Corticosteroids Treatment in Spinal Cord and Neuromuscular Disorders

Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of the vertebrates, as well as the synthetic analogs of these hormones that are synthesized in the laboratories. Two main classes of corticosteroids, glucocorticoids, and mineralocorticoids, are involved in a wide range of physiologic processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Corticosteroids have been used for almost 60 years in medicine and their roles in patients have always been discussed by researchers and clinicians dedicated in the related field. Currently, they are still used in the treatment of patients with neurological disorders. Usually, corticosteroids are used in the treatment of various inflammatory diseases and conditions. In this review, we present five key indications, i.e., neuromyelitis optica, acute spinal cord injury, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, polymyositis/dermatomyositis for the systemic use of corticosteroids in neurology based on a mix of quality of evidence, prevalence, and impact on disease management.

Isolation and identification of serum exosomes in the patients with polymyositis/dermatomyositis / 临床检验杂志

Objective To isolate and identify exosomes from serum samples of the patients with polymyositis / dermatomyositis (PM/ DM),and analyze their protein composition preliminarily.Methods Exosomes from serum samples of the patients with PM/DM were isolated and purified by the ExoQuickTM kit.The morphological characteristics and particle size of exosomes were determined by transmission electron microscope (TEM) and NanoSight analyzer,respectively.The surface markers of exosomes such as CD9,CD81 and Flotillin-2 were identified by western blot.The concentration and composition of exosome protein were determined by the BCA method and SDS-PAGE,respectively.Results The exosomes from serum samples of PM/DM patients displayed round or oval vesicles with membrane structure under TEM,and their diameter range was about (92 ± 67) nm.western blot showed that these exosomes expressed CD9,CD81 and Flotillin-2.The total protein concentrations of exosomes in the patients with PM/DM and healthy controls were 14.68 (6.00,32.55) μg/μL and 14.09 (8.00,23.28) μg/μL,respectively.SDS-PAGE showed that high-abundance proteins enriched in 55-70 kD in both PM/DM patients and healthy controls,and that there were different bands in 40-55 kD between them.Conclusion Exosomes are isolated from serum samples of the patients with PM/DM successfully,and their protein concentration and composition are analyzed preliminarily,which provides the experimental evidences for further finding differential proteins.

Effect of Artesunate on Experimental Immune Myositis Animal Model / 中华皮肤科杂志

Objective To investigate the effect of Artesunate (Art) on experimental immune myositis (EIM) animal model. Methods Guinea pigs were immunized with rabbit muscle homogenate in Freund′s adjuvant, and a generalized myositis fundamentally similar to human polymyositis was established. These animals were divided into two groups: the control group (injected intraperitoneally with normal saline everyday) and the treatment group (injected intraperitoneally with Art everyday). Results The serum levels of CPK, AST and LDH were significantly lower in the treatment group than those in the control group. EMG demonstrated that the treatment group kept improving along with the elapse of time. Histological findings revealed that the damage of muscle in the treatment group was less severe than that of the control group. Conclusion Art is effective in therapy of EIM guinea pigs. The result of this study provides a reliable evidence that Art might be used for the treatment of PM/DM and other immune diseases.

The Clinical Study On Inflammatory Myopathies / 대한류마티스학회지

OBJECTIVE: To determine possible similarities and differences in clinical and laboratory features and prognosis between patients with inflammatory myopathies diagnosed at Kangnam St. Mary's hospital and other reported series. METHODS: Medical records of 52 patients diagnosed as inflammatory myopathies by Bohan and Peter criteria who were admitted to Kangnam St. Mary's hospital between January 1989 and August 1994, were reviewed. The patients were further classified into 5 groups proposed by Bohan and Peter ; group I primary polymyositis(PM), group II primary dermatomyositis(DM), group III polymyositis/dermatomyositis(PM/DM) with neoplasia, group IV juvenile PM/DM, group V PM/DM associated with connective tissue disease. RESULTS: 1) There were 52 PM/DM cases with mean age at diagnosis of 34. 4 ranging from 6 to 67 years, and significantly more females(male:female = 9:43). 2) Of 52 patients, group II was the most frequent group(36.5%), and the next were as follows ; group I 28.8%, group V 25%, group IV 5.7%, group 3.8%. Systemic lupus erythematosus(SLE) was the commonest connective tissue disease, and malignancy was associated in 2 cases including breast cancer and small cell carcinoma of lung. 3) At presentation, 86.5% had proximal muscle weakness, 61.5% had arthralgia/arthritis and 44. 2% had Raynaud phenomenon. 4) The elevated serum LDH, aldolase and creatine kinase were found in 84. 6%, 84. 1%o, 71.2% of the patients respectively. Positive electromyography and muscle biopsy consistent with inflammatory myopathy were also found in 91.4%, 88.8% respectively. 5) Positive antinuclear antibody, rheumatoid factor, anti-Ro, anti-nRNP were found in 59.6%, 17.3%, 16.7%, 8.3% of patients respectively, mainly in group V. Anti-Jo-1 antibody was present in 25.8% of patients tested(8/31), including group II 4 cases, group V 3 cases and group I I case. 6) All 8 patients with anti-Jo-1 antibody had interstitial lung disease(ILD) and Raynaud phenomenon with statistical significance, compared with those without anti-Jo-1 antibody(P<0.01). 7) Among the 43 patients in whom follow up was available, 10 patients died(mortality rate 23.2%). The main cause of death were infection(4 cases), and respiratory muscle weakness, respiratory failure due to ILD, malignancy 2 cases respectively. CONCLUSION: The results of the study demonstrated that clinical expression of PM/DM were similar to previously reported series except relatively high frequency of primary dermatomyositis in this study. Anti-Jo-1 antibody was main autoantibody in inflammatory myopathies, which was associated with extramuscular manifestations, such as ILD and Raynaud's phnomenon.

