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首页> 外文期刊>Prion >Focal sharp waves are a specific early-stage marker of the MM2-cortical form of sporadic Creutzfeldt-Jakob disease
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Focal sharp waves are a specific early-stage marker of the MM2-cortical form of sporadic Creutzfeldt-Jakob disease

机译：焦点尖锐是MM2-皮质形式的孢子克雷托茨菲尔特-JAKOB疾病的特定早期标记

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摘要

Periodic sharp wave complexes (PSWCs), identified using electroencephalography, are observed in less than half of patients with the methionine homozygosity type 2 cortical (MM2c) form of sporadic Creutzfeldt-Jakob disease (sCJD), and only at a later stage of the disease. In this study, we identified early and specific markers on the electroencephalograms (EEGs) of patients with MM2c-sCJD. We retrospectively investigated the clinical records, EEGs, and magnetic resonance imaging (MRI) scans of patients diagnosed with sCJD and compared the EEG findings of MM2c-sCJD and MM1/classic sCJD groups. The records of six patients with MM2c-sCJD and eight with MM1/classic sCJD were included. The median ages of onset in the MM2c- and MM1/classic sCJD groups were 75.0 (range, 60–83) and 72.5 (range, 51–74) years, respectively, and the average durations between disease onset and the first EEG were 9.17 (range, 4–15) and 1.88 (range, 1–4) months, respectively. Focal sharp waves and/or focal spike-and-wave complexes in the brain regions corresponding with cortical hyperintensities on MRI scans were identified on the EEGs of patients with MM2c-sCJD in the early stages of disease progression. In contrast, EEGs of patients in the early stages of MM1/classic sCJD showed lateralized or generalized diffuse sharp waves and spike-and-wave complexes, which were not limited to cortical hyperintensities identified with MRI scans. Our findings indicate that focal sharp waves and/or focal spike-and-wave complexes on the EEGs of patients in the early phase of MM2c-sCJD are characteristic of the disease, suggesting the possible usefulness of this characteristic for early diagnosis.
机译：使用脑电图鉴定的定期尖峰复合物（PSWCS）在少于一半的蛋黄纯合型型孢子蛋白酶（MM2C）形式的孢子蛋白酶（SCJD）的患者中观察到少于一半的患者，并且仅在疾病的后期阶段。在这项研究中，我们在MM2C-SCJD患者的脑电图（EEG）上鉴定了早期和特异性标记。我们回顾性地研究了诊断患有SCJD的患者的临床记录，脑电图和磁共振成像（MRI）扫描，并比较了MM2C-SCJD和MM1 / CLAIRIC SCJD组的EEG结果。包括六名MM2C-SCJD患者和MM1 / CLASSIC SCJD患者的记录。 MM2C-和MM1 / CLASSIC SCJD组中发病中的中位数分别为75.0（范围，60-83）和72.5（范围，51-74）岁，并且疾病发病之间的平均持续时间和第一个脑电图是9.17 （范围，4-15）和1.88（范围，1-4）个月。在疾病进展早期MM2C-SCJD的患者的脑梗死中，对应于MRI扫描的皮质超萎缩的脑区中的焦点尖锐波和/或焦点峰值络合物。相反，MM1 /经典SCJD早期阶段的患者的脑电图显示横向化或广义弥漫性尖锐波和穗波和波复合物，其不限于用MRI扫描鉴定的皮质超萎缩性。我们的研究结果表明，在MM2C-SCJD早期患者患者脑梗死的焦点波浪和/或焦点穗络合物是疾病的特征，表明这种特征对于早期诊断的可能性。

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