Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt-Jakob disease
Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt-Jakob disease
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DOI：
10.1016/s1388-2457(03)00109-3
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发表时间：
2003-09-01
影响因子：
4.7
通讯作者：
Aksamit, AJ
中科院分区：
文献类型：
--
作者：
Cambier, DM;Kantarci, K;Aksamit, AJ
目的：克雅氏病（Creutzfeldt-Jakob disease，CJD）是一种进展迅速的致死性朊病毒病，临床表现为痴呆、运动功能障碍和肌阵挛，脑电图表现为双侧同步周期性尖波。弥散加权和液体衰减反转恢复（FLAIR）磁共振成像（MRI）技术在神经成像能力方面的进展已显示出在CJD非侵入性诊断中的前景。该系列显示了8例患者的临床、EEG和MRI FLAIR序列异常之间的相关性（7名男性和1名女性，55-73岁）患有CID。在马约诊所评价的8例患者的病例系列，在1996年至1999年期间，有快速进行性偏侧或局灶性神经功能障碍病史且实验室检查结果与CJD一致的患者，鉴定所有患者均行EEG、头颅MRI FLAIR序列及脑脊液检查。5例患者的症状局限于左半球，2例患者的症状局限于右半球。1例患者显示双侧枕叶受累。在所有患者中，EEG显示与临床脑功能障碍共定位的偏侧或局灶性周期性尖波。FLAIR MRI图像显示皮质带和深层灰质信号增加，与7名患者的侧化临床和EEG结果相对应。结论：CID可表现为大脑皮质偏侧或局灶性综合征，EEG和MRI表现一致。临床病史和实验室检查，FLAIR MRI信号增强区可为CID的诊断提供依据。意义：CID有时可表现为局灶性或偏侧性的临床表现，EEG和MRI的共同定位有助于CID的诊断。(C)2003年国际临床神经生理学联合会。由Elsevier爱尔兰有限公司出版。保留所有权利。
Objective: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal prion disorder with typical clinical findings of dementia, motor dysfunction, and myoclonus and characteristic electroencephalographic (EEG) findings of bilateral synchronous periodic sharp waves. Advances in neuroimaging capabilities with diffusion-weighted and fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) techniques have shown promise in the non-invasive diagnosis of CJD. This series illustrates the correlation between the lateralized and focal clinical, EEG, and MRI FLAIR sequence abnormalities in 8 patients (7 men and one woman 55-73 years old) with CID.Methods: A case series of 8 patients, evaluated at Mayo Clinic, who had a history of rapidly progressive lateralized or focal neurologic dysfunction and laboratory findings consistent with CJD between 1996 and 1999 were identified. EEG, MRI of the head with FLAIR sequence, and cerebrospinal fluid studies were performed in all patients.Results: Mean time to death from symptom onset was 4 months. Symptoms were lateralized to the left hemisphere in 5 patients and to the right hemisphere in two. One patient showed bilateral occipital lobe involvement. In all patients, the EEG showed lateralized or focal periodic sharp waves that colocalized with clinical cerebral dysfunction. FLAIR MRI images revealed increased signal in the cortical ribbon and deep gray matter corresponding to the lateralized clinical and EEG findings in 7 patients. The other patient had bilateral occipital increased signal on FLAIR MRI.Conclusions: CID may present with lateralized or focal cortical syndromes with colocalizing EEG and MRI findings. With the appropriate clinical history and laboratory evaluation, the corresponding areas of increased signal on FLAIR MRI provide supportive evidence of the disease.Significance: CID can sometimes present with more focal or lateralized clinical findings, and the colocalizing EEG and MRI findings can help make or confirm the diagnosis of CID. (C) 2003 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.