Efficacy of Intravenous Immunoglobulin Therapy in Refractory Polymyositis and Dermatomyositis / 대한류마티스학회지

OBJECTIVES: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many interventions including corticosteroids, immunosuppressive drug, and plasmapheresis to treat patients with PM/DM are not always effective, and may be associated with certain serious side effects. Intravenous immunoglobulin(IVIG) has been useful in a number of autoimmune diseases. An attempt was made to evaluate the efficacy of IVIG in refractory PM/DM. METHODS: Six patients with conventional treatment-refractory PM/DM [ 1 man and 5 women; mean age of 27(7-49)years ; PM(2), DM(2), juvenile DM(2)] received high doses of IVIG(2 mg/kg) dividing over consecutive 2 or 5 days. The IVIG infusion was conducted each month for 6 months. Clinical evaluations, including proximal muscle power, functional grading, and biochemical studies(creatine kinase(CK), aldolase) were performed before each or every IVIG infusion. Clinical evaluations were considered successful if more than 20% improvement of initial score was obtained. Biochemical results were considered "good" if muscle enzymes decreased more than 30% of initial values. RESULTS: Clinical improvement of proximal muscle power was noted in 5 patients after 6 courses of IVIG infusion. Timed stand test and functional grading score were also improved in all patients. All patients showed good response of biochemical results, except two cases with normal initial CK levels. Mean daily prednisolone dosage was significantly reduced(before IVIG: 39.2+/-9.2 mg/day vs after 6th IVIG: 7.3+/-2 mg/day, p<0.01). One patient experienced mild dyspnea and sweating during the course of IVIG infusion, which were disappeared with the reduction of daily IVIG dosage. CONCLUSIONS: These results suggest that IVIG is a safe and effective therapy in patients with PM/DM, who are resistant to traditional therapies or have limitations for their use.

Study on the relationship between the expressions of glucocorticoid receptor ? and ? mRNA in peripheral blood mononuclear cell of patients with polymyositis/dermatomyositis and the glucocorticoid efficacy / 中国免疫学杂志

Objective:To explore the relationship between expressions of guococorticoid receptor(GR)? and ? mRNA in peripheral blood mononuclear cell(PBMC) of patients with polymyositis/dermatomyositis(PM/DM) and the glucocorticoid(GC) efficacy.Methods:Reverse transcription-polymerase chain reaction(RT-PCR) was used to semiquantite the expressions of GR? and ? mRNA in PBMC of 18 cases of PM/DM patients sensitive to GC,10 cases of PM/DM patients resistant to GC and 20 cases of health blood donors.The levels of cortisol in sera of PM/DM patients and of health donors were detected using radioimmunoassay.Results:There was no significant difference in the levels of seral cortisol among PM/DM patients sensitive to GC, resistant to GC and the health donors.The expressions of GR? mRNA in PBMC from PM/DM patients either sensitive to GC or resistant to GC were significantly lower those that of the normal control. Moreover,the expression of GR? mRNA in PBMC of PM/DM patients resistant to GC was significantly lower than that of patients sensitive to GC. While the expression of GR? mRNA in PBMC of PM/DM patients was significantly higher than that of the normal control,there was no significant difference of the expression of GR? mRNA between PM/DM patients sensitive and resistant to GC.Conclusion:The lower expression of GR? mRNA in PM/DM patients may be related to resistance to GC therapy,whereas there is no significant role of the expression level of GR? mRNA.