Objective and importance: Acute disseminated encephalomyelitis (ADEM) varies widely in symptoms and severity. Some cases are associated with massive life-threatening cerebral edema refractory to conventional medical management.
Clinical presentation: A 51-year-old woman with ADEM who developed severe brain swelling and herniation despite aggressive medical management is described.
Intervention: A decompressive hemicraniectomy and durotomy led to rapid improvement and an excellent outcome.
Conclusion: This case report reinforces the place of this procedure in the armamentarium of treatment options for patients with medically refractory brain swelling and elevated intracranial pressure caused by ADEM. The potential for an increase in the incidence of ADEM with more frequent smallpox vaccinations emphasizes the significance of redefining the full range of management options for this treatable disease.