Objective To investigate the clinical features, diagnosis, treatment and prognosis of polymyositis patients with cardiac involvement.Methods Clinical data of 41 polymyositis patients with cardiac involvement were analyzed retrospectively.Results In our group, the percentage of polymyositis with cardiac involvement was 38.3%, and 25 (60.1%) out of the 41 patients were asymptomatic. Their average age was older than the cases without cardiac involvement ( P

96% 7-10 days after culture. Immunohistochemistry of Ⅷ factor polyclone antibody showed positive in cytomembrane and cytoplasm, but negative in cell nucleus. These were cells with blood-brain barrier. CONCLUSION: Ideal endothelial cells of blood-brain barrier can be cultured by the improved cultural method of double filtering.

Objective To investigate sensitivity and specificity of myositis specific autoantibodies (MSAs) in polymyositis/dematomyositis(PM/DM ) and other neuromuscular disease. Methods Taking serum from 63 patients with PM/DM (PM/DM group)and 60 definite neuromusclar disease(non-myositis) patients(control group). All the sera were detected for two kind autoantibodies:anti-Jo-1,anti-SRP by immunoblotting method. Calculating sensitivity,specificity of anti-Jo-1 and anti-SRP autoantibodies for the diagnosis PM/DM. Results Positive ratio for anti-Jo-1 and anti-SRP autoantidodies were 17% and 5% respectively in the PM/DM group, while none of the sera from control group detected positive.The specificity of both autoantibodies diagnosis for PM/DM were 100% and (95% CI:94%~100%).The sensitivity was 22%( 95%CI:13%～34%). Conclusion Anti-Jo-1 and anti-SRP autoantibodies are highly specific to PM/DM diagnosis.

Objective To investigate the influence of ginsenoside Rgl on the proliferation of neural stem cells in adult rats with fo- cal cerebral ischemia,and to explore the possible mechanism of ginsenoside Rg1 in brain protection and anti-aging action.Methods The animal model of left middle cerebral artery occlusion(MCAO)was reproduced in adult male Wistar rats.The thymidine analog bromode- oxyuridine(BrdU)was administered intraperitoneally(50mg/kg,Sigma,USA)every four hours for four times before executing the ani- mals to label the proliferating cells.By the employment of immunohistochemical single staining and double-immunofluorescence technique, the number of BrdU immunoreacted nuclei in the subventricular zone(SVZ),the number of nestin positive cells in the subgranular zone (SGZ)as well as the nestin/BrdU double-labeled positive cells were counted.The effect of ginsenoside Rg1 on the immunoreactivity for BrdU,nestin and nestin/BrdU at 1d,3d,7d and 14d after focal cerebral ischemia were also observed to compare with that of controls,Re- sults The immunoreacted cells of BrdU,nestin and nestin/BrdU were seen in SVZ and SGZ in the hippocampus following focal cerebral ischemia.The number of BrdU,nestin-positive cells and the double-labeled positive cells all reached the peak on 7d and decreased on 14d after MCAO,and the expressions markedly increased after the rats were given ginsenoside Rg1,compared with the control(P

Acupuncture Points ; Acupuncture Therapy ; Humans ; Periarthritis ; therapy ; Shoulder Pain ; therapy

Objective To investigate the clinicopathological features of tubular aggregate myopathy.Methods Eight patients as experimental group were diagnosed with tubular aggregate myopathy in Department of Neurology,People' s Liberation Army General Hospital,between March 2000 and March 2013.The data were retrospectively analyzed.Enzyme histochemical techniques and electron microscopy were taken to observe the muscle structures.Results The detection rate of tubular aggregate was 0.374％ (8/2 137).All of the 8 patients with tubular aggregate myopathy were male.Five patients presented with episodes of muscle weakness,while 3 patients presented chronic progressive muscle weakness.The main clinical features of all patients were muscle weakness.The creatine kinase level was mildly elevated in 4 patients,while it was normal in the other 4 patients.Electromyogram showed myogenic damage in 5 patients and normal in 3 patients.All of the 8 patients denied family history.By light microscopy,hematoxylin-eosin staining showed that tubular aggregates were multiple basophilic subsarcolemmal substance.Tubular aggregate stained red with modified Gomori trichrome stain,reacted intensely for nicotinamide adenine dinucleotidetetrazolium reductase,and remained unstained on oil red O,periodic acid Schiff,sudan black B,acid phosphatas,adenosine triphosphatase.By electron microscopy,tubular aggregates were densely packed tubules predominantly in the subsarcolemmal region.Conclusion Enzyme histochemical staining and electron microscopy show special features of tubular aggregate myopathy,are the critical techniques for the diagnosis of the disease.

