Zi-Jie Cao,1,2 Yi Sun,1,2 Feng-Ling Li1,2 

1Department of Neurology, Affiliated Hospital of Shandong Second Medical University, Weifang, 261000, People’s Republic of China; 2School of Clinical Medicine, Shandong Second Medical University, Weifang, 261000, People’s Republic of China

Correspondence: Feng-Ling Li, Department of Neurology,Affiliated Hospital of Shandong Second Medical University, No. 2428 of Yuhe Road, Dayu District, Weifang, 261000, People’s Republic of China, Tel +8615953647606, Fax +8605363081268, Email lifenglinglfl@126.com

Abstract: Acute necrotizing encephalopathy (ANE) is a rare and rapidly progressing acute encephalopathy characterized by seizures and changes in consciousness. This condition typically affects the thalamus, brainstem, and cerebellum in a symmetrical manner, and if left untreated, is associated with significantly higher mortality rates. In recent years, with in-depth research on the etiology and pathogenesis of ANE, as well as continuous advances in clinical diagnosis and treatment techniques, the understanding of this disease has gradually increased. However, the complexity and diversity of ANE still pose significant challenges to clinical diagnosis and treatment. In addition, due to its rarity, many clinical doctors have insufficient understanding of it, resulting in a high rate of misdiagnosis and missed diagnosis. Therefore, this article reviews the etiology, pathogenesis, risk factors, pathology, clinical manifestations, auxiliary examinations, diagnostic methods, treatment strategies, and prognosis related to ANE. Its purpose is to enhance clinical doctors’ ability to identify and manage ANE, provide reference for accurate diagnosis and effective treatment, and support ongoing research aimed at gaining a deeper understanding of this serious disease.

Keywords: acute necrotizing encephalopathy, inflammation, MRI, RanBP2, viral infection