Achmeh Bassel, Wahbi Mohamad N, Daood Huda
Damascus University, Faculty of Medicine.
Lecturer at Damascus University, Faculty of Medicine, Damascus, Syria.
Ann Med Surg (Lond). 2024 Jul 8;86(9):5497-5500. doi: 10.1097/MS9.0000000000002353. eCollection 2024 Sep.
Acute hemorrhagic leukoencephalopathy (AHLE) is a rare and devastating condition that can present with various neurological symptoms. The predisposing and initiating factors of AHLE are not fully understood. AHLE has a high morbidity and significant mortality rates, however, our case presents a surviving young girl.
Thirteen years old previously healthy girl was referred to the emergency department due to drowsiness, preceded by an upper respiratory infection 10 days earlier. Firstly, she was treated empirical with antiviral medication (Acyclovir) directed to herpes simplex virus and intravenous (IV) methylprednisolone pulses. When she did not respond well, intravenous immunoglobulin was administrated, which helped with the end-result diagnosis based on clinical and imaging findings.
AHLE is a fatal rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter, it is usually misdiagnosed due to being a diagnosis of exclusion, and the much more common other diseases, including infectious encephalitis, meningitis, fulminant multiple sclerosis, other causes of acute disseminated encephalomyelitis. Different types of CNS infiltrates, such as neutrophils in AHLE and lymphocytes in acute disseminated encephalomyelitis, do not support the idea of differentiating the two diseases. The process of differentiating between these two diseases relies mostly on laboratory and imaging findings, which are well demonstrated in this case report.
The authors conclude this report by highlighting the dearth in published knowledge about this disease, and encouraging further studies be conducted about this topic.
急性出血性白质脑病（AHLE）是一种罕见且严重的疾病，可表现出多种神经症状。AHLE的诱发因素和起始因素尚未完全明确。AHLE的发病率和死亡率都很高，然而，我们的病例是一位幸存的年轻女孩。
一名13岁此前健康的女孩因嗜睡被转诊至急诊科，10天前有上呼吸道感染病史。起初，她接受了针对单纯疱疹病毒的抗病毒药物（阿昔洛韦）及静脉注射甲基泼尼松龙冲击治疗。当她反应不佳时，给予了静脉注射免疫球蛋白，这有助于根据临床和影像学检查结果进行最终诊断。
AHLE是一种致命的罕见脱髓鞘疾病，其特征是白质急性快速进展性暴发性炎症，由于它是一种排除性诊断，且常被误诊为其他更常见的疾病，包括感染性脑炎、脑膜炎、暴发性多发性硬化症、急性播散性脑脊髓炎的其他病因。不同类型的中枢神经系统浸润，如AHLE中的中性粒细胞和急性播散性脑脊髓炎中的淋巴细胞，并不支持区分这两种疾病的观点。区分这两种疾病的过程主要依赖于实验室和影像学检查结果，本病例报告对此进行了充分展示。
作者在本报告结尾强调了关于该疾病已发表知识的匮乏，并鼓励对这一主题进行进一步研究。
