Solla Paolo, Mura Gioia, Cannas Antonino, Floris Gianluca, Fonti Davide, Orofino Gianni, Carta Mauro Giovanni, Marrosu Francesco
Department of Neurology, Movement Disorders Center, Institute of Neurology, University of Cagliari, SS 554 Bivio per Sestu, Monserrato, 09042, Cagliari, Italy.
Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
BMC Neurol. 2017 Apr 19;17(1):78. doi: 10.1186/s12883-017-0842-1.
Dementia with Lewy bodies (DLB) is the second most frequent diagnosis of progressive degenerative dementia in older people. Delusions are common features in DLB and, among them, Capgras syndrome represents the most frequent disturbance, characterized by the recurrent and transient belief that a familiar person, often a close family member or caregiver, has been replaced by an identical-looking imposter. However, other delusional conditions near to misidentification syndromes can occur in DLB patients and may represent a major psychiatric disorder, although rarely studied systematically.
We reported on a female patient affected by DLB who presented with an unusual delusion of duplication. Referring to the female professional caregiver engaged by her relatives for her care, the patient constantly described the presence of two different female persons, with a disorder framed in the context of a delusion of duplication. A brain 99Tc-hexamethylpropyleneamineoxime SPECT was performed showing moderate hypoperfusion in both occipital lobes, and associated with marked decreased perfusion in parieto-fronto-temporal lobes bilaterally.
An occipital hypoperfusion was identified, although in association with a marked global decrease of perfusion in the remaining lobes. The role of posterior lobes is certainly important in all misidentification syndromes where a natural dissociation between recognition and identification is present. Moreover, the concomitant presence of severe attentional and executive deficits evocative for a frontal syndrome and the marked global decrease of perfusion in the remaining lobes at the SPECT scan also suggest a possible dysfunction in an abnormal connectivity between anterior and posterior areas.
路易体痴呆(DLB)是老年人进行性退行性痴呆的第二常见诊断。妄想是DLB的常见特征,其中卡普格拉综合征是最常见的障碍,其特征是反复出现且短暂地认为一个熟悉的人,通常是亲密的家庭成员或照顾者,被一个长相相同的冒名顶替者所取代。然而,DLB患者可能会出现其他接近错认综合征的妄想情况,并且可能代表一种主要的精神障碍,尽管很少进行系统研究。
我们报告了一名患有DLB的女性患者,她出现了一种不寻常的复制妄想。提及亲属为照顾她而聘请的女性专业护理人员,患者一直描述有两个不同的女性存在,这种紊乱被界定为复制妄想的背景下。进行了脑99Tc-六甲基丙烯胺肟单光子发射计算机断层扫描(SPECT),显示双侧枕叶中度灌注不足,并伴有双侧顶叶-额叶-颞叶明显灌注减少。
虽然与其余脑叶明显的整体灌注减少相关,但发现了枕叶灌注不足。在所有存在识别与辨认自然分离的错认综合征中,后叶的作用肯定很重要。此外,SPECT扫描时同时存在提示额叶综合征的严重注意力和执行功能缺陷以及其余脑叶明显的整体灌注减少,也提示前后区域之间异常连接可能存在功能障碍。