Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
J Neuroimaging. 2009 Jul;19(3):283-7. doi: 10.1111/j.1552-6569.2008.00294.x.
The distinguishing feature in Heidenhain variant Creutzfeldt-Jakob Disease (HvCJD) is the presence of visual symptoms preceding the appearance of other clinical manifestations. The purpose of this report is to describe the broad range of visual symptomatology in a patient with HvCJD.
The authors describe the clinical, neuroimaging, and EEG features of a 51-year-old man with progressive visual symptoms that were most likely due to HvCJD. Migraine and epilepsy were initial diagnostic considerations.
Our patient presented with progressive visual decline and superimposed spells of visual dysfunction that had complex characteristics. Evolution of an abnormal signal in the parieto-occipital cortex on diffusion weighted MRI, changes on EEG, and clinical course were consistent with HvCJD.
HvCJD should be considered in all patients who present with unexplained visual phenomena. A remarkable spectrum of visual disturbances can be seen. Close follow-up as well as serial MRI and EEG can help clarify the underlying disease process. Diffusion-weighted and FLAIR sequences should be included in the MRI protocol.
海登海因变异型克雅氏病（HvCJD）的特征是在出现其他临床表现之前出现视觉症状。本报告的目的是描述一例 HvCJD 患者广泛的视觉症状。
作者描述了一位 51 岁男性患者的临床、神经影像学和脑电图特征，该患者的视觉症状逐渐加重，很可能是由于 HvCJD 引起的。最初的诊断考虑是偏头痛和癫痫。
我们的患者表现为进行性视力下降，并伴有视觉功能障碍的反复发作，其特征复杂。弥散加权 MRI 上顶枕叶皮质异常信号的演变、脑电图的变化以及临床病程均符合 HvCJD。
对于出现不明原因视觉现象的所有患者均应考虑 HvCJD。可以看到显著的一系列视觉障碍。密切随访以及连续的 MRI 和 EEG 可以帮助阐明潜在的疾病过程。MRI 方案中应包括弥散加权和 FLAIR 序列。
