Bungener C, Jouvent R, Delaporte C
Université de Bourgogne, Dijon, France.
J Neurol Neurosurg Psychiatry. 1998 Sep;65(3):353-6. doi: 10.1136/jnnp.65.3.353.
To evaluate psychopathological disturbances in patients with myotonic dystrophy (MD) and compare patients with MD to both patients with facioscapulohumeral dystrophy (FSHD) and healthy control subjects.
A semistructured interview was used to determine DSM III-R criteria for major depressive episodes, dysthymic episodes, and generalised anxiety. The Montgomery and Asberg and the Hamilton depressive scales, the Covi and Tyrer anxiety scales, the Abrams and Taylor scale for emotional blunting, and the depressive mood scale were all used in the study. Subjects were also asked to complete questionnaires for physical and social anhedonia.
Fifteen patients with MD, 11 patients with FSHD, and 14 healthy subjects were studied. Patients with MD were not more depressed or anxious than healthy controls. Patients with FSHD were the most depressed and most anxious. However, patients with MD had significantly lower scores for expressiveness and significantly higher scores for anhedonia than the other two groups.
Patients with MD did not present significant depressive or anxious symptomatology but rather an emotional deficit. This emotional deficit may be an adaptive reaction to the threatening implications of the disease, or the effect of the CNS lesions which occur with MD, or both.
评估强直性肌营养不良（MD）患者的精神病理障碍，并将MD患者与面肩肱型肌营养不良（FSHD）患者及健康对照者进行比较。
采用半结构式访谈来确定重度抑郁发作、心境恶劣发作及广泛性焦虑的DSM III-R标准。研究中使用了蒙哥马利和阿斯伯格抑郁量表、汉密尔顿抑郁量表、科维焦虑量表、泰勒焦虑量表、艾布拉姆斯和泰勒情感迟钝量表以及抑郁情绪量表。还要求受试者完成关于躯体性快感缺失和社会性快感缺失的问卷。
对15例MD患者、11例FSHD患者和14名健康受试者进行了研究。MD患者并不比健康对照者更抑郁或焦虑。FSHD患者是最抑郁和最焦虑的。然而，MD患者在表达能力方面的得分显著低于其他两组，而在快感缺失方面的得分显著高于其他两组。
MD患者未表现出明显的抑郁或焦虑症状，而是存在情感缺陷。这种情感缺陷可能是对该疾病威胁性影响的适应性反应，或者是MD所伴发的中枢神经系统病变的结果，或者两者兼而有之。
