School of Psychological Sciences, Tel Aviv University, Tel Aviv, Israel.
The Center for Psychobiological Research, Department of Educational Psychology and Educational Counseling, Max Stern Yezreel Valley College, Jezreel Valley, Israel.
Cerebellum. 2024 Aug;23(4):1265-1272. doi: 10.1007/s12311-023-01536-2. Epub 2023 Feb 28.
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.
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、感受和意图等方面思考和理解他人和自身心理状态的能力。本研究旨在填补我们对 MJD 心理化理解的空白，因为目前关于 MJD 和心理化的研究非常少且不一致。共有 18 名经临床和基因确诊的也门裔 MJD 患者、5 名携带阳性基因检测的 MJD 前症状患者和 17 名也门裔健康对照者接受了一系列测试，包括读心术（RME）、多伦多述情障碍量表（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，在 TAS-20 测试中得分较高。发现疾病持续时间与 RME 评分呈显著负相关。所有前症状参与者在所有测试中均得分正常。MJD 患者在临床水平上表现出广泛的心理化能力缺陷，具有自闭症特征。这些损伤可能会影响患者的人际体验和日常生活互动，并具有重要的临床意义。所有前症状参与者在所有测试中均表现出正常的心理化，这表明心理化损伤不会先于共济失调症状出现，而是 MJD 临床特征的一部分。
