Abstract
Background and methods: Acquired demyelinating syndromes (ADS) encompass distinct entities and occur in approximately 1/100,000 children. While the use of high dose intravenous corticosteroids is well-established, agreement on steroid taper and type of second line therapy is lacking. A comprehensive, unified and standardized treatment approach is crucial in the management of patients with rare diseases. Therefore, this study performed from July 2018 to June 2020 aimed at developing a national consensus on the management of ADS in the pediatric population using the Delphi approach. Consensus was defined as agreement in >75%. Designated Neuropediatricians with an expertise in the management of pediatric neuroinflammatory diseases in all university and cantonal hospitals of Switzerland were included. The response rate was 100%. Results: High-dose i.v. methylprednisolone (20–30 mg/kg/die for 5 days) is the first line treatment irrespective of the distinct entity of the ADS. An oral steroid taper is recommended in acute demyelinating encephalomyelitis (ADEM) and in neuromyelitis optica spectrum disorder (NMO-SD). However, in the latter more in the sense of bridging. The choice of second line treatment depends on the entity of ADS: in optic neuritis (ON) and ADS due to relapsing remitting multiple sclerosis, first line treatment should be repeated, whereas plasma exchange is recommended in NMO-SD, ADEM and transverse myelitis. Conclusions: A national guideline allowing for a more unified approach in the management of pediatric ADS will enhance future research in this field, making data more comparable. The definition of inadequate treatment response to first line therapy remains a challenge and requires future research.
Highlights
Demyelinating diseases are distinct entities amongst inflammatory brain diseases (IBrainD) and encompass a broad spectrum of different disorders including optic neuritis (ON), transverse myelitis (TM), acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorders (NMO-summarized as mean (SD)) and pediatric onset relapsing remitting multiple sclerosis (RRMS) [1]
71 patients were under ongoing care for an Acquired demyelinating syndromes (ADS) in the Swiss neuropediatric institutions in 2018 (Table 1)
A new diagnosis of ADS was made in 40 patients, most of them presenting with either optic neuritis (27.5%) or acute demyelinating encephalomyelitis (ADEM) (30%)
Summary
Demyelinating diseases are distinct entities amongst inflammatory brain diseases (IBrainD) and encompass a broad spectrum of different disorders including optic neuritis (ON), transverse myelitis (TM), acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorders (NMO-SD) and pediatric onset relapsing remitting multiple sclerosis (RRMS) [1]. An oral steroid taper is recommended in acute demyelinating encephalomyelitis (ADEM) and in neuromyelitis optica spectrum disorder (NMO-SD). In the latter more in the sense of bridging. The choice of second line treatment depends on the entity of ADS: in optic neuritis (ON) and ADS due to relapsing remitting multiple sclerosis, first line treatment should be repeated, whereas plasma exchange is recommended in NMO-SD, ADEM and transverse myelitis. The definition of inadequate treatment response to first line therapy remains a challenge and requires future research
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