High-dose methylprednisolone and tocilizumab improve survival of patients with high-risk pediatric acute necrotizing encephalopathy.

ABSTRACT:

Background

Acute necrotizing encephalopathy (ANE) is a rare but devastating neurological disorder in children that is typically triggered by viral infections such as influenza, sudden acute respiratory syndrome coronavirus 2, and human herpesvirus-6. ANE is characterized by cytokine storm and associated with high mortality; however, optimal immunomodulatory strategies remain undefined.

Purpose

To evaluate the effectiveness of multiple immunomodulatory strategies, including high-dose methylprednisolone (MP), plasma exchange (PLEX), and tocilizumab, at reducing short-term mortality among pediatric patients with ANE stratified by disease severity using the ANE severity score (ANE-SS).

Methods

We retrospectively reviewed 65 pediatric patients (median age, 4.8 years; inter

SUBMITTER: Fan C 

PROVIDER: S-EPMC12790894 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature