Submitted by: Leigh Maria Ramos-Platt
Edited by: Hristelina Ilieva, MD, PhD
Kesici, S. et al. A novel treatment strategy for severe Guillain-Barre syndrome: The zipper method
. Journal of Child Neurology
2019 DOI: 10.1177/0883073819826225.
Kesici et al describe a novel approach in the treatment of pediatric patients ages 6-16 years of age, with acute motor axonal neuropathy in an open-label study. In their study, 9 patients, who all presented within 2 days of symptom (complaint) onset, were admitted to the intensive care unit because of respiratory failure (required mechanical ventilation), had CSF studies within a day of admission, and were confirmed to have acute motor axonal neuropathy (EMG performed within 4 days of presentation). Not all patients had elevated protein. GM1 antibodies were not performed.
The zipper strategy consists of the following:
- Day 1: Treatment with 1.5x plasma exchange with 5% albumin as replacement followed by 0.4 grams/kg of IVIG immediately after
- Day 2: After 24 hours, 1x plasma exchange with 5% albumin followed by 0.4 grams/kg of IVIG
- Day 3: 1x plasma exchange with fresh-frozen plasma followed by 0.4 grams/kg of IVIG
- Day 4: A repeat of day 2
- Day 5: A repeat of day 2
This resulted in the following:
1. Mechanical ventilation need of 5-14 days and no permanent tracheostomy placement
2. A hospital stay of 10-30 days
3. All patients were able to walk unassisted by day 28
All of these results are superior to those reported in adult patients (in the medical literature) who received IVIG or plasmapheresis alone.
The worse outcome patients with acute motor axonal neuropathy (AMAN) were discussed in this article. It is also something we see in practice. This article is interesting as it presents the use of concomitant therapies. In practice, we tend to do sequential treatments rather than concomitant treatments. A larger, multi-center, randomized, controlled with single- either IVIG or PE -treatment group (s) study could help interpret the above findings. The jury is still out if this regimen will be supported in a more robust trial. Because of the rarity of AMAN patients, those trials may be difficult to execute in western countries. The practical use of this protocol in terms of insurance coverage may also be problematic until further proof is collected.