Plasma exchange in pediatric central nervous system inflammatory demyelination

Aims: Evaluation of the clinical features and treatment response to plasma exchange (PLEX) in children with severe inflammatory central nervous system (CNS) demyelination.

PLEX has been described in steroid refractory CNS inflammatory demyelination in adults, but few are known in children with CNS inflammatory demyelination.

Methods: Retrospective review of prospectively collected data from 409 consecutive children presenting at a tertiary pediatric hospital between 1999 and 2012. Included were all children who were treated with PLEX for an inflammatory CNS demyelination. Written informed consent was obtained. Case report forms, charts, and MRI scans were reviewed. Level of disability was scored using the expanded disability status scale (EDSS) and evaluated at PLEX start, at discharge and at 3 months after PLEX.

Results: About 12 patients (9 female) who were receiving PLEX were identified (average age 12 years, range 7 to 18 years). Diagnosis was acute transverse myelitis in 6/12, relapsing-remitting multiple sclerosis in 5/12, and ADEM in 1/12. Index attack leading to PLEX was symptomatic cord lesions in 10/12 and symptomatic brainstem lesions in 2/12. Therapy preceding PLEX included methylprednisolone in 7/12, IVIG in 1/12, methylprednisolone and IVIG in 2/12. About 2/12 received PLEX as initial therapy. Median time from symptom onset to PLEX was 6 days (range 1 to 24). Median EDSS at PLEX start was 7.25 (range 4 to 9.5). Three children experienced: hypersensitivity reaction (1), anemia necessitating transfusion (1), thrombosis (1), and hypotension necessitating intravenous fluids (2). Median time from PLEX start to discharge was 15 days (range 9 to 88). Median EDSS at discharge was 5.75 (range 2 to 9.5) and at 3 months 4 (range 0 to 8.5). At 3 months, 7/12 had an EDSS of < 4.

Conclusion: We describe our experience with PLEX in children with severe CNS demyelination. Side effects necessitating intervention were observed in 25%. Over half of the children in this series regained independent ambulation 3 months after PLEX. Given the retrospective nature and small cohort, prospective studies in larger pediatric cohorts are warranted.