ObjectiveTo investigate the clinical,myopathological and molecular changes in two Chinese families with oculopharyngodistal myopathy ( OPDM).MethodsWe performed muscle biopsy and histopathologic study on the probands of two families,and further examined molecular genetic testing on PABPN1 and GNE gene. Results Family 1 included 3 affected brothers in the same generation and family 2 involved 4 patients in 2 generations. Dysarthria rather than external ophthalmoplegia was the prominent oculopharyngeal symptoms for Chinese patients. No intranuclear inclusions were observed in ultrastructural examination.The number of GCG repeats in the PABPN1 gene was within normal range and no mutations were identified in the GNE gene.ConclusionsFamily 1 is the first publication on autosomal recessive OPDM in China.The age of onset of two families was comparable with Japanese patients and the pattern of muscle involvement was different. OPDM is a distinct phenotypical,histological,and genetic entity.

Objective To explore the expression of myostatin mRNA,a negative regulator of muscle growth,in the skeletal muscle of Duchenne muscular dystrophy(DMD)patients.Methods A semi-quantitative reverse transcription polymerase chain reaction was performed to evaluate the expression of myostatin in the skeletal muscle of 7 DMD patients and 4 healthy controls.Results The level of myostatin gene expression in the skeletal muscle of DMD patients was higher than that of healthy controls(0.56 ± 0.16 vs 0.34 ± 0.15,Z =-2.268,P =0.023).Conclusions The myostatin gene expression was increased in the DMD patients compared to the healthy controls.Enhanced expression of myostatin in the skeletal muscle might be involved in the pathogenesis of DMD.

Objective To analyze and summarize the clinical , pathological features of 16 patients with centronuclear myopathy.Methods All of the 16 patients performed clinical examination and sporadic patients and a proband with family history ( n=6 ) performed serum creatine kinase , electromyography and open muscle biopsies , and the specimens were used for a standard series of histological and histochemical stainings.The clinical and pathological features of these patients were analyzed.Results The proportion of centronuclear myopathy in suspected myopathy cases was 0.127%(6/4 724) in our department.The onset time ranged from infancy to adulthood.The common initial symptoms included mild to moderate weakness of lower limbs and bilateral ptosis ( n =4 ).The other symptoms were facial weakness ( n =1 ) and ophthalmoplegia (n=1).There were 12 patients performing distal weakness exceeding proximal weakness . One family with autosomal dominant inheritance of 11 patients had a later age of onset than the sporadic ones and manifested bilateral ptosis , bilateral lower limbs weakness , especially in distal muscle.Muscle biopsies showed pronouncedly increased amount of fibers with centrally placed nuclei with predominant type Ⅰfibers and a clear perinuclear halo surrounding the centrally placed nuclei and an appearance of spoke of a wheel in some fibers.Conclusions This series of centronuclear myopathy patients manifest clinical heterogeneity.Muscle biopsies show features of centralized nuclei pronounced increase , type Ⅰfibers predominance , etc.These can provide evidences for the diagnosis of the disease.

Objective To study the clinical and pathological features of nemaline myopathy(NM) in 12 cases.Methods Clinical manifestations and pathological features of muscle-biopsy specimens were summarized and analyzed retrospectively in 12 NM cases.Results In 12 cases,7 patients with typical congenital type exhibited lower or four limbs weakness as the first symptom and benign course.Three patients with childhood onset type exhibited lower limbs weakness and progressive course,and this type of patient might have muscle atrophy.Two patients with adult onset type exhibited four limbs and throat muscle weakness,rapidly progressive course and obvious muscle atrophy,and one patient had already shown acute respiratory failure.High arched feet and elongated face were observed.Creatin kinase value in all patients was normal or mildly elevated,and all electromyography showed myogenic changes.In light microscopy,the nemaline bodies were observed in more than half muscle fibers,especially in type 1 fibers.All patients showed type 1 predominance and atrophy.Modified Gomori trichrome stains showed characteristic purplecolored rods.Muscle electron microscopy showed high electron dense nemaline bodies around nucleus and disorganized myofibrillar apparatus such as broken myofilaments,irregular myofibril and Z lines.Nemaline bodies under electron microscopy may be part of myofibril or high electron-dense bodies with no structure.Conclusions The 12 patients in this study with NM are divided into 3 types,of which adult onset type is the most severe one.The key diagnosis is based on the appearance of nemaline bodies in more than half of the muscle fibers and the muscle electron microscopy observation.