Behavioral variant frontotemporal dementia in a 23-year-old man.

IF 0.6 4区 医学 Q4 CLINICAL NEUROLOGY Neurocase Pub Date : 2023-02-01 Epub Date: 2023-03-30 DOI:10.1080/13554794.2023.2191858
Tania C Ghazarian, Matthew G H Hall, Leslie A Horton, Bruce L Miller, Robert T Rubin
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Abstract

A 23-year-old man presented with behavioral disinhibition, stereotypies, motor apathy, flattened affect, and inappropriate laughter. CT demonstrated generalized cerebral atrophy. He was admitted with a diagnosis of unspecified psychosis and discharged on antipsychotic medication. He was readmitted 3 months later, was diagnosed with schizophrenia, and antipsychotic medication was continued. Owing to symptom progression and aggressive behavior, he was readmitted 2 months later. CT again demonstrated moderate central and cortical cerebral atrophy. MRI showed severe, stable atrophy with frontotemporal predominance, and he was diagnosed with probable behavioral variant frontotemporal dementia (bvFTD).  Over the next year he rapidly deteriorated, with loss of cognitive abilities. Genetic testing revealed several variants, none of which are clearly disease-causing.

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一名23岁男性的行为变异性额颞叶痴呆。
一名23岁的男子表现出行为去抑制、刻板印象、运动冷漠、平淡的情感和不恰当的笑声。CT显示全身性脑萎缩。他被诊断为精神病,出院后服用抗精神病药物。3个月后,他再次入院,被诊断为精神分裂症,并继续服用抗精神病药物。由于症状进展和攻击性行为,他在2个月后再次入院。CT再次显示中度中枢和皮质脑萎缩。MRI显示严重、稳定的萎缩,以额颞叶为主,他被诊断为可能的行为变异性额颞叶痴呆(bvFTD)。在接下来的一年里,他的病情迅速恶化,丧失了认知能力。基因检测发现了几种变体,但没有一种是明显致病的。
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来源期刊
Neurocase
Neurocase 医学-精神病学
CiteScore
1.40
自引率
12.50%
发文量
70
审稿时长
6-12 weeks
期刊介绍: Neurocase is a rapid response journal of case studies and innovative group studies in neuropsychology, neuropsychiatry and behavioral neurology that speak to the neural basis of cognition. Four types of manuscript are considered for publication: single case investigations that bear directly on issues of relevance to theoretical issues or brain-behavior relationships; group studies of subjects with brain dysfunction that address issues relevant to the understanding of human cognition; reviews of important topics in the domains of neuropsychology, neuropsychiatry and behavioral neurology; and brief reports (up to 2500 words) that replicate previous reports dealing with issues of considerable significance. Of particular interest are investigations that include precise anatomical localization of lesions or neural activity via imaging or other techniques, as well as studies of patients with neurodegenerative diseases, since these diseases are becoming more common as our population ages. Topic reviews are included in most issues.
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