Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
Neuromuscul Disord. 2012 Nov;22(11):980-6. doi: 10.1016/j.nmd.2012.05.004. Epub 2012 Jun 26.
There are currently no effective treatments to restore the muscle function in sporadic inclusion body myositis. Natural history studies of this disease are scarce. The goal of this study consisted in defining the functional pattern of patients with sporadic inclusion body myositis and to follow its change over a 9-month period to determine the most sensitive outcome measures for future clinical trials. Twenty-two patients with definite sporadic inclusion body myositis were assessed using clinical and functional scales. Dynamometry was used to evaluate the strength for hand grip and wrist, elbow, ankle and knee flexion and extension. Among the patients, 16 were reassessed 9months later. The mean whole composite index was at 43.3±16.5% of the predicted normal values. The weakest muscle functions were hand grip, wrist flexion and elbow flexion at the upper limbs and knee extension and ankle flexion at the lower limbs. Muscle weakness was generally asymmetrical, especially for upper limbs where all tested functions were significantly stronger at the dominant side. The patient strength was correlated with the disease duration only for knee extension, which was also the only muscle function to change significantly over 9months. Knee extension strength seems to be the most relevant marker of disease progression in sporadic inclusion body myositis when measured with suitable dynamometry.
目前尚无有效的治疗方法可以恢复散发性包涵体肌炎的肌肉功能。该疾病的自然史研究很少。本研究的目的是确定散发性包涵体肌炎患者的功能模式,并在 9 个月内观察其变化,以确定未来临床试验中最敏感的结局指标。22 例明确诊断为散发性包涵体肌炎的患者接受了临床和功能量表的评估。测力计用于评估手握力和腕关节、肘关节、踝关节和膝关节屈伸的力量。其中 16 例患者在 9 个月后再次接受评估。全复合指数平均为预测正常值的 43.3±16.5%。上肢最弱的肌肉功能是手握力、腕关节屈曲和肘关节屈曲,下肢最弱的肌肉功能是膝关节伸展和踝关节屈曲。肌肉无力通常是不对称的,特别是在上肢,所有测试的功能在优势侧明显更强。患者的力量仅与疾病持续时间相关,仅与膝关节伸展相关,这也是 9 个月内唯一显著变化的肌肉功能。使用适当的测力计测量时,膝关节伸展力量似乎是散发性包涵体肌炎疾病进展的最相关标志物。