Sharma Divya K, Boggild Mike, van Heuven Annemarie W, White Richard P
*Mater Health Services North Queensland †Department of Neurology, The Townsville Hospital, Townsville, Qld, Australia.
Neurologist. 2017 Mar;22(2):48-53. doi: 10.1097/NRL.0000000000000107.
Creutzfeldt-Jacob disease (CJD) is a human prion disease generally characterized by subacute changes in behavior and intellectual function, often followed by ataxia, vision changes, and myoclonus. Ten percent of cases may present atypically, both symptomatically and in respect to initial investigations.
We report a case of CJD mimicking acute stroke and review all similar cases in the magnetic resonance imaging era reported in English, identified through a PubMed and SCOPUS search.
A 68-year-old woman presented with an acute left parietal syndrome, initially referred as a left middle cerebral artery territory stroke. Structural imaging was unremarkable and focal electroencephalogram changes suggested nonconvulsive status epilepticus. Subsequent clinical progression, with the development of cortical high signal on diffusion-weighted imaging and positive 14-3-3 protein in the cerebrospinal fluid, confirmed a diagnosis of CJD. Review of the literature identified 14 further cases mimicking both anterior and posterior stroke syndromes.
CJD develops primarily within a population in whom stroke risk factors are common and represents a rare but important stroke mimic. Negative vascular imaging in elderly patients with apparent acute stroke syndromes should prompt diagnostic review including consideration of prion diseases.
克雅氏病（CJD）是一种人类朊病毒病，通常表现为行为和智力功能的亚急性变化，常伴有共济失调、视力改变和肌阵挛。10%的病例可能在症状和初始检查方面表现不典型。
我们报告一例疑似急性卒中的克雅氏病病例，并回顾在磁共振成像时代以英文报道的所有类似病例，通过PubMed和SCOPUS检索识别。
一名68岁女性出现急性左顶叶综合征，最初被诊断为左侧大脑中动脉供血区卒中。结构成像无异常，局灶性脑电图改变提示非惊厥性癫痫持续状态。随后的临床进展，出现弥散加权成像上的皮质高信号和脑脊液中14-3-3蛋白阳性，确诊为克雅氏病。文献回顾发现另外14例类似前后循环卒中综合征的病例。
克雅氏病主要在具有常见卒中危险因素的人群中发生，是一种罕见但重要的卒中模拟疾病。老年患者出现明显急性卒中综合征且血管成像阴性时，应促使进行诊断性复查，包括考虑朊病毒病。
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