"我不知道你的感受和我的感受":马查多-约瑟夫病患者的心理障碍。

IF 2.7 3区 医学 Q3 NEUROSCIENCES Cerebellum Pub Date : 2024-08-01 Epub Date: 2023-02-28 DOI:10.1007/s12311-023-01536-2
Zohar Elyoseph, Dario Geisinger, Erez Nave-Aival, Roy Zaltzman, Carlos R Gordon
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引用次数: 0

摘要

马查多-约瑟夫病(MJD)又称脊髓小脑共济失调 3 型(SCA3),是一种常染色体显性神经退行性疾病。心理化是指从思想、情感和意图等方面思考和理解他人和自己的心理状态的能力。由于目前关于 MJD 和 mentalizing 的研究很少且不一致,本研究旨在填补我们对 MJD 中 mentalizing 的认识空白。共有 18 名经临床和基因证实患有 MJD 的也门裔犹太人、5 名基因检测呈阳性的症状前 MJD 患者和 17 名健康对照组也门裔犹太人接受了一系列测试,包括 "眼中读心"(RME)、"多伦多 Alexithymia 量表"(TAS-20)和 "错误信念测试"(FBt)。与对照组相比,MJD 组在 RME 和 FBt 测试中得分较低,而在 TAS-20 测试中得分较高。病程与 RME 分数之间存在明显的负相关。所有症状前参与者在所有测试中的得分都在正常临床范围内。MJD 患者在临床水平上表现出广泛的心智能力缺陷,具有自闭症特征。这些缺陷可能会影响患者的心理体验和日常生活互动,具有重要的临床意义。症状出现前的参与者在所有测试中均表现出正常的思维能力,这表明思维能力障碍并不先于共济失调症状出现,而是共济失调临床表现的一部分。
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"I Do Not Know How You Feel and How I Feel About That": Mentalizing Impairments in Machado-Joseph Disease.

Machado Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is an autosomal dominant neurodegenerative disease. Mentalizing is the ability to think and understand the mental state of the other and of the self in terms of thoughts, feelings, and intentions. The aim of this study is to fill the gap in our understanding of mentalizing in MJD since there is currently very little and inconsistent research on MJD and mentalizing. A total of 18 Jews of Yemenite origin with clinically and genetically confirmed MJD, 5 pre-symptomatic MJD with a positive genetic test, and 17 Jews of Yemenite origin healthy controls, underwent a battery of tests consisting of reading the mind in the eyes (RME), Toronto Alexithymia Scale (TAS-20), and false belief test (FBt). The MJD group scored lower on the RME and FBt, and higher on TAS-20 test compared to control. A significant negative correlation was found between disease duration and RME score. All the pre-symptomatic participants scored within the normal clinical range in all tests. MJD patients demonstrated a widespread deficiency in the ability to mentalizing on a clinical level with autistic characteristics. These impairments may impact the patient's interpsychic experience and daily life interactions and have important clinical implication. Pre-symptomatic participants demonstrated normal mentalizing in all tests, suggesting that the mentalizing impairments do not precede the symptoms of ataxia and are part of the clinical picture of MJD.

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来源期刊
Cerebellum
Cerebellum 医学-神经科学
CiteScore
6.40
自引率
14.30%
发文量
150
审稿时长
4-8 weeks
期刊介绍: Official publication of the Society for Research on the Cerebellum devoted to genetics of cerebellar ataxias, role of cerebellum in motor control and cognitive function, and amid an ageing population, diseases associated with cerebellar dysfunction. The Cerebellum is a central source for the latest developments in fundamental neurosciences including molecular and cellular biology; behavioural neurosciences and neurochemistry; genetics; fundamental and clinical neurophysiology; neurology and neuropathology; cognition and neuroimaging. The Cerebellum benefits neuroscientists in molecular and cellular biology; neurophysiologists; researchers in neurotransmission; neurologists; radiologists; paediatricians; neuropsychologists; students of neurology and psychiatry and others.
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