Asymmetric neuroimaging in Creutzfeldt-Jakob disease: A ruse

Asymmetric neuroimaging in Creutzfeldt-Jakob disease: A ruse
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DOI:
10.1177/1051228403254785
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发表时间:
2003-10-01
影响因子:
2.4
通讯作者:
Clark, P
Clark, P
中科院分区:
医学4区
文献类型:
--
作者:
Bavis, J;Reynolds, P;Clark, P

文献摘要

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克雅氏病(CJD)会引起弥漫性神经症状,但常规磁共振成像(MRI)和弥散加权成像(DWI)已发现不对称病变。偶尔,正电子发射断层扫描 (PET) 扫描也可以显示克雅氏病患者的不对称病变。这种成像可能会误导临床医生。作者介绍了一名患有克雅氏病的妇女的病例,她被诊断为中风,因为她的不对称 T2 加权成像 (T2WI) MRI 异常被解释为中风。值得注意的是,该患者具有与克雅氏病一致的临床特征,包括快速进展性痴呆、肌阵挛、小脑功能障碍以及锥体和锥体外系体征。脑电图 (EEG) 上的周期性癫痫样放电和脑脊液中 14-3-3 蛋白升高支持了这一诊断。 MRI T2WI 和 DWI 显示左侧皮质显着不对称异常。 PET 研究发现左大脑半球和右小脑半球的新陈代谢下降。该患者的临床、脑电图和实验室数据均与克雅氏病一致,而不是其他疾病,但MRI和PET有不典型、不对称的表现,该病例表明,在鉴别诊断快速进行性神经功能减退的患者时,即使有不对称的影像学表现,也应考虑克雅氏病。
Creutzfeldt-Jakob disease (CJD) causes diffuse neurological symptoms, but asymmetric lesions have been found on conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI). Less often, positron emission tomography (PET) scanning can also reveal asymmetric lesions in patients with CJD. Such imaging may mislead clinicians. The authors present a case of a woman with CJD who was diagnosed as having suffered a stroke because she had asymmetric T2-weighted imaging (T2WI) MRI abnormalities that were interpreted as a stroke. It was noted that the patient had clinical features consistent with CJD, including rapidly progressive dementia, myoclonus, cerebellar dysfunction, and pyramidal and extrapyramidal signs. This diagnosis was supported by periodic epileptiform discharges on the electroencephalogram (EEG) and by elevated 14-3-3 protein in the cerebrospinal fluid. MRI T2WI and DWI showed dramatically asymmetric abnormalities involving the left cortex. A PET study found decreased metabolism in the left cerebral and right cerebellar hemispheres. The patient's clinical, EEG, and laboratory data were all consistent with CJD, not other diseases, but the MRI and PET had atypical, asymmetric findings, This case demonstrates that CJD should be considered in the differential diagnosis of patients with rapidly progressive neurological decline, even if they have asymmetric imaging findings.