Decompressive hemicraniectomy for fulminant acute disseminated encephalomyelitis following dengue fever: Case report and literature review

Background: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated demyelinating disorder of the central nervous system that typically occurs after viral or bacterial infections. Although most patients respond favorably to immunomodulatory therapy, a small subset develops fulminant ADEM characterized by malignant cerebral edema, refractory intracranial hypertension, and a high risk of mortality. Decompressive craniectomy has rarely been reported as a life-saving intervention in such cases. Case Description: A 25-year-old woman developed fulminant ADEM following dengue fever and initially presented with recurrent seizures progressing to status epilepticus. Brain magnetic resonance imaging demonstrated extensive bilateral demyelinating lesions involving the frontoparietal, occipital, temporal, and cerebellar white matter, with extension into the corpus callosum. Despite aggressive medical management, including high-dose intravenous methylprednisolone, osmotherapy, sedation, and plasma exchange, the patient experienced progressive neurological deterioration associated with diffuse cerebral edema, anisocoria, and radiological evidence of impending cerebral herniation. Owing to the failure of maximal medical therapy, a left decompressive hemicraniectomy was performed. The patient subsequently underwent additional immunomodulatory treatment, survived the acute phase of the disease, and demonstrated significant neurological recovery. A review of the literature identified nine previously reported cases of fulminant ADEM treated with decompressive craniectomy. Conclusion: Fulminant ADEM is an uncommon but potentially fatal neurological emergency. In cases complicated by malignant cerebral edema and refractory intracranial hypertension, decompressive craniectomy may represent a life-saving therapeutic option. Early recognition and timely surgical intervention appear to be associated with favorable neurological outcomes.