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Autoscopic phenomena as an atypical psychiatric presentation of Huntington's disease: A case report including longitudinal clinical and neuroimaging data.
Cortex ( IF 3.6 ) Pub Date : 2020-02-08 , DOI: 10.1016/j.cortex.2020.01.024
Saul Martínez-Horta 1 , Jesús Perez-Perez 1 , Javier Pagonabarraga 2 , Frederic Sampedro 2 , Andrea Horta-Barba 1 , Olaf Blanke 3 , Jaime Kulisevsky 1
Affiliation  

Huntington's disease (HD) is a monogenetic neurodegenerative disease prototypically characterized by the progressive presentation of motor abnormalities, cognitive deterioration and neuropsychiatric symptoms. Even when the disorder is diagnosed based on the presence of unequivocal motor symptoms, subtle cognitive and behavioral changes emerge decades before the first motor manifestations. Here we present the atypical case of a young premanifest gene-mutation carrier who developed progressive complex autoscopic phenomena (feelings of presence, out of body experience, and heautoscopic hallucinations). To our knowledge, this is the first report of these symptoms in the context of HD. Considering the availability of serial neurologic, neuropsychological and neuroimaging data collected before and after the presentation of these symptoms, this case provides new insights into the brain mechanisms leading to autoscopic phenomena and atypical phenotypes that may occur in HD.

中文翻译:

自动镜现象是亨廷顿舞蹈病的一种非典型精神病表现:包括纵向临床和神经影像学数据的病例报告。

亨廷顿舞蹈病(HD)是一种单基因的神经退行性疾病,其典型特征是逐渐出现运动异常,认知能力下降和神经精神症状。即使基于明确的运动症状诊断出该疾病,在第一个运动表现出现之前的几十年,仍会出现微妙的认知和行为变化。在这里,我们介绍了一个年轻的预言基因突变携带者的非典型案例,该携带者发展了进行性复杂的自动镜现象(临场感,身体体验和自动镜幻觉)。据我们所知,这是在HD背景下这些症状的首次报道。考虑到在出现这些症状之前和之后收集的一系列神经,神经心理学和神经影像学数据的可用性,
更新日期:2020-02-10
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