Stübgen J P, Stipp A
Department of Neurology, Cornell University Medical College, New York, New York 10021, USA.
Muscle Nerve. 1997 Apr;20(4):453-60. doi: 10.1002/(sici)1097-4598(199704)20:4<453::aid-mus8>3.0.co;2-9.
This 6-year prospective follow-up study evaluated the rate of clinical disease progression in 19 of 20 previously reported patients with strictly selected limb girdle muscular dystrophy. There was no significant deterioration in muscle strength (assessed by manual muscle testing). An activities of daily living (ADL) scale showed significant functional deterioration in 30% of patients with predominant involvement in a single functional domain variable between patients, and determined by the patient's previous level of function. A functional grading system showed that 70% of patients had deteriorated at least one grade in the arms and/or legs. Deterioration of the ADL score correlated with the loss of functional grades. We conclude that assessment of functional ability may be more sensitive to detect clinical disease progression compared to testing of muscle strength. The clinical parameter that correlates best with the activity of the underlying disease process is not determined. Tests for strength and functional impairment are complementary. Patients expressed appreciation that functional disability was addressed during follow-up visits.
这项为期6年的前瞻性随访研究评估了先前报告的20例经过严格筛选的肢带型肌营养不良患者中19例的临床疾病进展率。肌肉力量(通过徒手肌力测试评估)没有明显恶化。日常生活活动(ADL)量表显示,30%的患者出现明显功能恶化,主要累及单个功能领域,患者之间存在差异,且由患者先前的功能水平决定。功能分级系统显示,70%的患者上肢和/或下肢至少恶化了一个等级。ADL评分的恶化与功能等级的丧失相关。我们得出结论,与肌肉力量测试相比,功能能力评估可能对检测临床疾病进展更敏感。与潜在疾病进程活动最相关的临床参数尚未确定。力量测试和功能损害测试是互补的。患者对随访期间关注功能残疾表示感激。