# Fulminant demyelinating disease of the central nervous system effectively treated with a combination of decompressive craniectomy and immunotherapy: A case report and literature review

**Authors:** Toshihiro Ide, Ryo Ebashi, Makoto Eriguchi, Shinichi Aishima, Tatsuya Abe, Hideo Hara

PMC · DOI: 10.1002/ccr3.9059 · 2024-07-09

## TL;DR

A rare brain disease was successfully treated with surgery and immunotherapy, leading to full recovery and no relapse for four years.

## Contribution

Demonstrates successful treatment of fulminant demyelinating disease using decompressive craniectomy and immunotherapy.

## Key findings

- A patient recovered fully after decompressive craniectomy and immunotherapy.
- The patient remained relapse-free for four years following treatment.
- Surgical intervention was effective when medical management failed.

## Abstract

Accurately identifying fulminant demyelinating diseases is important for sudden onset of asymmetric cerebral white matter lesions with mass effect. Initially, immunotherapy should be administered; however, surgical intervention should be performed with poor response to medical management and evident signs of cerebral herniation.

A case of fulminant demyelinating disease of the central nervous system that required decompressive craniectomy 8 days after symptom onset is presented. The patient recovered without sequelae after a combination of neurosurgery and immunotherapy with steroids and has remained relapse‐free for 4 years.

## Linked entities

- **Diseases:** central nervous system disease (MONDO:0002602)

## Full-text entities

- **Diseases:** demyelinating diseases (MESH:D003711), cerebral herniation (MESH:D004677), cerebral white matter lesions (MESH:D056784), demyelinating disease of the central nervous system (MESH:D020278)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11233259/full.md

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Source: https://tomesphere.com/paper/PMC11233259