Decompressive craniectomy as a lifesaving intervention for acute disseminated encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM), is an immune-mediated demyelinating disease of the central nervous system that commonly affects children and young adults of both sexes. Hyperacute variants of ADEM represent 2% of cases and are associated with rapid progression of symptoms, malignant brain edema, and high mortality rates. We report a case of a young woman presenting with a hyperacute storming course of few days who was managed with pulse steroid therapy and emergency craniectomy with an excellent outcome. We believe that our patient's acute clinical deterioration and findings on neuroimaging warranted prompt neurosurgery. Although treatment with immunomodulatory medications was commenced, the severity of her condition indicated that only surgical intervention was likely to be lifesaving. We recommend immediate neurosurgical consultation to consider prompt decompressive craniectomy in hyperacute variants with significant brain swelling. Multidisciplinary care including neurologist, neuroradiologist, neurosurgeon, neuropathologist, and neurointensivist is the only way to achieve success and improve survival in patients presenting with hyperacute ADEM.