Last modified: 2018-09-09
Abstract
Objective: To determine safety and clinical effect of plasma exchange (PE) in paediatric inflammatory CNS syndromes.
Methods: Single-center retrospective review of patient records over the period 2003-2017, to assess clinical characteristics and remission status after PE. All patients admitted with a steroid-refractory acute event presumed to be inflammatory and who required PE were included.
Results: 582 PE procedures to treat 87 attacks in 74 children (63.5% neuromyelitis optica, 8% acute disseminated encephalomyelitis, 4% multiple sclerosis, 12% clinically isolated syndromes, 12% others) were assessed. Mean age at PE was 10.4 years (2-18), 47% girls. Serostatus in 42 patients was 17% AQP4-IgG+, 31% MOG-IgG+, 52% double negative. Attack phenotypes were optic neuritis (ON) 42%, transverse myelitis (TM) 31%, ON+TM 15%, other combined syndromes 11%. Visual outcome, Bladder control, and Hauser ambulation scales, and Expanded Disability Status Scale were evaluated before and after PE in every patient.
No improvement was seen in 15% of events in children with diagnoses other than demyelinating syndromes (infectious myelitis, chiasm glioma, LHON-mutation, sarcoidosis). In the remaining, moderate to marked improvement was observed in 72% of attacks at discharge, increasing to 88.5% at 6 months. No marked differences were observed in response rate among attack phenotypes or serostatus. Adverse events occurred in 23/87 (26%) PE treatments, graded as moderate in 20 and severe in 3.
Conclusions: Our results show that PE is an effective treatment alternative to intravenous steroids for severe attacks of inflammatory CNS syndromes in children. Serious adverse events may occur and should be considered.