案例成果神经病pm

1、 Polymyositis is an idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness; elevated skeletal muscle enzyme levels; and characteristic electromyography (EMG) and muscle biopsy findings (see the images below). Clinically similar to polymyositis, dermatomyositis is an idiop

2、athic, inflammatory myopathy associated with characteristic dermatologic manifestations. Inclusion body myositis is a slowly progressive, idiopathic, inflammatory myopathy with characteristic pathologic findings that is generally found in older males. n 炎性肌病分为特发性和感染性炎性肌病分为特发性和感染性n特发性包括：多发性肌炎、皮肌炎及包含体

3、特发性包括：多发性肌炎、皮肌炎及包含体肌炎等肌炎等n感染性肌炎包括病毒、细菌及寄生虫感染所感染性肌炎包括病毒、细菌及寄生虫感染所致的肌炎致的肌炎1. 单纯型多发性肌炎单纯型多发性肌炎(PM)2. 皮肌炎皮肌炎(DM)3. 儿童多发性肌炎或皮肌炎儿童多发性肌炎或皮肌炎4. 多发性肌炎和皮肌炎合并结缔组织病多发性肌炎和皮肌炎合并结缔组织病5. 多发性肌炎和皮肌炎合并恶性肿瘤多发性肌炎和皮肌炎合并恶性肿瘤 The major manifestation of polymyositis is inflammation leading to destruction of muscle and incre

4、asing muscular weakness. As the disease progresses, the muscle tissue is replaced by functionless scar tissue. Both have features in common with rhematoid arthritis, lupus, and progressive systemic sclerosis (scleroderma).流行病学流行病学 n性别：女性略多于男性性别：女性略多于男性n年龄：两个高峰年龄：两个高峰, 儿童和儿童和4070岁岁n发病率：北美发病率：北美25/10,

5、0000 The causes of polymyositis and dermatomyositis are unknown. It appears that abnormal immunological factors are responsible for at least part of inflammatory attack against muscle tissue. The symptoms of muscular weakness usually wax and wane, but in some instances, the disease may progress rapi

6、dly. The disease may affect persons of any age, but the peak incidence is in the fifth and sixth decades of life. Women are affected twice as commonly as men. An increased association of myositis has been found with human leukocyte antigen (HLA) haplotypes A1, B8, and DR3, which also increase the ri

7、sk for autoimmune diseases. Environmental triggers, especially infectious agents, have been suggested as etiologic agents. These include the following: Coxsackievirus B1 HIV HTLV-1 Hepatitis B Influenza Echovirus Adenovirusl 病毒感染：如感冒病毒感染可引起轻型的肌炎，病毒感染：如感冒病毒感染可引起轻型的肌炎，HIV(+)，HTLV-I感染者常伴感染者常伴PM，但未分离出病毒

8、，但未分离出病毒和和 测出升高的抗病毒抗体。测出升高的抗病毒抗体。l 免疫：免疫：PM主要细胞免疫主要细胞免疫, 抗原性质不清；抗原性质不清；DM主要体液免疫，肌肉小血管壁可见免疫复合物主要体液免疫，肌肉小血管壁可见免疫复合物IgG、IgM和补体和补体C3等沉积。等沉积。l 该病常合并胶原血管病该病常合并胶原血管病, 如如Crolone氏病氏病, SLE, 结结节性肉芽肿节性肉芽肿, 类风湿类风湿, 重症肌无力等重症肌无力等Muscle Weakness: Muscle Weakness: Muscles of the shoulder, arms, pelvis, and thighs ar

9、e the most frequent targets, but the reasons are unknown. As the disease spreads, other muscles may become involved. For example, the diaphragm and chest wall muscles, which are needed for breathing, may be attacked. The pharyngeal muscles, which are needed for swallowing, may be weakened to the poi

10、nt that swallowing solid foods becomes impossible. Heart muscle is another potential target.1隐袭、逐渐进展、几周或几个月达高峰；隐袭、逐渐进展、几周或几个月达高峰；2近端肌肉无力近端肌肉无力3颈部肌肉无力：抬头困难颈部肌肉无力：抬头困难, 伸肌较屈肌受累重伸肌较屈肌受累重4球部肌肉：吞咽困难球部肌肉：吞咽困难(约约50%), 构音障碍少见构音障碍少见5肌肉疼痛占肌肉疼痛占65%，晚期远端肌肉可有轻度萎缩，晚期远端肌肉可有轻度萎缩6合并其它结缔组织病可伴有其它症状合并其它结缔组织病可伴有其它症状7DM临

