17例中国人有镶边空泡远端肌病临床病理特点及预后随访

目的 探讨17例中国人有镶边空泡远端肌病(DMRV)患者的临床病理特点和部分患者预后情况.方法 对山东大学齐鲁医院神经内科1986至2007年问17例DMRV患者的临床和病理资料进行回顾性分析和总结并对其预后情况进行门诊或电话随访.结果 17例患者中女12例,男5例,平均起病年龄23.1岁.下肢远端肌早期受累,股四头肌受累相对较轻,病情缓慢进展.肌肉病理改变主要为镶边空泡形成,肌纤维坏死和再生少见,电镜观察可见肌浆或肌核内管状细丝包涵体.有11例患者获得了其随访资料,其中10年以上2例,5～10年2例,1～5年5例,1年以内2例.随访结果显示尽管出现于成年早期的始发症状是下肢远端肌的萎缩和无力,但疾病晚期可见全身骨骼肌广泛严重受累,患者丧失行走和生活自理能力,生活质量差.结论 中国人DMRV的临床和病理特点与日本报道的病例基本一致.病情缓慢进展,病后7-10年丧失独立行走能力.就日常生活而言,DMRV的预后是相当差的.     

外伤性颌面部骨化性肌炎1例报告

骨化性肌炎是指骨骼肌间质内的结缔组织、肌、腱、韧带腱膜及骨骼肌的胶原性支持组织的异常骨化现象。其中,外伤性局限性骨化性肌炎常见于肘部、髋部、股部和臀部,发生于颌面部的骨化性肌炎较为罕见。本文报告1例典型病例,并对其发病机制、临床特点、诊断和治疗进行讨论。     

骨骼肌弥漫性钙化伴随皮下囊肿一例报告

儿童期出现皮下组织弥漫性钙化或骨化改变属于罕见的临床表现,局部的骨骼肌钙化或骨化作为一种自限性疾病可以出现在手术、外伤或神经损伤之后.弥漫性的骨骼肌钙化可以见于获得性的皮肌炎,而弥漫性的骨骼肌骨化见于遗传性骨化性疾病,包括进行性骨化性肌炎、进行性骨发育异常和Albright遗传性骨营养障碍等遗传性疾病中[1,2].     

Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features

Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders predominantly affecting skeletal muscles, resulting in muscle inflammation and weakness. The 3 most common inflammatory myopathies are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. This review details the clinical findings noted in PM, DM, and the emerging entity of autoimmune necrotizing myopathy.     

A primer on electrophysiologic studies in myopathy

Electromyography and nerve conduction studies are the primary electrodiagnostic studies employed in the evaluation of patients with weakness and suspected myopathy. This article discusses the physiologic principles that serve as a framework for understanding the purpose, limitations, and interpretation of these tests. In the process the authors also review the differential diagnosis of myopathy.     

Imaging of skeletal muscle

Various diagnostic imaging techniques such as sonography, computed tomography, scintigraphy, radiography, and magnetic resonance imaging (MRI) have made possible the noninvasive evaluation of skeletal muscle injury and disease. Although these different modalities have roles to play, MRI is especially sensitive in the diagnosis of muscle disorders and injury and has proved to be useful in determining the extent of disease, in directing interventions, and in monitoring the response to therapies. This article describes how magnetic resonance images are formed and how the signal intensities in T1- and T2-weighted images may be used for diagnosis of the above-mentioned conditions and injuries.