甲状腺功能减退症伴多发性肌炎样综合征三例

目的提高对甲状腺功能减退致多肌炎综合征的认识。方法报道3例甲状腺功能减退症致多肌炎综合征的临床特点。男2例,年龄分别为33、38岁;女1例,年龄64岁,均表现为四肢近端肌肉肌无力,肌酶显著升高。结果 2例男性诊断为原发性甲状腺功能减退症,女性为亚临床型甲状腺功能减退症,甲状腺激素替代治疗后效果好。结论临床上对有肌病表现、肌酶显著升高的患者须注意有无甲状腺功能减退症,多发性肌炎样综合征,应注意筛查甲状腺功能。     

成人甲状腺功能减退性肌病的临床表现

目的 了解成人甲状腺功能减退性肌病(HTM)的特点.方法 对19例成人HTM的临床资料和实验审检查进行分析.结果 成人HTM可表现为肢体近端肌无力、肌肉酸痛、肌痉挛、肌强直样改变、活动后肌僵硬,也可伴发全身多处肌肉肥大,咀嚼时出现颞肌肿胀.给予甲状腺素替代治疗可获得满意效果.甲状腺功能减退还可引起重症肌无力.需与多发性肌炎相鉴别.结论 对有肌病表现,肌酶升高的患者,应检查甲状腺功能,避免误诊为多发性肌炎或其他疾病.     

以肌炎为首发症状的甲状腺功能减退症13例临床分析

甲状腺功能减退症(简称甲减)可致血清肌酶升高,以肌无力为首发症状,易误诊为多发性肌炎。现将13例以肌炎为首发症状的甲减患者的资料分析如下。     

甲状腺功能减退并多发性肌炎重叠综合征及心脏损害1例分析

对甲状腺功能减退并多发性肌炎重叠综合征及心脏损害1例分析如下。1病历摘要女,54岁。因肌酶高7 a,乏力伴气喘2 a入院。7 a前体检发现肌酸肌酶(CK)高,在神经内科诊断多发性肌炎和桥本氏甲状腺炎,亚临床甲减。给予强的松和甲状腺素治疗,CK下降至正常,0.5 a后自行停强的松,间断服用甲状腺素。2 a     

实验性肌炎动物模型中肌电图变化分析

目的建立类似于人类多发性肌炎(PM)的豚鼠模型,观察肌电图在此模型中的变化。方法用兔肌匀浆加完全佛氏佐剂免疫注射豚鼠制作实验性自身免疫性肌炎(EAM)动物模型,将EAM豚鼠随机分为强的松治疗组及空白对照组,比较模型前后及治疗前后肌电图、肌肉病理和血清肌酶改变。结果模型制作后肌电图呈肌源性改变,肌肉病理出现骨骼肌多发性炎症病变,血清肌酶显著升高;治疗后肌电图、肌肉病理、肌酶显著好转。结论本实验所建立的EAM豚鼠模型与人类PM相似,肌电图对于评估EAM模型建立及药物疗效有重要意义。     

亚急性甲状腺炎伴肌炎样综合征一例

炎性肌病包括多发性肌炎和皮肌炎等．是以肌细胞炎性细胞浸润为主的一组疾病．可为原发性、合并肿瘤或合并其他自身免疫病。本文报告亚急性甲状腺炎合并肌炎并以肌炎症状就诊一例．探讨肌炎样综合征在甲状腺疾病的临床表现和治疗效果．以期提高该病诊断率和治愈率。     

多发性肌炎或皮肌炎临床特征及其误诊20例原因分析

多发性肌炎或皮肌炎(PM或DM)在临床上并非罕见.特发性炎症性肌病是指病因不明,临床上少数多发性肌炎或皮肌炎患者出现典型的近端肌无力、肌痛和血清肌酶升高.临床上把伴有皮肤损害的肌炎称为DM,不伴有皮肤损害的肌炎为PM[1].现对我院收治的5例及市第一医院15例多发性肌炎或皮肌炎共20例病患诊治经过回顾性分析如下.     

