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

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.

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.

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.

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.

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.