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Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination
Published on: September 12, 2016
Outcomes after early administration of plasma exchange in pediatric central nervous system inflammatory demyelination
Sandra Bigi1, Brenda Banwell2, E Ann Yeh3
1The Hospital for Sick Children, Division of Neurology, Toronto, Ontario, Canada Sandra.Bigi@insel.ch sandra.bigi@gmx.ch.
Insights
Plasma exchange may effectively treat severe pediatric central nervous system inflammatory demyelination. This treatment showed improved mobility in children with conditions like acute transverse myelitis and multiple sclerosis.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Plasma exchange (PLEX) is used for adult steroid-refractory central nervous system inflammatory demyelination.
- Limited data exists on PLEX efficacy in pediatric central nervous system inflammatory demyelination.
Purpose of the Study:
- To evaluate the effectiveness of plasma exchange in children and adolescents with acute severe central nervous system inflammatory demyelination.
Main Methods:
- Retrospective review of 12 pediatric patients treated with plasma exchange for acute severe central nervous system inflammatory demyelination.
- Analysis of clinical presentation, diagnosis, Expanded Disability Status Scale (EDSS) scores, and outcomes.
Main Results:
- Patients presented with spinal cord (10) or brainstem (2) lesions.
- Diagnoses included acute transverse myelitis (6), relapsing-remitting multiple sclerosis (5), and acute disseminated encephalomyelitis (1).
- Median EDSS score at treatment initiation was 7.5; 7 patients achieved ambulatory status (EDSS ≤4) at 3 months.
Conclusions:
- Plasma exchange may be effective in improving symptoms and functional outcomes in severe pediatric central nervous system inflammatory demyelination.
- Further randomized controlled trials are needed to confirm efficacy due to the retrospective nature of this study.
Abstract:
The use of plasma exchange has been described in steroid-refractory central nervous system inflammatory demyelination in adults, but less has been published regarding its use in children and adolescents. We describe 12 children treated with plasma exchange for acute severe central nervous system inflammatory demyelination. The clinical attack leading to plasma exchange included symptomatic spinal cord lesions in 10 and symptomatic brainstem lesions in 2 children. Diagnosis was acute transverse myelitis in 6, relapsing-remitting multiple sclerosis in 5, and acute disseminated encephalomyelitis in 1 child. Adverse events related to plasma exchange necessitating intervention were observed in 3 children. Median Expanded Disability Status Scale score at plasma exchange start was 7.5 (range 4-9.5). At 3 months, 7 children were ambulatory without aid (Expanded Disability Status Scale score of ≤4). This retrospective study suggests that plasma exchange can be effective in ameliorating symptoms in severe pediatric central nervous system inflammatory demyelination, although lack of randomization or control group limits the ability to attribute this outcome entirely to plasma exchange.
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