Modified zipper method in steroid-resistant ADEM: a case report.

Abstract

Background: Acute disseminated encephalomyelitis (ADEM) is a rare, inflammatory, demyelinating central nervous system disorder typically responsive to corticosteroid therapy. However, some cases are resistant to steroids, requiring alternative treatment options.

Case presentation: We present an 8-year-old male child with steroid-resistant ADEM who underwent treatment with the modified zipper technique, combining intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Despite initial high-dose intravenous methylprednisolone, which failed to improve his condition, the patient showed significant clinical and radiological improvement after five cycles of the modified zipper technique.

Methods: The patient received alternating cycles of TPE and IVIG (0.4 g/kg/day) over five cycles, with a 48-hour interval between each TPE and IVIG session. TPE sessions used fresh frozen plasma, followed by IVIG to enhance antibody clearance and immunomodulation.

Results: After two cycles of TPE, clinical improvement was observed. Follow-up MRI on day 15 showed marked regression of white matter lesions. The patient was discharged on day 20 with near-complete recovery. Follow-up at one, three, and six months showed no neurological deficits, and MRI lesions continued to regress.

Conclusions: This case suggests the modified zipper technique as a promising alternative for steroid-resistant ADEM. The combination of TPE and IVIG may provide beneficial effects, but further controlled studies are needed to evaluate its efficacy and safety in broader populations.