Decompressive Hemicraniectomy for Severe Acute Disseminated Encephalomyelitis: A Case Report

Hadi A Rabee, Mohammad Gharbeyah, Ahmad Abuhassan, Jawad Ziyadeh, Hamza Hamayel, Amr Adas and Dina Abugaber

Department of Internal Medicine, An-Najah National University Hospital, Palestine Department of Critical Care, An-Najah National University Hospital, Palestine Department of Neurology, An-Najah National University Hospital, Palestine Department of Neurosurgery, An-Najah National University Hospital, Palestine These authors contributed equally to this work

^*Correspondance to: Hadi A Rabee 

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Abstract:

This report present a rare case of adult-onset severe Acute Disseminated Encephalomyelitis (ADEM) complicated by uncal herniation, which is a very rare complication of the disease, requiring lifesaving decompressive hemicraniectomy with complete recovery. This is a 27–year -old female, who presented with headache, right-sided body weakness, dysarthria, blurred vision, and progressive altered level of consciousness over a course of 2 days, preceded by an upper respiratory tract infection 7 days prior to the onset of the above - mentioned symptoms. Brain MRI showed extensive abnormal white matter lesions which appear hyperintense, involving occipital, parietal, and temporal lobes, more on the left side. CSF exam showed high protein. According to the clinical and MRI findings, she was diagnosed as severe ADEM. She was started on high dose steroids and plasma exchange. Unfortunately, her condition deteriorated rapidly with sudden drop in her level of consciousness with fixed dilated pupils. Urgent imaging showed uncal herniation. Lifesaving urgent left decompressive hemicraniectomy was performed with subsequent resolution of herniation radiologically and clinically. She was kept on steroid and plasmapheresis, followed by Intravenous Immunoglobulin (IVIG) with gradual improvement. After one month, she completely recovered and became independent in her activities, with significantly improved MRI lesions. In conclusions, severe ADEM complicated by uncal herniation is an extremely rare presentation of the disease. Urgent and aggressive interventions, including decompressive surgeries, can save lives and lead to favorable outcomes.

Keywords:

ADEM disease; Decompression; Craniectomy; IVIG

Cite the Article:

Rabee HA, Gharbeyah M, Abuhassan A, Ziyadeh J, Hamayel H, Adas A, et al. Decompressive Hemicraniectomy for Severe Acute Disseminated Encephalomyelitis: A Case Report. Ann Clin Case Rep. 2021; 6: 1972..