Creutzfeldt-Jakob disease-like diffusion-weighted imaging hyperintensity paralleled with neuropsychiatric symptoms in a patient with limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies
Creutzfeldt-Jakob disease-like diffusion-weighted imaging hyperintensity paralleled with neuropsychiatric symptoms in a patient with limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies
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与抗电压门控钾通道复合物抗体相关的边缘脑炎患者的克雅氏病样弥散加权成像高信号与神经精神症状平行
DOI：
10.1111/cen3.12525
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发表时间：
2019
期刊：
影响因子：
--
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et al.
中科院分区：
文献类型：
--
作者：
Okadome T;et al.
将克雅病(CJD)与其他潜在的可治疗疾病区分开来是至关重要的。脑电(EEG)上的周期性尖波复合波是脑脊液中的神经元损伤标志物，弥散加权成像(DWI)上的皮质和皮质下高信号支持CJD的诊断。然而，这些功能不是CJD所特有的。我们报告一位与抗电压门控钾通道复合体抗体(抗VGKCC-ab)相关的边缘脑炎患者，其临床和DWI表现与CJD的特征相似。患者是一名58岁的男性，逐渐出现记忆障碍。连续两次(发病后2个月和5个月)在当地诊所进行的磁共振成像扫描在液体衰减的反转恢复成像中发现左侧内侧颞叶有一过性高信号病变，随后内侧颞叶萎缩。未发现弥散加权像异常。随后，他的记忆障碍加重，出现幻觉和抑郁状态。他的精神症状无休止地恶化。症状出现9个月后，他住进我们的诊所(日本福冈九州大学医院)进行进一步的评估。第一次就诊时，神经系统检查正常，但有严重的逆行性和顺行性健忘、幻觉和抑郁状态。未观察到面臂肌张力障碍发作。韦氏成人智力量表III在正常范围内。修订后的韦氏记忆量表在逻辑记忆、视觉记忆和延迟记忆方面存在异常，分别为26分、46分和32分。血清分析显示低钠血症(132mEq/L)。肿瘤标志物和胶原病抗体均为阴性。脑脊液细胞计数和蛋白质水平正常，病毒聚合酶链式反应检测(HSV，HHV6和VZV)，14-3-3和tau蛋白检测阴性，实时震动诱导转换
It is crucial to differentiate Creutzfeldt–Jakob disease (CJD) from other potentially treatable diseases. The diagnosis of CJD is supported by periodic sharp wave complexes on electroencephalography (EEG), a neuronal injury marker in cerebrospinal fluid, and cortical and subcortical hyperintensities on diffusionweighted imaging (DWI). However, these features are not specific for CJD. We report a patient with limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies (anti-VGKCC-ab) who presented similar clinical and DWI findings to those characteristic of CJD. The patient was a 58-year-old man who gradually developed memory impairment. Two consecutive brain magnetic resonance imaging scans (2 and 5 months after onset) carried out at a local clinic detected a transient hyperintense lesion in the left mesial temporal lobe on fluid-attenuated inversion recovery imaging, with subsequent mesial temporal lobe atrophy. No DWI abnormality was observed. Subsequently, his memory impairment was aggravated, and hallucinations and a depressive state occurred. His mental symptoms deteriorated relentlessly without remission. Then, 9 months after symptom onset, he was admitted to our clinic for further evaluation (Kyushu university hospital, Fukuoka, Japan).On his first visit, the neurological examination was normal, except for severe retrograde and anterograde amnesia, hallucinations, and a depressive state. Faciobrachial dystonic seizure was not observed. The Wechsler Adult Intelligence Scale III was within the normal limits. The Wechsler Memory Scale-Revised was abnormal in logical, visual and delayed memory, with scores of 26, 46 and 32, respectively. Serum analysis showed hyponatremia (132 mEq/L). Tumor markers and antibodies for collagen diseases were all negative. Cerebrospinal fluid was normal for cell count and protein level, and negative for viral polymerase chain reaction testing (HSV, HHV6 and VZV), 14-3-3 and tau protein testing, and real-time quaking-induced conversion