64例包涵体肌炎患者的临床特征与临床病程  

Inclusion body myositis: Clinical features and clinical course of the disease in 64 patients

作　　者：Badrising U.A Maat-Schieman M.L.C Van Houw-elingen J.C 陈海 

机构地区：[1]Dept. of Neurology, Van Weel-Bethesda Hospital, 3247 BD, Dirksland, Netherlands

出　　处：《世界核心医学期刊文摘（神经病学分册）》2006年第5期25-25,共1页Digest of the World Core Medical Journals:Clinical Neurology

摘　　要：The clinical features of inclusion body myositis (IBM) were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors reported “ typical" signs. To re-assess the clinical spectrum of IBM, a single investigator using a standard protocol studied a cohort of 64 patients cross-sectionally. Symptom onset was before the age of 50 years in 20 % of cases. Only a few patients (14 % ) started with weakness other than that of quadriceps, finger flexor or pharyngeal muscles. The sequence of power loss was erratic, but onset of symptoms with quadriceps weakness predicted an earlier onset of dysphagia in older patients (≥ 56 years) compared with younger ones (< 56 years) (p = 0.02). Despite widespread weakness patients had favourable scores on three commonly used function scales and they kept their employment. Complete wheel-chair dependency was rare (3 % ). A dominant characteristic was the anatomical distribution of afflicted muscles: ventral extremity muscle groups were more affected than dorsal muscle groups and girdle muscles were least affected, the latter preserving postural stability. Ankylosis, especially in extension of the fingers, was frequently present. Together with the sparing of intrinsic hand muscles it was helpful in the preservation of many skilful movements. IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease.The clinical features of inclusion body myositis （IBM） were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors reported ＂typical＂ signs. To re-assess the clinical spectrum of IBM, a single investigator using a standard protocol studied a cohort of 64 patients cross-sectionally. Symptom onset was before the age of 50 years in 20 % of cases. Only a few patients （14 % ） started with weakness other than that of quadriceps, finger flexor or pharyngeal muscles. The sequence of power loss was erratic, but onset of symptoms with quadriceps weakness predicted an earlier onset of dysphagia in older patients （ ≥ 56 years） compared with younger ones （ 〈 56 years） （p = 0.02） . Despite widespread weakness patients had favourable scores on three commonly used function scales and they kept their employment Complete wheel-chair dependency was rare （3 % ）. A dominant characteristic was the anatomical distribution of afflicted muscles： ventral extremity muscle groups were more affected than dorsal muscle groups and girdle muscles were least affected, the latter preserving postural stability. Ankylosis, especially in extension of the fingers, was frequently present. Together with the sparing of intrinsic hand muscles it was helpful in the preservation of many skilful movements. IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease.

关 键 词：包涵体肌炎 年轻患者 临床特征 临床病程 全身肌无力 功能量表评分 关节强直 股四头肌 受累肌肉 横断面研究 

分 类 号：R685.2[医药卫生—骨科学] R713.42[医药卫生—外科学]