肌肉电生理及病理诊断多发性肌炎的临床意义

目的总结和分析多发性肌炎患者的临床特征、肌肉电生理及病理改变的临床意义,指导临床诊治。方法收集分析34例多发性肌炎患者的首发临床症状、体征、肌酶谱、肌电图、肌活检结果。结果多发性肌炎患者以近端肌受累为主,以肌肉无力、酸痛多见;该组病例中发现肌酸激酶升高、肌电图异常和肌活检异常的百分率分别是79．41％、68．97％、58．33％。结论肌电图及肌活检病理技术是多发性肌炎的重要诊断手段。     

甲状腺功能减退性肌病3例

目的：探讨甲状腺功能减退性肌病的临床特点和诊断方法。方法：对3例甲状腺功能减退性肌病患者的临床资料进行回顾性分析。结果：3例患者主要表现为近端肌无力、运动后肌肉疼痛、肌强直和假肥大。实验室检查显示肌酶升高、甲状腺激素水平降低、促甲状腺激素水平升高。肌电图检查主要呈肌源性损害。使用甲状腺激素替代治疗，预后良好。结论：甲状腺功能减退性肌病临床少见。对于有运动后肌肉疼痛、肌强直、肌肥大等肌病表现患者，甲状腺功能检查有助于明确诊断。     

特发性炎性肌病时血清新蝶呤的浓度

多发性肌炎和皮肌炎统称特发性炎性肌病，为研究炎性肌病的活动程度和血清新蝶呤（Neovterin）浓度之间的关系，检查特发性炎性肌病44例，男11例，女33例，年龄17岁～66岁（平均38岁），病程8月～48月（平均24月），均经肌肉活俭及肌电摘记法确诊。分3组，第1组原发性多肌炎15例（3例有关节炎，13例有肺问质病）；第2组原发性皮肌炎13例（5例有关节炎，9例有肺间反病）；第3组16例，多肌炎或皮肌炎合并系统性硬皮病（15例有关节炎，16例有肺间质病）。用放射免疫法测定血清新蝶吟浓度，对照组为31例健康人。检查结果健康入血清新蝶哈浓度平…     

抗HMGCR/SRP抗体阳性的特发性炎性肌病特点分析

目的:探讨抗HMGCR/SRP抗体阳性的特发性炎性肌病(IIM)的临床和病理特点。方法:收集HMGCR/SRP抗体阳性的IIM患者5例,对其危险因素、临床表现、实验室检查、肌电图、肌肉MRI、肌肉病理、肌炎自身抗体及药物治疗进行分析。结果:抗HMGCR/SRP抗体阳性的IIM患者临床变异较大,但大多有肌肉无力;血清肌酸激酶均较高;5例患者肌电图均表现为典型的肌源性损害。肌肉MRI主要表现为肌肉水肿。3例为典型坏死性肌病表现,1例镜下偶见肌细胞坏死,1例为多发性肌炎表现。HMGCR抗体阳性患者其中1例为正服用他汀药物,另一患者服用抗精神病药物;SRP抗体阳性患者1例为自身免疫性疾病患者,1例长期服用他汀,另1例病因未明确。激素治疗效果不一,2例加用丙种球蛋白治疗,1例加用免疫抑制剂治疗。结论:抗HMGCR/SRP抗体阳性的IIM临床表现各异,肌电图仅能定位肌肉损害,主要依靠肌肉活检,肌炎自身抗体检查更有助于诊断和具体分型,疗效各型不一。     

Inclusion body myositis masquerading as polymyositis: a case study

A case of inclusion body myositis masquerading as unresponsive polymyositis is presented. A 56-year-old woman diagnosed with "biopsy-proven" polymyositis in 1991 was referred to our clinic in 1997 with progressive, painless weakness that was unresponsive to steroid therapy. Further evaluation, including electromyography and review of the original muscle biopsy specimen, found a diagnosis of inclusion body myositis, leading to a change in the patient's prognosis and management. Inclusion body myositis is frequently mistaken for polymyositis, despite the fact that it is now the most common inflammatory myopathy affecting people older than 50 years. The purpose of this report is to increase awareness of this disease, to enhance early diagnosis, and to ensure appropriate management. We discuss the clinical findings, pathogenesis, and physiatric management, as well as compare this disease with other idiopathic inflammatory myopathies.     

