Consultant Neurologist, Neurology Department, Deenanath Mangeshkar Hospital and Research Center, Pune, India; Consultant Neurologist, Neurology Department, Noble Hospital, Pune, India.
Consultant Neurologist, Neurology Department, Deenanath Mangeshkar Hospital and Research Center, Pune, India.
J Neuroimmunol. 2021 Dec 15;361:577751. doi: 10.1016/j.jneuroim.2021.577751. Epub 2021 Oct 13.
Acute haemorrhagic leukoencephalitis (AHLE), a rare variant of acute disseminated encephalomyelitis (ADEM), often presents differently from classical ADEM, thereby posing a diagnostic challenge to the clinician.
To report AHLE, its clinic-radiological manifestations, process of diagnosis and prognosis.
Eight patients presented with altered sensorium, acute focal deficits with or without seizures. Initial workup showed evidence of haemorrhagic lobar or thalamic lesions in seven patients. All patients underwent extensive evaluation for collagen vascular disease and vasculitis profile, autoimmune encephalitis panel and aquaporin-4 antibody, which were found to be normal. Cerebrospinal fluid (CSF) biochemistry and microscopy was non-contributory and CSF viral PCRs, toxoplasma antibodies, cryptococcal antigen were also negative. All patients had progressively worsening sensorium and neurological deficits. Repeat MRIs showed increase in oedema in the lesions and appearance/expansion of haemorrhage in the thalamic/hemispherical lesions. All patients received intravenous methylprednisolone (IVMP) without any benefit. Four patients underwent plasmapheresis (PLEX), one received intravenous immunoglobulin (IVIG) and one received both second line immunotherapies, without significant improvement. Brain biopsy (performed in three patients) showed inflammatory demyelination and areas of haemorrhage, thus confirming the diagnosis. Six patients succumbed in 7-30 days of the illness, despite aggressive treatment and only two survived, albeit with a significant disability.
AHLE is a rare, yet very severe variant of ADEM. MRI shows lesions with haemorrhages, oedema and mass effect and histology findings reveal inflammatory infiltrates, haemorrhagic foci and fibrinoid necrosis of vessel walls. Prognosis is worse as compared to the classic ADEM, with a high mortality rate. To the best of our knowledge, this is one of the largest series of AHLE to have been reported anywhere in the world.
Acute encephalopathy, multifocal deficits accompanied by haemorrhagic CNS demyelinating lesions with oedema and mass effect are the key features of AHLE. It is a rare, yet very severe form of ADEM with very high morbidity and mortality.
急性出血性脑白质脑炎（AHLE）是急性播散性脑脊髓炎（ADEM）的一种罕见变体，其临床表现常与经典 ADEM 不同，从而对临床医生构成诊断挑战。
报告 AHLE 的临床-影像学表现、诊断过程和预后。
8 例患者以意识改变、急性局灶性缺失伴或不伴癫痫发作为表现。7 例患者的初始检查显示出出血性大脑半球或丘脑病变的证据。所有患者均接受了广泛的结缔组织病和血管炎谱、自身免疫性脑炎谱和水通道蛋白-4 抗体评估，结果均正常。脑脊液（CSF）生化和显微镜检查无明显异常，CSF 病毒 PCR、弓形虫抗体和隐球菌抗原也均为阴性。所有患者的意识和神经功能缺损均进行性恶化。重复 MRI 显示病变水肿增加，丘脑/半球病变出现/扩大出血。所有患者均接受了静脉注射甲基强的松龙（IVMP）治疗，但均无获益。4 例患者接受了血浆置换（PLEX）治疗，1 例接受了静脉注射免疫球蛋白（IVIG）治疗，1 例接受了二线免疫治疗，但均无明显改善。3 例患者进行了脑活检，结果显示炎症性脱髓鞘和出血区，从而确诊。6 例患者在发病后 7-30 天内死亡，尽管进行了积极治疗，但只有 2 例存活，尽管有严重的残疾。
AHLE 是 ADEM 的一种罕见但非常严重的变体。MRI 显示病变伴有出血、水肿和占位效应，组织学检查发现炎症浸润、出血灶和血管壁纤维蛋白样坏死。与经典 ADEM 相比，其预后更差，死亡率较高。据我们所知，这是全球范围内报告的最大的 AHLE 系列之一。
急性脑病、多灶性缺损伴出血性中枢神经系统脱髓鞘病变伴水肿和占位效应是 AHLE 的关键特征。它是 ADEM 的一种罕见但非常严重的形式，发病率和死亡率都很高。
