Background: Antibodies (Ab) against the acetylcholine receptor (AChR) are found in 80-90% of generalized myasthenia gravis (MG),but only in 50% of pure ocular MG. Furthermore,Ab against the muscle-specific receptor ty...Background: Antibodies (Ab) against the acetylcholine receptor (AChR) are found in 80-90% of generalized myasthenia gravis (MG),but only in 50% of pure ocular MG. Furthermore,Ab against the muscle-specific receptor tyrosine kinase (MuSK) were detected in 38-54% of patients with AChR-Ab negative (“ seronegative” ) MG,but not in pure ocular MG. Methods: 2 case reports have been analysed: Two patients (42 years old and 61 years old) with primary ocular MG were studied. Both patients complained of fluctuating bilateral ptosis and double vision lasting for 2 to 3 months. Results: AChR-Ab were negative but MuSK-Ab were clearly positive. In spite of treatment with pyridistigmin and prednisolone,the symptoms in patient 1 generalized within 4 weeks causing dysphagia,limb muscle weakness and respiratory crisis. Symptoms immediately responded to plasmapheresis. Patient 2 clearly improved under treatment with pyridostigmine,methylprednisone,and azathioprine. There was no generalizationwithin a course of 12 months. Conclusions: Both cases clearly indicate that MuSKAb can be found in primary isolated ocular MG lasting for 2 to 3 months. In a primary ocular manifestation of MG the detection of MuSK might indicate the risk for subsequent generalization and might have implications for immunosuppressive therapy. Therefore,seronegative MG requires testing for MuSK-Ab.展开更多
目的:本研究通过分析神经源性诱导因子Agrin及其受体-肌特异性受体酪氨酸激酶Mu SK在成长期(离乳-性成熟)骨骼肌的表达水平,探讨其在神经肌肉连接(NMJ)发育中的作用及早期运动干预的影响。方法:出生18天SD雄性大鼠130只随机分为运动组5...目的:本研究通过分析神经源性诱导因子Agrin及其受体-肌特异性受体酪氨酸激酶Mu SK在成长期(离乳-性成熟)骨骼肌的表达水平,探讨其在神经肌肉连接(NMJ)发育中的作用及早期运动干预的影响。方法:出生18天SD雄性大鼠130只随机分为运动组50只、悬吊组和对照组各40只,运动组和悬吊组分别进行运动增强(跑台运动)和运动减弱(尾部悬吊)干预。对照组3周龄时,各组4周龄、5周龄、6周龄、8周龄时分别取材10只。测定并比较正常发育(3~8周龄)及运动干预下腓肠肌Agrin和Mu SKm RNA水平(RT-PCR法)及腓肠肌Mu SK蛋白水平(ELISA法)的差异。结果:(1)对照组的Agrin m RNA在4周龄较3周龄表达减少(P<0.01),5周龄再次出现表达高峰(P<0.01),在4周龄后Mu SK m RNA表达减少(P<0.01),6周龄再次出现Mu SK m RNA表达高峰(P<0.01);运动组Mu SK m RNA表达水平在5周龄时显著高于对照组(P<0.01),而悬吊组Mu SK m RNA表达水平在各周龄均低于对照组(P<0.05,P<0.01)。(2)运动组Mu SK蛋白水平在各周龄组均高于对照组(P<0.05);悬吊组在4周龄时低于对照组(P<0.05)。结论:成长期腓肠肌Agrin及其受体Mu SK的表达具有一定的时间规律,在出生3~4周(离乳前后)呈现逐渐减少的趋势,5~6周(青春期)再次出现表达高峰;在此期间进行适当的运动训练可以促使Agrin及其受体Mu SK表达增多,这可能作为一种机制,促进NMJ在成长期的发育过程。展开更多
文摘Background: Antibodies (Ab) against the acetylcholine receptor (AChR) are found in 80-90% of generalized myasthenia gravis (MG),but only in 50% of pure ocular MG. Furthermore,Ab against the muscle-specific receptor tyrosine kinase (MuSK) were detected in 38-54% of patients with AChR-Ab negative (“ seronegative” ) MG,but not in pure ocular MG. Methods: 2 case reports have been analysed: Two patients (42 years old and 61 years old) with primary ocular MG were studied. Both patients complained of fluctuating bilateral ptosis and double vision lasting for 2 to 3 months. Results: AChR-Ab were negative but MuSK-Ab were clearly positive. In spite of treatment with pyridistigmin and prednisolone,the symptoms in patient 1 generalized within 4 weeks causing dysphagia,limb muscle weakness and respiratory crisis. Symptoms immediately responded to plasmapheresis. Patient 2 clearly improved under treatment with pyridostigmine,methylprednisone,and azathioprine. There was no generalizationwithin a course of 12 months. Conclusions: Both cases clearly indicate that MuSKAb can be found in primary isolated ocular MG lasting for 2 to 3 months. In a primary ocular manifestation of MG the detection of MuSK might indicate the risk for subsequent generalization and might have implications for immunosuppressive therapy. Therefore,seronegative MG requires testing for MuSK-Ab.
文摘目的:本研究通过分析神经源性诱导因子Agrin及其受体-肌特异性受体酪氨酸激酶Mu SK在成长期(离乳-性成熟)骨骼肌的表达水平,探讨其在神经肌肉连接(NMJ)发育中的作用及早期运动干预的影响。方法:出生18天SD雄性大鼠130只随机分为运动组50只、悬吊组和对照组各40只,运动组和悬吊组分别进行运动增强(跑台运动)和运动减弱(尾部悬吊)干预。对照组3周龄时,各组4周龄、5周龄、6周龄、8周龄时分别取材10只。测定并比较正常发育(3~8周龄)及运动干预下腓肠肌Agrin和Mu SKm RNA水平(RT-PCR法)及腓肠肌Mu SK蛋白水平(ELISA法)的差异。结果:(1)对照组的Agrin m RNA在4周龄较3周龄表达减少(P<0.01),5周龄再次出现表达高峰(P<0.01),在4周龄后Mu SK m RNA表达减少(P<0.01),6周龄再次出现Mu SK m RNA表达高峰(P<0.01);运动组Mu SK m RNA表达水平在5周龄时显著高于对照组(P<0.01),而悬吊组Mu SK m RNA表达水平在各周龄均低于对照组(P<0.05,P<0.01)。(2)运动组Mu SK蛋白水平在各周龄组均高于对照组(P<0.05);悬吊组在4周龄时低于对照组(P<0.05)。结论:成长期腓肠肌Agrin及其受体Mu SK的表达具有一定的时间规律,在出生3~4周(离乳前后)呈现逐渐减少的趋势,5~6周(青春期)再次出现表达高峰;在此期间进行适当的运动训练可以促使Agrin及其受体Mu SK表达增多,这可能作为一种机制,促进NMJ在成长期的发育过程。
