无法解释的急性同名半视眼作为克鲁茨菲尔特-雅各布病的表现
Omua Esezoobo1, David Gosal1, KeiraAnnie Markey1,2
1Department of Neurology, Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, Manchester, GBR.
Cureus
|February 16, 2026
概括
海登海因变体克鲁茨菲尔特-雅各布病 (HvCJD) 呈现出模仿其他疾病的视力损失. 早期诊断需要连续的MRI和EEG,因为在这种罕见的子疾病中,初始扫描可能是正常的.
科学领域:
- 神经学 神经学
- 神经科学是一个神经科学.
- 罕见疾病 罕见疾病
背景情况:
- 克鲁茨菲尔特 - 雅各布病 (CJD) 是一种致命的子疾病.
- 海登海因变体 (HvCJD) 是一种罕见的零散性CJD (sCJD) 亚型,其特征是突出的视觉障碍.
- 由于最初的非特异性症状和正常的早期成像,诊断hvcjd可能具有挑战性.
研究的目的:
- 报告HVCJD病例出现视野损失.
- 突出诊断挑战,并强调连续调查的重要性.
- 强调在特定的临床环境中对病的早期考虑的必要性.
主要方法:
- 一个患有无法解释的视野损失的病人的病例报告.
- 最初的治疗包括MRI,眼科评估和血液检查.
- 随后的调查涉及连续电脑电图 (EEG) 和磁共振成像 (MRI).
主要成果:
- 患者最初呈现出左侧同名半视,没有明显的初始MRI和检查结果.
- 病情恶化包括意识受损,非自愿的运动,以及类似性沉默症.
- 连续EEG显示有周期性放电的脑病变,后来MRI显示 parieto-occipital皮质带状,与HVCJD一致.
结论:
- HvCJD可以模仿后部循环中风或功能神经系统障碍,造成诊断困难.
- 正常的初始MRI并不能排除CJD;序列成像和EEG对于诊断至关重要.
- 早期考虑性疾病标志物,如CSF中的RT-QuIC,对于在具有视觉症状的快速进展性脑病变中及时诊断至关重要.
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