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Abstract
Objectives To determine the safety and clinical benefit of therapeutic plasma exchange (TPE) as rescue therapy in children with acute inflammatory demyelinating CNS syndromes and to identify baseline prognostic indicators of treatment improvement.
Methods This single-center retrospective pediatric cohort included all consecutive patients admitted to our hospital over the period from 2003 to 2017 because of a steroid-refractory acute CNS event presumed to be inflammatory who required TPE. Functional status assessment to identify improvement included the following performance category scales: visual outcome, bladder control, gait, and Expanded Disability Status Scale (EDSS). These assessments were performed before and after TPE in every patient.
Results Sixty-five children requiring TPE to treat 78 CNS attacks were included for analysis. Median age at TPE was 10.5 years (1.9–18 years); 45% were girls. Seropositivity (aquaporin-4 water channel–immunoglobulin G [IgG] or myelin oligodendrocyte glycoprotein–IgG) was found in 20 of 42 (48%) patients. Attack phenotypes leading to TPE were optic neuritis (ON) in 42%, longitudinally extensive transverse myelitis (LETM) in 31%, ON + LETM in 15%, and other combined syndromes in 11%. Overall, moderate to marked neurologic improvement was observed in 72% of children at the end of TPE and in 88.5% at 6 months of follow-up. Lower baseline scores on the EDSS, visual outcome, and gait scales were found to be independent prognostic indicators of treatment benefit. Sex, age at onset and at TPE, attack phenotype, disease duration, and time from attack onset to TPE initiation were not significantly associated with treatment outcome. Adverse events were observed in 31 of 524 (5.9%) procedures, being severe in 4.
Conclusions TPE was an effective rescue therapy associated with functional improvement. No therapeutic window for TPE initiation was identified in this pediatric cohort. Overall frequency of adverse events was low; however, serious events should be anticipated and avoided.
Classification of evidence This study provides Class IV evidence that for children with acute inflammatory demyelinating CNS syndromes, TPE leads to functional improvement.
Glossary
- ADEM=
- acute disseminated encephalomyelitis;
- AQP4=
- aquaporin-4 water channel;
- BCS=
- bladder control scale;
- CI=
- confidence interval;
- CVC=
- central vein catheter;
- EDSS=
- Expanded Disability Status Scale;
- GS=
- gait scale;
- HDIC=
- high-dose IV corticosteroids;
- IgG=
- immunoglobulin G;
- LETM=
- longitudinally extensive transverse myelitis;
- MOG=
- myelin oligodendrocyte glycoprotein;
- MS=
- multiple sclerosis;
- NMOSD=
- neuromyelitis optica spectrum disorder;
- ON=
- optic neuritis;
- OR=
- odds ratio;
- TPE=
- therapeutic plasma exchange;
- VOS=
- visual outcome scale
Footnotes
Presented in part as a poster at the 34th Congress of the European Committee for Treatment and Research in Multiple Sclerosis, October 10 to 12, 2018, Berlin, Germany; and the International Child Neurology Congress, November 15 to 18, 2018, Mumbai, India.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Editorial, page 953
Class of Evidence: NPub.org/coe
Podcast: NPub.org/ujyrl1
CME Course: NPub.org/cmelist
- Received March 11, 2019.
- Accepted in final form July 19, 2019.
- © 2019 American Academy of Neurology
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