Diagnosis and Management of Immune-Mediated Myopathies

Immune-mediated myopathies (IMMs) are a heterogeneous group of acquired muscle disorders characterized by muscle weakness, elevated creatine kinase levels, and myopathic electromyographic findings. Most IMMs feature the presence of inflammatory infiltrates in muscle. However, the inflammatory exudate may be absent. Indeed, necrotizing autoimmune myopathy (NAM), also called immune-mediated necrotizing myopathy, is characterized by a necrotizing pathologic process with no or minimal inflammation in muscle. The recent discovery of antibodies associated with specific subtypes of autoimmune myopathies has played a major role in characterizing these diseases. Although diagnostic criteria and classification of IMMs currently are under revision, on the basis of the clinical and muscle histopathologic findings, IMMs can be differentiated as NAM, inclusion body myositis (IBM), dermatomyositis, polymyositis, and nonspecific myositis. Because of recent developments in the field of NAM and IBM and the controversies around polymyositis, this review will focus on NAM, IBM, and dermatomyositis.     

Bilateral leg pain and unilateral calf atrophy caused by polymyositis accompanying lumbar spinal stenosis and disc herniation: a case report

Polymyositis is a subgroup of idiopathic inflammatory myopathies characterized by symmetric proximal limb weakness and chronic skeletal muscle inflammation. We herein report the first case of bilateral leg pain and unilateral calf atrophy caused by polymyositis accompanying lumbar spinal stenosis and disc herniation. A 52-year-old man presented with intermittent claudication and calf pain that had become gradually aggravated during the last 3 months. Magnetic resonance imaging showed spinal stenosis at the L3/4 and L4/5 levels and lumbar disc herniation at the L4/5 level. Preoperative laboratory investigations revealed elevated muscle enzyme concentrations. Magnetic resonance imaging also showed atrophy, fatty degeneration, and edema in both calf muscles. Histological examination showed inflammatory myositis and fibrosis in the perifascicular connective tissues. The patient was diagnosed with polymyositis. We performed decompressive laminectomy at the L3/4 and L4/5 levels and discectomy at the L4/5 level. After administration of prednisolone for 6 months and methotrexate for 3 months, the patient’s bilateral calf pain and abnormal laboratory findings improved. The combination of surgical decompression and adequate medical treatment resulted in a successful recovery. Polymyositis should be suspected in patients with lumbar spinal stenosis or lumbar disc herniation who exhibit increased muscle enzyme concentrations or lower extremity muscle atrophy.     

Focal myositis of unilateral leg

Focal myositis is a rare, benign inflammatory pseudotumor of the skeletal muscle of unknown etiology. In Korea, there is no case report of focal myositis, which is not combined with connective tissue disease. We present an unusual case of focal myositis with ankle contracture, involving more than two muscles. A 26-year-old man visited our clinic complaining of right ankle contracture and leg muscle pain. Physical examination revealed no muscle weakness or any other neurological abnormality. T2-weighted magnetic resonance imaging of the right leg demonstrated diffuse high signal intensity of the right gastrocnemius, flexor digitorum longus, and tibialis anterior muscles. Needle electromyography showed profuse denervation potentials with motor unit action potentials of short duration and small amplitude from the involved muscles. All these findings suggested a diagnosis of focal inflammatory myositis and the patient was put under oral prednisolone and physical therapy.     

甲状腺功能减退性肌病合并糖尿病1例报告及文献复习

[目的]提高对甲状腺功能减退性肌病的认识和诊治水平.[方法]报道甲状腺功能减退性肌病1例,并检索2000～2006年中国期刊全文数据库检索国内报道及PUBMED上检索到的国外文献,进行汇总研究,并讨论该病相应进展.[结果]病例为中年男性,合并糖尿病,2周甲状腺激素替代治疗后效果好.共检索到国内报道16例,国外报道若干例,多为男性患者.发病机制可能与遗传、甲状腺机能减退代谢异常以及自身免疫有关.肌无力、肌痛是普遍存在的症状.肌酸激酶常中度升高.一般肌电图和肌活检无特异性改变.[结论]甲状腺功能减退性肌病好发于男性,该病患者具有甲状腺功能减退的症状群和肌病的症状群.甲状腺激素替代是主要治疗手段.该病预后尚好.     

