Neuropediatrics 2021; 52(S 01): S1-S53
DOI: 10.1055/s-0041-1739635
Abstract Salzburg

Modified Zipper Method: A Promising Treatment Option in Severe Pediatric Cases of Immune-Mediated Neurological Disorders

Marc Nikolaus
1   Charité-Universitätsmedizin Berlin, Department of Pediatric Neurology, Berlin, Germany
2   Charité-Universitätsmedizin Berlin, Center for Chronically Sick Children, Berlin, Germany
,
Fabienne Kuehne
1   Charité-Universitätsmedizin Berlin, Department of Pediatric Neurology, Berlin, Germany
2   Charité-Universitätsmedizin Berlin, Center for Chronically Sick Children, Berlin, Germany
,
Anna Tietze
3   Universitätsmedizin Berlin, Department of Neuroradiology, Berlin, Germany
,
Ellen Knierim
1   Charité-Universitätsmedizin Berlin, Department of Pediatric Neurology, Berlin, Germany
2   Charité-Universitätsmedizin Berlin, Center for Chronically Sick Children, Berlin, Germany
,
Alexander Gratopp
4   Charité-Universitätsmedizin Berlin, Department of Pediatric Pneumology, Immunology and Intensive Care, Berlin, Germany
,
Petra Bittigau
1   Charité-Universitätsmedizin Berlin, Department of Pediatric Neurology, Berlin, Germany
2   Charité-Universitätsmedizin Berlin, Center for Chronically Sick Children, Berlin, Germany
,
Angela M. Kaindl
1   Charité-Universitätsmedizin Berlin, Department of Pediatric Neurology, Berlin, Germany
2   Charité-Universitätsmedizin Berlin, Center for Chronically Sick Children, Berlin, Germany
5   Charité-Universitätsmedizin Berlin, Institute of Cell Biology and Neurobiology, Berlin, Germany
› Author Affiliations
› Further Information
 

Background/Purpose: Guillain–Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are immune-mediated neurological disorders characterized by rapid-onset and progressive clinical course. Despite generally favorable outcome in children, severe cases are reported regularly. Immunotherapy regimes including intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) exist, but outcome of severe cases remains unsatisfactory and mortality high. In 2018, a novel treatment strategy with alternating IVIG and PLEX (“zipper method”) was reported in nine pediatric GBS patients with promising results. Here, we present a modified “zipper method” for treatment of various neuroimmunological diseases.

Methods: Modified “zipper method” comprised longer intervals between PLEX-IVIG cycles (48 hours instead of 24 hours), more cycles (7–10 instead of 5), plasma replacement with FFP (instead of albumin), and variable infusion times for IVIGs (4–8 hours, 0.4 g/kg/d). Modified “zipper method” was applied as individual treatment approach once standard therapy failed. Follow-up ranged from 6 months to 2 years. Cases were analyzed retrospectively.

Results: Four children (9–15 years) with severe immune-mediated neurological disorders were treated by modified “zipper method”: (1) Miller-Fisher syndrome, (2) Bickerstaff's brainstem encephalitis, (3) common GBS, (4) full-blown ADEM. Results for duration of mechanical ventilation (12 days), hospital stay (23 days), and time to unaided walking (38 days) exceed previous studies with IVIG/PLEX alone or IVIG + PLEX treatment combinations unlike “zipper method.” None of our patients died. All showed rapid restitution with excellent outcome.

Conclusion: Modified “zipper method” lowers mortality, reduces time on mechanical ventilation, shortens hospital stays, and leads to excellent outcome in children with severe GBS/ADEM. Our version is streamlined for easier applicability. Results emphasize its robust effectiveness in various neuroimmunological diseases.



Publication History

Article published online:
28 October 2021

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