11、床有皮肤受累的表现，肌痛较明显临床有皮肤受累的表现，肌痛较明显840岁后发病者岁后发病者10%可能伴有恶性肿瘤可能伴有恶性肿瘤Goetz: Textbook of Clinical Neurology, 2nd ed., Copyright 2003 Elsevier NormalPolyphasicMyopathicGoetz: Textbook of Clinical Neurology, 2nd ed., 2003 Elsevier RestingInterference patternFigure 2: Histological findings in polymyositis and

12、 dermatomyositisA, B: Depletion of capillaries in dermatomyositis (A) with dilatation of the lumen of the remaining capillaries, compared with a normal muscle (B).C: Perifascular atrophy in dermatomyositis. D: Endomysial inflammation in polymyositis and inclusion-body myositis with lymphocytic cells

13、 invading healthyfibres. E: The MHC-I/CD8 complex in polymyositis and inclusion-body myositis. MHC-I (green) is upregulated on all the muscle fibres, and CD8-positiveT cells (orange) that also express MHC-I, invade the fibres.l 血生化：血生化：CK等等, ESR异常异常, 免疫球蛋白免疫球蛋白，部分部分RF(+), 恢复期可正常；恢复期可正常；l 24小时尿肌酸增加小时

14、尿肌酸增加l EMG：肌源性损害：肌源性损害l 肌肉活检肌肉活检Figure and caption from American College of Rheumatology slide collectionMRI of thighs showing increased signal in the quadriceps muscles bilaterally consistent with inflammatory myositis. Histopathology of polymyositis showing endomysial mononuclear inflammatory infi

15、ltrate and muscle fiber necrosis. l PM：肌纤维斑片状坏死和再生：肌纤维斑片状坏死和再生, 血管周围有血管周围有淋巴细胞浸润淋巴细胞浸润(75%); 组织化学证实为组织化学证实为CD8, T-淋淋 巴细胞巴细胞l DM：肌束膜结缔组织炎性细胞浸润，束周：肌束膜结缔组织炎性细胞浸润，束周肌纤维萎缩。该病原发的病理改变在血管肌纤维萎缩。该病原发的病理改变在血管1. 病史及临床表现病史及临床表现2. 血清肌肉酶谱测定血清肌肉酶谱测定3. EMG提示肌源性损害提示肌源性损害4. 肌肉活检肌肉活检: 典型的病理改变典型的病理改变 Treatment may invol

16、ve using a corticosteroid drug, although their effectiveness is less predictable in these disorders than in some other inflammatory diseases. As a result, many rheumatologists turn to one of the cytotoxic drugs (agents that destroy abnormal cells) in conjunction with the corticosteroids, in an attem

17、pt to suppress muscle inflammation and retard the destructive process. Usually by combining the two classes of drugs, lower and safer doses of both can be used thereby avoiding some of the more serious side- effects that might be produced when either is used alone in higher doses. 1. 肾上腺皮质激素治疗肾上腺皮质激

18、素治疗: 强的松强的松6080mg 或或 氢考氢考 200300 mg 或地或地塞米松塞米松 1020 mg 静脉点滴静脉点滴2周后改为强的周后改为强的松口服症状好转后渐减量；松口服症状好转后渐减量；3个月内减至维个月内减至维持量持量520mg/天天, 维持维持25年。年。2. 其它免疫抑制剂其它免疫抑制剂 依米兰依米兰 100200mg 1/天天 Cyclophosphamide 50100mg/day Methotroxate 3060mg /week *注意血相注意血相, 肝功能等肝功能等3. 免疫球蛋白静脉滴注，免疫球蛋白静脉滴注，0.4g/（kg.d）4. 血浆交换血浆交换In mo

19、st patients, polymyositis responds well to treatment, although residual weakness occurs in approximately 30% of patients. Osteoporosis, a common complication of long-term corticosteroid therapy, may cause significant morbidity. Poor prognostic factors include the following:Advanced ageFemale sexAfri

20、can American raceInterstitial lung diseasePresence of anti-Jo-1 (lung disease) and anti-SRP antibodies (severe muscle disease, cardiac involvement)Associated malignancyDelayed or inadequate treatmentDysphagia, dysphoniaCardiac and pulmonary involvementComplications of polymyositis may include the follow