Contributions
Abstract: P835
Type: Poster
Abstract Category: Clinical aspects of MS - Paediatric MS
Introduction: Acute disseminated encephalomyelitis followed by monophasic or recurrent optic neuritis (ADEM-ON) is a distinctive and rare entity in the spectrum of acquired demyelinating syndromes (ADS). Here we describe our experience with treatment in a Dutch and German cohort.
Methods: We included children < 18 years old who fulfilled criteria for ADEM, followed by at least one ON. Clinical and demographic data, including presence of antibodies directed to myelin oligodendrocyte glycoprotein (MOG-IgG), were collected.
Results: Eight patients were identified with ADEM-ON. Four of them were boys. The median age at onset was 6.7 years old . All but one were tested seropositive for MOG-IgG (88%). Number of relapses varied from 1 to 9 with a median follow-up duration of 3.0 years. In 6 patients immunosuppressive therapy was initiated with Azathioprine or Mycophenolate Mofetil (MMF) combined with an oral prednisone taper. Remarkably, all of these patients were highly responsive to corticosteroids: no relapse occurred while on high dose oral prednisone taper and there was an overall good recovery after intravenous infusion. Relapses occurred when the prednisone taper reached a low dose, or shortly after cessation. Two patients had multiple relapses under Azathioprine or MMF therapy, and therefore treatment was switched to Rituximab infusions. One patient received IvIG pulse therapy combined with Rituximab. Afterwards relapses still occurred, but with a larger interval.
Conclusion: Children with ADEM-ON are highly responsive to corticosteroids. They do not all respond to Azathioprine or MMF. In those cases Rituximab is an alternative treatment option. Further studies in international collaboration are needed to optimize treatment regimens for children with this rare variant of ADS.
Disclosure: YYM Wong: nothing to disclose
ED van Pelt: nothing to disclose
IA Ketelslegers: nothing to disclose
CE Catsman-Berrevoets: nothing to disclose
K Rostasy: nothing to disclose
RQ Hintzen: nothing to disclose
RF Neuteboom: nothing to disclose
This study is supported by the Dutch Multiple Sclerosis Research Foundation
Abstract: P835
Type: Poster
Abstract Category: Clinical aspects of MS - Paediatric MS
Introduction: Acute disseminated encephalomyelitis followed by monophasic or recurrent optic neuritis (ADEM-ON) is a distinctive and rare entity in the spectrum of acquired demyelinating syndromes (ADS). Here we describe our experience with treatment in a Dutch and German cohort.
Methods: We included children < 18 years old who fulfilled criteria for ADEM, followed by at least one ON. Clinical and demographic data, including presence of antibodies directed to myelin oligodendrocyte glycoprotein (MOG-IgG), were collected.
Results: Eight patients were identified with ADEM-ON. Four of them were boys. The median age at onset was 6.7 years old . All but one were tested seropositive for MOG-IgG (88%). Number of relapses varied from 1 to 9 with a median follow-up duration of 3.0 years. In 6 patients immunosuppressive therapy was initiated with Azathioprine or Mycophenolate Mofetil (MMF) combined with an oral prednisone taper. Remarkably, all of these patients were highly responsive to corticosteroids: no relapse occurred while on high dose oral prednisone taper and there was an overall good recovery after intravenous infusion. Relapses occurred when the prednisone taper reached a low dose, or shortly after cessation. Two patients had multiple relapses under Azathioprine or MMF therapy, and therefore treatment was switched to Rituximab infusions. One patient received IvIG pulse therapy combined with Rituximab. Afterwards relapses still occurred, but with a larger interval.
Conclusion: Children with ADEM-ON are highly responsive to corticosteroids. They do not all respond to Azathioprine or MMF. In those cases Rituximab is an alternative treatment option. Further studies in international collaboration are needed to optimize treatment regimens for children with this rare variant of ADS.
Disclosure: YYM Wong: nothing to disclose
ED van Pelt: nothing to disclose
IA Ketelslegers: nothing to disclose
CE Catsman-Berrevoets: nothing to disclose
K Rostasy: nothing to disclose
RQ Hintzen: nothing to disclose
RF Neuteboom: nothing to disclose
This study is supported by the Dutch Multiple Sclerosis Research Foundation