Kalafatakis Konstantinos, Margoni Anna, Liakou Maria-Eleni, Stenos Christos, Toulas Panagiotis, Korkolopoulou Penelope, Lakiotaki Eleftheria, Lafazanos Spiridon A, Zekiou Katerina, Kardara Panagiota, Terentiou Aspasia, Nikolaou Georgios, Stouraitis Georgios
Department of Neurology, 417 Army Share Fund Hospital, Athens, Greece.
Faculty of Medicine & Dentistry (Malta Campus), Queen Mary University of London, Victoria, Malta.
Heliyon. 2024 Feb 2;10(3):e25545. doi: 10.1016/j.heliyon.2024.e25545. eCollection 2024 Feb 15.
Acute hemorrhagic leukoencephalitis (AHLE), is a rare inflammatory demyelinating disorder, variant of acute disseminated encephalomyelitis. The diagnosis of AHLE remains challenging due to the rarity of the disease and the lack of a reliable biomarker. We report here a case of a 73-year-old male patient with a progressive, low-grade febrile confusional syndrome 20 days after receiving the first dose of BNT162b2 vaccine against SARS-CoV-2. Evidence indicative of the underlying condition by an extensive panel of imaging (brain magnetic resonance imaging, computed tomography and digital subtraction angiography), laboratory (complete blood count, biochemistry, coagulation, tests for autoimmune or infectious disorders, tumor markers, hormonal levels, cerebrospinal fluid analysis) and electrodiagnostic tests were scarce, and mainly non-specific. Sequential neuroimaging revealed the appearance of extensive T2 lesions (signs of gliosis) along with multiple hemorrhagic lesions at various cortical sites. The patient was treated with corticosteroids, discontinued due to severe adverse effects, and subsequently with sessions of plasmapheresis and monthly intravenous administration of cyclophosphamide. Considering the rapid aggravation of the patient's neurological status, the MRI findings of cortical lesions and the lack of response to any treatment, a biopsy of a frontal lobe lesion was conducted, confirming the presence of confluent, inflammatory-edematous lesions with scattered areas of necrosis and hemorrhage, and ultimately areas of demyelination, thus confirming the diagnosis of AHLE. After more than 5 months of hospitalization the patient was transferred in a primary care facility and remained in a permanent vegetative state until his death, more than 2 years later.
急性出血性白质脑炎（AHLE）是一种罕见的炎症性脱髓鞘疾病，是急性播散性脑脊髓炎的一种变异型。由于该疾病罕见且缺乏可靠的生物标志物，AHLE的诊断仍然具有挑战性。我们在此报告一例73岁男性患者，在接种第一剂抗SARS-CoV-2的BNT162b2疫苗20天后出现进行性、低热性意识模糊综合征。通过广泛的影像学检查（脑磁共振成像、计算机断层扫描和数字减影血管造影）、实验室检查（全血细胞计数、生化检查、凝血检查、自身免疫或感染性疾病检测、肿瘤标志物、激素水平、脑脊液分析）和电诊断检查，提示潜在疾病的证据很少，且主要是非特异性的。连续的神经影像学检查显示，在多个皮质部位出现广泛的T2病变（胶质增生迹象）以及多个出血性病变。患者接受了皮质类固醇治疗，但因严重不良反应而停药，随后进行了血浆置换治疗，并每月静脉注射环磷酰胺。考虑到患者神经状态迅速恶化、皮质病变的MRI表现以及对任何治疗均无反应，对额叶病变进行了活检，证实存在融合性、炎症性水肿病变，伴有散在的坏死和出血区域，最终出现脱髓鞘区域，从而确诊为AHLE。住院5个多月后，患者被转至初级保健机构，一直处于永久性植物人状态，直到2年多后死亡。
