Movement Disorders Unit, Neurology Department, Sant Pau Hospital, Barcelona, Spain.
Universitat Autònoma de Barcelona (U.A.B.), Medicine Department, Barcelona, Spain.
J Int Neuropsychol Soc. 2023 Jul;29(6):615-619. doi: 10.1017/S1355617722000583. Epub 2022 Sep 5.
A previously healthy woman began to present recurrent episodes of reduplicative paramnesia within her home and later structured visual hallucinations. The case was initially oriented as an incipient vascular dementia. Detailed anamnesis and neuropsychological examination suggested a rapidly progressive pattern of neuropsychological deficits mostly attributable to parieto-occipital disturbances with some component of fronto-temporal involvement. Subsequently, cerebellar symptoms were added. Although the initial imaging studies were inconclusive, the MRI performed during follow-up showed a series of findings compatible with a prion disease. Based on the neuropsychological and clinical features and the imaging pattern, the diagnosis of Heidenhain Variant of Creutzfeldt-Jakob disease was established. This is the first report of a Heidenhain Variant of Creutzfeldt-Jakob disease presenting as a reduplicative paramnesia as the first manifestation of this disease.
一位既往健康的女性开始在家中出现复发性双重错觉，并随后出现结构性幻视。最初的诊断为早期血管性痴呆。详细的病史和神经心理学检查提示神经心理学缺陷呈快速进展模式，主要归因于顶枕叶紊乱，伴有额颞叶受累的一些成分。随后出现了小脑症状。尽管最初的影像学研究不确定，但后续进行的 MRI 检查显示一系列与朊病毒病相符的发现。基于神经心理学和临床特征以及影像学模式，诊断为海登海因变异型克雅氏病。这是首例以复发性双重错觉为疾病首发表现的海登海因变异型克雅氏病的报道。
