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痴呆相关的强制性歌唱(DACS):未发表的临床病例系列介绍。

Dementia-Associated Compulsive Singing (DACS): Presentation of Unpublished Clinical Cases Miniseries.

机构信息

Complex Operative Unit of Neurology, "F. Ferrari" Hospital, Casarano, 73042 Lecce, Italy.

Laboratory of Neuroproteomics, Multiple Sclerosis Centre, "F. Ferrari" Hospital, Casarano, 73042 Lecce, Italy.

出版信息

Int J Environ Res Public Health. 2022 Aug 31;19(17):10844. doi: 10.3390/ijerph191710844.

Abstract

Dementia-associated compulsive singing (DACS) is a neurotransmettitorial-based behavioral disturbance, characterized by an unabating melodic expression, occurring in patients that suffer from evolved dementia. Previously described only as a "punding" aspect of the dopamine dysregulation syndrome (DDS) in the Parkinson's disease (PD), compulsive singing has now been described, for the first time, in four non-PD patients effectively treated with Haloperidol or Quetiapine. Unlike the DDS-associated conditions, in our cases DACS is not pharmacologically induced, being that all patients were L-dopa-free. We detected a diffuse hyperintensity of the white matter and brain atrophy, with insular shrinkage as well as ventricular system and/or sub-arachnoid space enlargement in our DACS patients. Furthermore, similarly to the other behavioral symptoms of dementia, DACS also seems to be correlated to the degree of cognitive and functional impairment, rather than its subtype. In conclusion, DACS is a non-cognitive, unpublished clinical aspect of evolved dementia, which is interesting due to the involvement of the extra-nigral dopaminergic system, resulting in an unabating altered behavior, but also to the enrichment of our knowledge in the involutional diseases of the central nervous system and their physiopathological manifestations.

摘要

与痴呆相关的强制性歌唱(DACS)是一种基于神经递质的行为障碍,其特征是旋律表达持续不断,发生在患有进展性痴呆的患者中。以前仅在帕金森病(PD)的多巴胺失调综合征(DDS)中被描述为“冲动行为”,现在首次在 4 名非 PD 患者中进行了描述,这些患者有效地接受了氟哌啶醇或喹硫平治疗。与 DDS 相关的疾病不同,在我们的病例中,DACS 不是药物引起的,因为所有患者均未使用左旋多巴。我们在 DACS 患者中检测到脑白质弥漫性高信号和脑萎缩,以及脑岛萎缩以及脑室系统和/或蛛网膜下腔扩大。此外,与痴呆的其他行为症状一样,DACS 似乎也与认知和功能障碍的程度相关,而与亚型无关。总之,DACS 是非认知性的、未发表的进展性痴呆的临床表现,由于涉及到额外的黑质多巴胺能系统,导致持续不断的行为改变,这很有趣,但也丰富了我们对中枢神经系统退行性疾病及其生理病理学表现的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aabc/9517776/fdec28df81ec/ijerph-19-10844-g001.jpg

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