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

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.

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.

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