=9, p=0.0008), but the EDSS remain stable with these DMDs. Regular IVIG (n=12) was associated with an improvement in ARR
(1.7, p< 0.0001) and EDSS (mean improvement 1.0, 95%CIs: 0.2466 to 1.753, p=0.0127).
Conclusions: Children with MOG-Ab associated disease did not seem to benefit from conventional MS treatments, whilst B-cells targeted treatments, and particularly IVIG, were associated with a significant reduction in relapse frequency.
Disclosure:
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen,, Robert Forsyth,, Banu Anlar, Rogier Hintzen,, Matthias Baumann, Kevin Rostasy, Rinze Neuteboom and Kumaran Deiva have nothing to declare.
Evangeline Wassmer has received speaker honoraria and/or travel funding from Biogen, Teva, Genzyme, Shire, UCB, and Merck Serono
Cheryl Hemingway has received speaking honoraria from Biogen and terumo, and an educational grant from Merck Serono, Biogen and Bayer.
Olga Ciccarelli serves as a consultant for GE, Biogen Idec, and Novartis, and all the payments are made to the institution (Queen Square MS Centre, UCL Institute of Neurology, London, UK).
Maria Leite is involved in AQP4 testing; is supported by the National Health Service National Specialised Commissioning Group for Neuromyelitis Optica and by the National Institute for Health Research Oxford Biomedical Research Centre; and has received speaking honoraria from Biogen Idec and travel grants from Novartis.
Romain Marignier serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva
Jackie Palace is partly funded by highly specialized services to run a national congenital myasthenia service and a national neuromyelitis optica service. She has received conference funding for scientific meetings and honoraria for advisory work from Bayer Schering, Biogen Idec, Chugai Pharma, Merck Serono, Novartis, Ono Pharmaceutical Co. Ltd and Teva. She has received unrestricted research grants from Bayer Schering, Biogen Idec, Merck Serono and Novartis.Jackie Palace
Ming Lim receives research grants from Action Medical Research, DES society, GOSH charity, NIHR, MS Society, SPARKS charity and; receives research support grants from the London Clinical Research Network and Evelina Appeal; has received consultation fees from CSL Behring; received travel grants from Merck Serono; and awarded educational grants to organize meetings by Novartis, Biogen Idec, Merck Serono and Bayer

Abstract: P313

Type: Poster

Abstract Category: Clinical aspects of MS - 3 Paediatric MS

Background: Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are associated with a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center-specific, with no formal consensus guidelines. We therefore conducted this multicenter study to describe the disease course, and response to different treatment strategies in children with relapsing MOG-Ab-associated disease.
Methods: We retrospectively collected demographic, clinical, and radiological data of 102 patients with MOG-Ab associated relapsing disease, from 8 countries that are part of the EU Paediatric Demyelinating Disease Consortium. Patients were treated according to local protocols. Annual relapse rate (ARR) and Expanded Disability Status Scale (EDSS) before and on treatment were compared in patients receiving immunotherapy for over 1 year.
Results: 102 children were identified whose original diagnoses were neuromyelitis optica spectrum disorder (NMOSD, 43.1%), multiphasic disseminated encephalomyelitis (MDEM, 19.6%), and relapsing optic neuritis (RON, 17.6%). A total of 464 demyelinating events were reported in the cohort. Clinical events under the age of 9yrs were more likely to affect the brain, whereas events over the age of 9yrs were more likely to affect the optic nerve (p< 0.0001). Brain MRI abnormalities were also more common in the younger group (p< 0.0001). Disease modifying drugs (DMDs) were given in 52 (51%) children: 28 (53.8%) patients were treated with 1 DMD, 16 (30.7%) with two and 7 (13.5%) with 3 or more sequential DMDs. The treated group had a more severe phenotype than the untreated group, with higher total number of relapses and EDSS at last follow-up (p=0.0094 and P< 0.0001 respectively). No changes in relapses frequency and EDSS were observed between the pre- and post-initiation of Interferon-beta and Glatiramer acetate, n=11). ARR was reduced by 0.8 with Azathioprine (n=20, p=0.0004), 1.3 with Mycophenolate mofetil (n=15, p=0.0028), and 1.6 with Rituximab (n=9, p=0.0008), but the EDSS remain stable with these DMDs. Regular IVIG (n=12) was associated with an improvement in ARR
(1.7, p< 0.0001) and EDSS (mean improvement 1.0, 95%CIs: 0.2466 to 1.753, p=0.0127).
Conclusions: Children with MOG-Ab associated disease did not seem to benefit from conventional MS treatments, whilst B-cells targeted treatments, and particularly IVIG, were associated with a significant reduction in relapse frequency.
Disclosure:
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen,, Robert Forsyth,, Banu Anlar, Rogier Hintzen,, Matthias Baumann, Kevin Rostasy, Rinze Neuteboom and Kumaran Deiva have nothing to declare.
Evangeline Wassmer has received speaker honoraria and/or travel funding from Biogen, Teva, Genzyme, Shire, UCB, and Merck Serono
Cheryl Hemingway has received speaking honoraria from Biogen and terumo, and an educational grant from Merck Serono, Biogen and Bayer.
Olga Ciccarelli serves as a consultant for GE, Biogen Idec, and Novartis, and all the payments are made to the institution (Queen Square MS Centre, UCL Institute of Neurology, London, UK).
Maria Leite is involved in AQP4 testing; is supported by the National Health Service National Specialised Commissioning Group for Neuromyelitis Optica and by the National Institute for Health Research Oxford Biomedical Research Centre; and has received speaking honoraria from Biogen Idec and travel grants from Novartis.
Romain Marignier serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva
Jackie Palace is partly funded by highly specialized services to run a national congenital myasthenia service and a national neuromyelitis optica service. She has received conference funding for scientific meetings and honoraria for advisory work from Bayer Schering, Biogen Idec, Chugai Pharma, Merck Serono, Novartis, Ono Pharmaceutical Co. Ltd and Teva. She has received unrestricted research grants from Bayer Schering, Biogen Idec, Merck Serono and Novartis.Jackie Palace
Ming Lim receives research grants from Action Medical Research, DES society, GOSH charity, NIHR, MS Society, SPARKS charity and; receives research support grants from the London Clinical Research Network and Evelina Appeal; has received consultation fees from CSL Behring; received travel grants from Merck Serono; and awarded educational grants to organize meetings by Novartis, Biogen Idec, Merck Serono and Bayer

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