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病例报告:暴发性急性出血性白质脑炎(AHLE):一种伴有脑疝形成(与COVID-19相关)的罕见且严重的结局。

CASE REPORT: Fulminant acute hemorrhagic Leukoencephalitis (AHLE): A rare and ruinous outcome with cerebral herniation (COVID-19).

作者信息

Safan Abeer Sabry, Noorain Zeba, Atta Mohamed A, Thekkoth Razna, Suliman Aasir M, Fadlelmula Abdalrazig, Abdelatey Mohammed

机构信息

Department of Neurology, Neurosciences Institute, Hamad Medical Corporation, Doha, Qatar.

Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.

出版信息

eNeurologicalSci. 2024 Apr 6;35:100499. doi: 10.1016/j.ensci.2024.100499. eCollection 2024 Jun.

Abstract

BACKGROUND

Acute hemorrhagic leukoencephalitis (AHLE) is a very rare demyelinating disease with rapid fulminant inflammation of the white matter. Although the exact etiology is unknown, AHLE usually manifests post a viral or bacterial infection and less often seen post vaccination for measles or rabies. AHLE has a very poor prognosis and a high mortality rate. Owing to the rarity of this entity there is not clear consensus on the proper line of management. In this report, we present a case of AHLE as a para-infectious sequel to COVID-19 in a young patient.

CLINICAL PRESENTATION

We report a 30-year-old turkish patient with an initial presentation of upper respiratory tract infection due to COVID-19. Initially, she was admitted to the hospital with generalized tonic-clonic seizure (GTCS) and deterioration in her level of consciousness lapsing into a coma. An initial CT scan showed diffuse brain edema and an MRI head confirmed the suspicion of Acute hemorrhagic leukoencephalitis (AHLE). Despite prompt and diligent osmotic therapy and pulsed intravenous (IV) methylprednisolone, her condition rapidly depreciated and progressed into cerebral edema with gravid sequela of brainstem herniation.

CONCLUSIONS

AHLE is a very rare entity and perhaps its fulminant debilitating course and high mortality should warrant further studies on disease pathophysiology and its optimal treatment parameters. Life-saving decompressive hemicraniectomy should be considered in the multidisciplinary approach of the management with tailored osmotic and immunotherapy.

摘要

背景

急性出血性白质脑炎(AHLE)是一种非常罕见的脱髓鞘疾病,其白质会迅速发生暴发性炎症。尽管确切病因尚不清楚,但AHLE通常在病毒或细菌感染后出现,较少见于麻疹或狂犬病疫苗接种后。AHLE预后很差,死亡率很高。由于这种疾病罕见,对于恰当的治疗方案尚无明确共识。在本报告中,我们介绍了一名年轻患者,其AHLE是新冠病毒感染后的副感染性后遗症。

临床表现

我们报告了一名30岁的土耳其患者,最初表现为新冠病毒感染所致的上呼吸道感染。最初,她因全身强直阵挛性发作(GTCS)和意识水平恶化陷入昏迷而入院。最初的CT扫描显示弥漫性脑水肿,头部MRI证实了急性出血性白质脑炎(AHLE)的怀疑。尽管迅速且积极地进行了渗透性治疗和静脉注射甲基强的松龙冲击治疗,她的病情仍迅速恶化,进展为脑水肿并伴有脑干疝的严重后遗症。

结论

AHLE是一种非常罕见的疾病,或许其暴发性的衰弱病程和高死亡率应促使对疾病病理生理学及其最佳治疗参数进行进一步研究。在采用定制的渗透性和免疫疗法进行多学科管理时,应考虑进行挽救生命的减压性颅骨切除术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/924f/11019095/6ccc44297a0a/gr1.jpg

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