Sacchi Luca, Rotondo Emanuela, Pozzoli Sara, Fiorentini Alessio, Schinco Giuseppina, Mandelli Clara, Coppola Carlotta, Fumagalli Giorgio G, Carandini Tiziana, Pietroboni Anna M, Galimberti Daniela, Triulzi Fabio, Marotta Giorgio, Scarpini Elio, Cesari Matteo, Brambilla Paolo, Arighi Andrea
Dino Ferrari Center, University of Milan, Italy.
Neurodegenerative Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
BJPsych Open. 2021 Feb 2;7(2):e43. doi: 10.1192/bjo.2020.171.
Diogenes syndrome is a neurobehavioural syndrome characterised by domestic squalor, hoarding and lack of insight. It is an uncommon but high-mortality condition, often associated with dementia.
To describe the clinical features and treatment of Diogenes syndrome secondary to behavioural variant frontotemporal dementia (bvFTD).
We describe a case of bvFTD in a 77-year-old man presenting with Diogenes syndrome.
The patient's medical and psychiatric histories were unremarkable, but in recent years he had begun packing his flat with 'art pieces'. Mental state examination revealed confabulation and more structured delusions. Neuropsychological evaluation outlined an impairment in selective attention and letter verbal fluency, but no semantic impairment, in the context of an overall preserved mental functioning. Brain magnetic resonance imaging and positron emission tomography (PET) with fluorodeoxyglucose showed mild bilateral temporo-insular atrophy and hypometabolism in the left-superior temporal gyrus respectively. An amyloid PET scan and genetic analysis covering the dementia spectrum were normal. A diagnosis of bvFTD was made.
The clinical framing of behavioural symptoms of dementia such as hoarding poses a diagnostic challenge. This case illustrates the importance of a deeper understanding of Diogenes syndrome, leading to timelier diagnosis and effective therapeutic strategies.
第欧根尼综合征是一种神经行为综合征，其特征为家居环境脏乱、囤积行为及缺乏自知力。它是一种罕见但死亡率高的疾病，常与痴呆症相关。
描述行为变异型额颞叶痴呆（bvFTD）继发的第欧根尼综合征的临床特征及治疗方法。
我们描述了一例77岁患有第欧根尼综合征的bvFTD男性患者。
患者的病史和精神病史无明显异常，但近年来他开始在公寓里堆满“艺术品”。精神状态检查发现虚构和更有条理的妄想。神经心理学评估显示在整体精神功能保留的情况下，选择性注意力和字母言语流畅性受损，但无语义损害。脑磁共振成像和氟脱氧葡萄糖正电子发射断层扫描（PET）分别显示双侧颞叶岛叶轻度萎缩和左侧颞上回代谢减低。淀粉样蛋白PET扫描和涵盖痴呆谱系的基因分析均正常。诊断为bvFTD。
痴呆行为症状如囤积的临床诊断构成挑战。该病例说明了深入了解第欧根尼综合征的重要性，从而实现更及时的诊断和有效的治疗策略。
