Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, 36-1 Nishi-Cho, Yonago, Tottori 683-8504, Japan; Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii-cho Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan.
Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, 36-1 Nishi-Cho, Yonago, Tottori 683-8504, Japan.
Brain Dev. 2022 Feb;44(2):122-130. doi: 10.1016/j.braindev.2021.09.011. Epub 2021 Oct 13.
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic seizures and white matter lesions with reduced diffusion, which are often accompanied by involuntary movements. The neurological outcomes of AESD vary from normal to mild or severe sequelae, including intellectual disability, paralysis, and epilepsy. The present study aimed to clarify the prognostic factors of AESD, including involuntary movements.
We enrolled 29 patients with AESD admitted to Tottori University Hospital from 1991 to 2020 and retrospectively analyzed their clinical data. Neurological outcomes were assessed by the Pediatric Cerebral Performance Category score and cerebral paralysis as neurological sequelae.
Of the 29 patients, 12 had favorable outcomes and 17 had unfavorable outcomes. Univariate analysis revealed that the presence of underlying diseases, a decline in Glasgow Coma Scale (GCS) score 12-24 h after early seizures, and involuntary movements were associated with unfavorable outcomes. In multivariate analysis, a decline in GCS score and involuntary movements were associated with unfavorable outcomes. The sensitivities and specificities of underlying diseases, a decline of ≥ 3 points in GCS score 12-24 h after early seizures, and involuntary movements for unfavorable outcomes were 53% and 92%, 92% and 65%, and 59% and 92%, respectively.
The appearance of involuntary movements may be associated with unfavorable outcomes of AESD. The prognostic factors identified herein are comparable with previously known prognostic factors of consciousness disturbances after early seizures.
急性脑病伴双相发作和后期弥散受限（AESD）的特征是双相发作和弥散受限的白质病变，常伴有不自主运动。AESD 的神经结局从正常到轻度或重度后遗症不等，包括智力障碍、瘫痪和癫痫。本研究旨在阐明 AESD 的预后因素，包括不自主运动。
我们纳入了 1991 年至 2020 年期间在鸟取大学医院住院的 29 例 AESD 患者，并对其临床资料进行了回顾性分析。神经结局通过儿科脑功能分类评分和脑瘫作为神经后遗症进行评估。
29 例患者中，12 例预后良好，17 例预后不良。单因素分析显示，存在基础疾病、早期发作后 12-24 小时格拉斯哥昏迷量表（GCS）评分下降以及不自主运动与不良预后相关。多因素分析显示，GCS 评分下降和不自主运动与不良预后相关。基础疾病、早期发作后 12-24 小时 GCS 评分下降≥3 分和不自主运动对不良预后的敏感性和特异性分别为 53%和 92%、92%和 65%以及 59%和 92%。
不自主运动的出现可能与 AESD 的不良预后相关。本研究确定的预后因素与早期发作后意识障碍的已知预后因素相当。
