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表现为双相性癫痫发作和晚期扩散受限的急性脑病(AESD)的婴儿缺氧性脑病被诊断为一次短暂性不明原因事件(BRUE)发作。

Infantile Hypoxic Encephalopathy Mimicking Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion (AESD) Identified as an Episode of Brief Resolved Unexplained Event (BRUE).

作者信息

Fujino Shuhei, Enokizono Mikako, Kono Tatsuo, Miyama Sahoko

机构信息

Department of Neurology, Tokyo Metropolitan Children's Medical Center, Tokyo 183-8561, Japan.

Department of Radiology, Tokyo Metropolitan Children's Medical Center, Tokyo 183-8561, Japan.

出版信息

J Clin Med. 2023 Aug 11;12(16):5239. doi: 10.3390/jcm12165239.

Abstract

Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is characterized by biphasic seizures following febrile viral infections and delayed reduced diffusion of the cerebral white matter on magnetic resonance imaging (MRI) diffusion-weighted imaging (DWI) (bright tree appearance, BTA). However, hypoxic encephalopathy with biphasic seizures and AESD-mimicking imaging findings has not been reported. We report a case of hypoxic encephalopathy due to suffocation with concomitant biphasic seizures and BTA, mimicking AESD. On day 1, a healthy 5-month-old girl was found face down with decreased breathing and a deteriorating consciousness level, suggesting a brief resolved unexplained event (BRUE). Electroencephalography (EEG) revealed periodic epileptic discharges, suggesting possible nonconvulsive status epilepticus. Despite improvements in consciousness level and EEG abnormalities on day 2, her consciousness level deteriorated again with generalized tonic-clonic seizures on day 3, and a head MRI-DWI revealed restricted diffusion predominantly in the subcortical areas, suggesting BTA. Treatment for acute encephalopathy resolved the clinical seizures and EEG abnormalities. Persistence of abnormal EEG, reflecting abnormal excitation and accumulation of neurotoxic substances caused by hypoxia, may have contributed to the development of AESD-like findings. As hypoxic encephalopathy causes AESD-like biphasic seizures, monitoring consciousness level, seizure occurrence, and EEG abnormalities even after acute symptoms have temporarily improved following hypoxia is essential.

摘要

伴有双相性癫痫发作和弥散受限的急性脑病(AESD)的特征是在发热性病毒感染后出现双相性癫痫发作,并且在磁共振成像(MRI)弥散加权成像(DWI)上脑白质的弥散受限延迟出现(亮树征,BTA)。然而,伴有双相性癫痫发作和类似AESD影像学表现的缺氧性脑病尚未见报道。我们报告一例因窒息导致的缺氧性脑病,伴有双相性癫痫发作和BTA,酷似AESD。第1天,一名健康的5个月大女孩被发现面朝下,呼吸减弱,意识水平下降,提示短暂不明原因事件(BRUE)。脑电图(EEG)显示周期性癫痫放电,提示可能为非惊厥性癫痫持续状态。尽管第2天意识水平和EEG异常有所改善,但第3天她的意识水平再次恶化,出现全身强直阵挛性发作,头部MRI-DWI显示主要在皮质下区域弥散受限,提示BTA。急性脑病的治疗使临床癫痫发作和EEG异常得到缓解。EEG异常持续存在,反映了缺氧引起的神经毒性物质的异常兴奋和蓄积,可能促成了类似AESD表现的出现。由于缺氧性脑病可导致类似AESD的双相性癫痫发作,即使在缺氧后急性症状暂时改善后,监测意识水平、癫痫发作情况和EEG异常也是至关重要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/adae/10455681/3204f4ae57cd/jcm-12-05239-g001.jpg

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