• Chen Wu, Weiliang Zhang, Yan Jiao, Minhui Dong, Heng Zhou, Yun Lv, and Jun Yang.
• Department of Neurology, Xi'an Jiaotong University Second Affiliated Hospital, Shaanxi, China.
• Medicine (Baltimore). 2022 Dec 2; 101 (48): e32073e32073.

RationaleAcute hemorrhagic leukoencephalitis (AHLE) is a rare but fetal fulminant demyelinating disease of unknown etiology. It is commonly regarded as a severe variant of acute demyelinating encephalomyelitis (ADEM). Its rapid clinical deterioration and high mortality appeal to clinicians to attach importance to early diagnosis. Immunosuppressive therapy is the main management to attenuate the autoimmune process, but with varied response and prognosis.Patient ConcernsA young male presented with moderate fever, headache and seizures after extraction of impacted teeth, and then deteriorated rapidly to deep coma. Initial magnetic resonance imaging (MRI) revealed multiple plaque-like lesions in bilateral cerebra, right thalamus and pontobulbar region with massive edematous swelling and multifocal small hemorrhagic foci. Inflammatory parameters in the peripheral blood were only mild higher with a pleocytosis in CSF.DiagnosisHis clinical presentation, laboratory evaluation and radiological features were consistent with a suspected diagnosis of AHLE.InterventionsHe underwent pulse corticosteroids initially but failed to respond to it. However, his consciousness improved obviously when he was treated with multiple courses of intravenous injection of immunoglobulin (IVIG) combined with mycophenolate mofetil (MMF).OutcomesThe patient regained consciousness gradually on day 180 and was in minimally conscious state (MCS) during the two-year follow-up.LessonsAHLE presents distinctly from classical ADEM, and the situation may pose a diagnostic challenge. Clinicians should be vigilant in recognizing AHLE because of its rapid clinical deterioration and high mortality. We highlight the critical role of multimodal MRI, particularly susceptibility-weighted imaging (SWI) in the diagnosis of AHLE if cerebral biopsies are unavailable. Multiple courses of IVIG with MMF may be effective when early single pulse of corticosteroids fails. Individual who survives the initial insult may carry relatively good prognosis.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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