Institute of Neurology, Clinical Centre of Serbia, Belgrade, Serbia.
Acta Neurol Belg. 2010 Mar;110(1):71-7.
The aim was to assess factors that might influence health-related quality of life (HRQoL) in patients with two different neuromuscular disorders - myotonic dystrophy type 1 (DM1) and amyotrophic lateral sclerosis (ALS). A cross-sectional study was performed on 79 patients with DM1 and 74 with ALS. The HRQoL was evaluated by SF-36, Serbian version. Depressive and anxiety symptoms were assessed using the Hamilton rating scale for depression and the Hamilton rating scale for anxiety respectively. Severity of muscular involvement in DM1 was measured with MRC scale and severity of ALS with ALSFRSr score. The mean total score as well as all domain scores of SF-36 were similar in DM1 and ALS patients (p > 0.05), except that ALS patients experienced less bodily pain (p < 0.05). Depressiveness was found in 51% and marked anxiety in 38% of DM1 patients. Emotional status and severity of muscular involvement emerged as significant independent contributing factors to the total SF-36 in DMI patients (p < 0.05). Only 3% of ALS patients showed depressiveness and 4% anxiety symptoms. The factors found to contribute to HRQoL in ALS patients were severity of disease and educational level ofpatients (p < 0.05). We found significant percentage of potentially treatable emotional disturbances which together with severity of disease significantly contributed to HRQoL in DM1 patients. On the other hand, in ALS patients depressiveness and anxious symptoms were uncommon and the factors found to contribute to HRQoL were severity of disease and educational level.
目的是评估可能影响两种不同神经肌肉疾病 - 肌强直性营养不良 1 型（DM1）和肌萎缩侧索硬化症（ALS）患者健康相关生活质量（HRQoL）的因素。对 79 例 DM1 患者和 74 例 ALS 患者进行了横断面研究。HRQoL 通过 SF-36，塞尔维亚语版进行评估。抑郁和焦虑症状分别使用汉密尔顿抑郁评定量表和汉密尔顿焦虑评定量表评估。DM1 中肌肉受累的严重程度用 MRC 量表测量，ALS 用 ALSFRSr 评分测量。DM1 和 ALS 患者的 SF-36 总评分和所有领域评分均相似（p>0.05），但 ALS 患者的身体疼痛程度较低（p<0.05）。51%的 DM1 患者出现抑郁，38%的 DM1 患者出现明显焦虑。情感状态和肌肉受累的严重程度是 DM1 患者 SF-36 总分的重要独立影响因素（p<0.05）。只有 3%的 ALS 患者出现抑郁，4%的 ALS 患者出现焦虑症状。影响 ALS 患者 HRQoL 的因素是疾病的严重程度和患者的教育水平（p<0.05）。我们发现存在大量可能需要治疗的情绪障碍，这些障碍与疾病的严重程度一起显著影响 DM1 患者的 HRQoL。另一方面，在 ALS 患者中，抑郁和焦虑症状不常见，影响 HRQoL 的因素是疾病的严重程度和教育水平。
