Hofer, Seline; Bauder, Florian; Capone Mori, Andrea; Chan, Andrew; Dill, Patricia; Garcia-Tarodo, Stéphanie; Goeggel Simonetti, Barbara; Hackenberg, Annette; Kalser, Judith; Maier, Oliver; Schmid, Regula; Strozzi, Susi; Bigi, Sandra (2021). Management of Acute Demyelinating Attacks in the Pediatric Population: A Swiss Consensus Statement. Clinical and translational neuroscience, 5(2), p. 17. MDPI 10.3390/ctn5020017
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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.
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