Shukla Ankit, Nayyar Nishant, Kumari Pooja, Kumar Ankush, Takkar Preeti
Department of Surgery, Dr Rajendra Prasad Government Medical College, Kangra 176001, Himachal Pradesh, India.
Department of Radiology, Dr Rajendra Prasad Government Medical College, Kangra 176001, Himachal Pradesh, India.
World J Clin Cases. 2025 Oct 6;13(28):107759. doi: 10.12998/wjcc.v13.i28.107759.
Acute hemorrhagic leukoencephalitis (AHLE), also known as Weston-Hurst syndrome, is a very rare and fulminant form of demyelinating disorder. It is considered a hyperacute and severe variant of acute disseminated encephalomyelitis. Clinically, patients present with fever, headache, seizures, and altered sensorium, which can rapidly progress to coma or death. Magnetic resonance imaging (MRI) is the investigation of choice and plays a pivotal role in diagnosing AHLE. The purpose of this article is to make readers familiar with the typical MRI features of AHLE and to discuss differentials.
This case series reports the clinical presentation and typical neuroimaging findings in four patients diagnosed with AHLE. All patients presented with acute neurological symptoms, such as severe headaches, seizures, and altered consciousness, often following a history of fever suggesting an infectious etiology. Additionally, laboratory investigations demonstrated elevated levels of serum inflammatory markers and neutrophilic pleocytosis on cerebrospinal fluid analysis, supporting a post-infectious etiology. MRI findings consistently revealed characteristic white matter lesions with hemorrhagic foci and vasogenic edema, indicative of widespread demyelination characteristic of AHLE. The outcomes varied, with two patients surviving but experiencing neurological sequelae, while two others unfortunately succumbed to the disease. The clinical data, laboratory results, and imaging findings from this case series were systematically compared with those from previously published studies. The key similarities and differences in clinical presentation, imaging characteristics, and outcomes are presented in a tabulated format.
AHLE is associated with high morbidity and mortality rates, emphasizing the need for early recognition, prompt intervention, and multidisciplinary management. Further research is needed to explain the pathophysiological mechanisms underlying AHLE, identify potential biomarkers for early diagnosis, and develop targeted therapies to improve patient outcomes.
急性出血性白质脑炎（AHLE），也称为韦斯顿 - 赫斯特综合征，是一种非常罕见且暴发性的脱髓鞘疾病。它被认为是急性播散性脑脊髓炎的超急性和严重变体。临床上，患者表现为发热、头痛、癫痫发作和意识改变，可迅速进展为昏迷或死亡。磁共振成像（MRI）是首选的检查方法，在诊断AHLE中起关键作用。本文的目的是使读者熟悉AHLE的典型MRI特征并讨论鉴别诊断。
本病例系列报告了4例诊断为AHLE患者的临床表现和典型神经影像学表现。所有患者均出现急性神经症状，如严重头痛、癫痫发作和意识改变，通常在有发热病史提示感染病因之后出现。此外，实验室检查显示血清炎症标志物水平升高，脑脊液分析显示中性粒细胞增多，支持感染后病因。MRI表现一致显示具有出血灶和血管源性水肿的特征性白质病变，提示AHLE特征性的广泛脱髓鞘。结果各不相同，2例患者存活但有神经后遗症，而另外2例不幸死于该病。将该病例系列的临床数据、实验室结果和影像学表现与先前发表的研究进行了系统比较。以表格形式列出了临床表现、影像学特征和结果的关键异同点。
AHLE与高发病率和死亡率相关，强调需要早期识别、及时干预和多学科管理。需要进一步研究来解释AHLE的病理生理机制，确定早期诊断的潜在生物标志物，并开发针对性治疗以改善患者预后。
