Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
Acta Neurol Scand. 2014 Apr;129(4):219-25. doi: 10.1111/ane.12173. Epub 2013 Sep 3.
To analyze frequency and type of personality pattern in patients with myotonic dystrophy type 1 (DM1), to correlate these findings with clinical data, and to assess its possible influence on quality of life (QoL).
This cross-sectional study comprised 62 patients with DM1. Following measures were used: Muscular Impairment Rating Scale, Raven's Standard Progressive Matrices (RSPM), Millon Multiaxial Clinical Inventory I (MMCI), SF-36, and Individualized Neuromuscular Quality of Life (INQoL) questionnaires.
The presence of at least one pathological personality trait with score above 85 on MMCI was found in 47 (75.8%) patients. After clinical interview, 36 (58.1%) subjects had significant personality impairment. The most common personality trait in our cohort of patients was dependent found in 51.6% of patients, followed by paranoid (38.7%). Higher score on dependent personality scale correlated with lower education (rho = -0.251, P = 0.049). Dependent personality scores significantly differed between patients with physical and intellectual work (93.1 ± 8.9 vs 66.9 ± 31.7, P = 0.011). Paranoid score was higher in patients with lower education (rho = -0.293, P = 0.021), lower score on RSPM test (rho = -0.398, P = 0.004) and larger number of CTG repeats (rho = 0.254, P = 0.046). Presence of dependent personality was not in association with QoL scores (P > 0.05). On the other hand, patients with paranoid personality trait had worse QoL than those without it (P < 0.05).
Almost 60% of our patients with DM1 had clinically significant personality impairment, with dependent and paranoid personality patterns being the most common. Paranoid personality may decrease QoL in these patients, which gives us new opportunities for symptomatic therapy in DM1.
分析 1 型肌强直性营养不良（DM1）患者的人格模式频率和类型，将这些发现与临床数据相关联，并评估其对生活质量（QoL）的可能影响。
本横断面研究包括 62 例 DM1 患者。使用以下措施：肌肉损伤评分量表、瑞文标准渐进矩阵（RSPM）、米隆多轴临床量表 I（MMCI）、SF-36 和个体化神经肌肉生活质量（INQoL）问卷。
在 47 例（75.8%）患者中发现至少存在一种病理性人格特征，MMCI 评分高于 85 分。经过临床访谈，36 例（58.1%）患者存在明显的人格障碍。在我们的患者队列中，最常见的人格特征是依赖性，在 51.6%的患者中发现，其次是偏执型（38.7%）。依赖性人格量表的评分与教育程度较低呈正相关（rho=-0.251，P=0.049）。具有身体和智力工作的患者之间的依赖性人格评分存在显著差异（93.1±8.9 vs 66.9±31.7，P=0.011）。教育程度较低的患者偏执评分较高（rho=-0.293，P=0.021），RSPM 测试评分较低（rho=-0.398，P=0.004）和 CTG 重复次数较多（rho=0.254，P=0.046）。依赖性人格的存在与 QoL 评分无关（P>0.05）。另一方面，具有偏执人格特征的患者的 QoL 比没有该特征的患者差（P<0.05）。
我们的 DM1 患者中近 60%存在明显的人格障碍，以依赖性和偏执性人格模式最为常见。偏执人格可能会降低这些患者的生活质量，这为 DM1 的症状治疗提供了新的机会。