Idiopathic Inflammatory Myopathies: Current Trends in Pathogenesis,Clinical Features,and Up-to-Date Treatment Recommendations

Recently, there have been important advances in the understanding of the pathophysiologic features, assessment, and management of patients with a newly diagnosed idiopathic inflammatory myopathy (IIM). Myositis-specific autoantibodies have been identified to define patient subgroups and offer prognostic implications. Similarly, proinflammatory cytokines, such as interleukin 6 and type 1 interferon–dependent genes, may serve as potential biomarkers of disease activity in adult and juvenile patients with dermatomyositis (DM). Moreover, magnetic resonance imaging has become an important modality for the assessment of muscle inflammation in adult IIM and juvenile DM. Immune-mediated necrotizing myopathies also are being recognized as a subset of IIM triggered by medications such as statins. However, confusion exists regarding effective management strategies for patients with IIM because of the lack of large-scale, randomized, controlled studies. This review focuses primarily on our current management and treatment algorithms for IIM including the care of pediatric patients with juvenile DM. For this review, we conducted a search of PubMed and MEDLINE for articles published from January 1, 1970, to December 1, 2011, using the following search terms: idiopathic inflammatory myopathies, dermatomyositis, polymyositis, juvenile dermatomyositis, sporadic inclusion body myositis, inclusion body myositis, inflammatory myositis, myositis, myopathies, pathogenesis, therapy, and treatment. Studies published in English were selected for inclusion in our review as well as additional articles identified from bibliographies.     

Magnetic resonance imaging of inflammatory myopathies

The following article reviews the role of magnetic resonance imaging (MRI) in patients with idiopathic inflammatory myopathies (IIMs), focusing on the 3 major types of IIM: polymyositis, dermatomyositis, and inclusion-body myositis. After a brief introduction with general information about IIM, we will discuss the reasons why MRI plays an important role in the diagnosis and management of patients with polymyositis, dermatomyositis, and inclusion-body myositis. Magnetic resonance imaging can confirm the diagnosis and can help to phenotype the disease. Moreover, the support of MRI is important in addressing the muscle biopsy site and in reducing the high false-negative rate of biopsy when performed in a blind fashion. In monitoring therapy, MRI can add important information about the activity of the muscle disease and can identify cases where continued immunosuppressive therapy is no longer warranted owing to complete fatty replacement of the muscles. Lastly, we provide an overview about some advanced MRI techniques that focus more on function than on morphology of muscle.     

Sleep apnea and hypothyroidism

Thyroid deficiency states are now a well recognized cause of the sleep apnea syndrome. The spectrum of disease ranges from mild, asymptomatic hypothyroidism to severe myxedema, and the disorder is associated with both obstructive and central types of sleep apnea. A variety of factors may be involved, including upper airway obstruction with or without obesity, and alterations in ventilatory drive. The definitive therapy is thyroid hormone replacement, which has been shown to diminish or completely eliminate apneic episodes and arterial oxygen desaturation, as well as to effect many improvements in sleep patterns and overall sleep efficiency. The incidence of thyroid deficiency states in patients with sleep apnea syndrome is not known, but it seems reasonable to evaluate thyroid function in all patients. Thyroid replacement therapy seems logical for the treatment of sleep apnea in patients with previously unrecognized subclinical hypothyroidism. Much remains to be learned about the diagnosis and treatment of sleep apnea syndromes associated with thyroid hormone deficiency, and further studies are needed.     

特发性肌病的病理分型及其与患者功能障碍和预后的关系

背景肌肉活检病理是诊断特发性炎症性肌病(inflammatory myopathies,IM)的重要手段.然而目前,对于肌肉病理改变与患者的功能障碍及预后的联系,缺少大型系统性的研究.目的提出并探讨IM的肌肉活检病理类型及其与患者的功能障碍及预后的联系.设计以诊断为依据,回顾性研究.单位济南市中心医院神经内科及山东大学齐鲁医院神经内科.对象山东大学齐鲁医院神经病理实验室的630例肌活检标本中检出病理确诊和拟诊的炎症性肌病.方法回顾性分析IM的病理改变,归纳出各病理类型的特点.随访并评估IM患者的预后情况.主要观察指标①肌肉病理改变,主要包括纤维坏死的程度、坏死的分布特点和炎症细胞浸润的情况.②临床特点(吞咽、肌痛、肌无力、皮肤损害等)及其愈后.结果共检出IM119例,其中重、中、轻度坏死性肌炎分别为11例、19例和27例,束周坏死/萎缩性肌炎20例,无炎细胞浸润的坏死性肌炎22例,间质性肌炎11例,肌筋膜炎3例,包涵体肌炎4例,肉芽肿性肌炎和增殖性肌炎各1例.其中72例获得随访资料,轻度坏死性肌炎和束周坏死/萎缩性肌炎的好转和治愈的百分比高于中、重度坏死性肌炎.间质性肌炎和无炎细胞浸润的坏死性肌炎高于重度坏死性肌炎.结论IM的病理类型对判断预后具有重要的参考价值.