A cohort study on myasthenia gravis patients in China.

Clinical classification and age distribution in myasthenia gravis (MG) cases seem different between Oriental and Caucasian populations, but there have rarely been any clinical studies on MG patients from mainland China. The goal of the current study was to perform a comprehensive survey of myasthenia gravis in a hospital in China, establishing contemporary cohort data and clinical features. 1,108 unselected patients with MG attending the 309th Hospital of PLA, Beijing, China were studied during a 36-month period from July 2008 to June 2011. The sex ratio was 1:1 (F:M). 62.5 % of patients presented as adolescents and adults. Ocular MG cases accounted for 65.6 % childhood MG patients. A positive response was observed in 96.8 % of the patients for neostigmine tests, whereas a positive decremental response to low frequency repetitive nerve stimulation (RNS) was observed in 77.4 % of the patients. The highest stimulating positive rate was 65.3 % in stimulated facial nerve. Thymoma was significantly increased in those patients with severe MG, especially in the cohort involving the respiratory muscles (p < 0.001). The study revealed higher frequency of ocular and childhood MG compared to other studies in USA and European countries, which can be a result of optimum case ascertainment, increased disease duration, or application of complex diagnostic tests. The relative increase in the prevalence of ocular myasthenia can be attributed to the impact of an aging population.

[The clinical efficacy and safety of tacrolimus in patients with myasthenia gravis].

OBJECTIVE: To evaluate the efficacy and safety of tacrolimus in patients with generalized myasthenia gravis (MG). METHODS: A total of 69 cases admitted to our hospital were given 2-6 mg/day tacrolimus (FK506) for 12 months. The MG absolute and relative clinical scores were used to monitor the efficacy of tacrolimus. Clinical evaluation was conducted at month 1, 3, 6, and 12, while the serum concentration of FK506 was measured at one month after administration of tacrolimus for one month. RESULTS: The therapeutic response presenting as improved muscular strength showed within one month after administration of tacrolimus. The overall response rates (MG relative clinical score ≥ 25%) at month 1, 3, 6 were 81.2%, 87.6%, 92.2% respectively. It reached 93.8% by the final visit at month 12. MG score to evaluate disease severity decreased significantly as the subjects continued to take tacrolimus. Statistic analysis suggested that the serum concentration of FK506 was correlated with its therapeutic effect. Serum trough levels in remission and response groups [(7.1 ± 3.9) µg/L and (6.3 ± 3.8) µg/L, respectively] were significantly higher than that of no response group [(3.4 ± 1.3) µg/L]. The most common adverse effects included hyperglycemia (5 cases), myelosuppression (3 cases), and dizziness tinnitus (3 cases), majority of which were temporary and manageable. CONCLUSIONS: Our study has shown that tacrolimus significantly improved muscular strength of generalized MG patients. The treatment is well tolerated. The therapeutic effect of tacrolimus is observed within 1 month after initial use. Adverse events were manageable and not common.

[Thymoma T helper type 17 cells and related cytokines in myasthenia gravis].

OBJECTIVE: To investigate the immunoregulatory role of Th17 cell and the related cytokines in myasthenia gravis. METHODS: Totally 51 myasthenia gravis (MG) patients were divided into MG with thymomas (TM group) and the MG with normal thymus (NT group), as well as 22 healthy subjects as controls. Th17 cells from peripheral blood mononuclear cells were measured by flow cytometry. Th17 related cytokines were detected by ELISA and real-time quantitative-PCR. RESULTS: The quantity of Th17 cells in MG patients with thymomas (1.53 ± 0.59)% were significantly increased compared with that of healthy control (0.94% ± 0.32%, P < 0.05). There was no significant difference in the number of Th17 cells between healthy controls and NT group. The expression levels of IL-17 mRNA (23.7 ± 4.5) were up-regulated significantly versus those in healthy controls (13.4 ± 3.2, P < 0.01). The levels of mRNA expression of IL-1ß, IL-6 and IL-23 were up-regulated significantly in TM group. The mean concentration of IL-17 was up-regulated significantly in TM group (30.4 ± 7.3) ng/L versus healthy controls [(19.2 ± 4.9) ng/L, P < 0.05]. Serum levels of IL-23 and IL-1ß were always increased in TM group versus healthy controls. CONCLUSION: The elevated levels of IL-17 and other Th17 related cytokines in thymomas may aggravate the autoimmunity disorder.

[The clinical characteristics of patients with thymoma-associated myasthenia gravis].

OBJECTIVE: To investigation the clinical characteristics in myasthenia gravis (MG) patients with thymomas. METHODS: A total of 856 MG patients admitted to the department during 2008.7 - 2010.12 were reviewed retrospectively. The patients with MG were divided into two groups based on thymic pathology, which were 162 cases with thymoma and 694 cases without thymoma. We compared the different clinical features including the gender, age of onset, MG symptoms and the incidence rate of myasthenia crisis. And the relationship between the WHO types, Maosaoka stages of thymoma and the severe of MG was also studied. RESULTS: The percentage of thymoma-associated MG patients was 18.9 percent of hospitalized MG patients at the same period. Of the 162 thymoma-associated patients, 94 were male and 68 were female, with a ratio of 1.38:1 and a mean age of (42.9 ± 12.4) years old. Thymoma was more frequent in middle-old aged patients than in children. Compared with non-thymoma MG, more thymomatous patients showed generalised MG, but not only ocular muscles weakness (90.1% vs 62.4%, P < 0.001). There were significant differences of the incidence rate of myasthenic crisis in the two groups (14.8% vs 2.3%). (2) WHO type B2 and Maosaoka I, II thymoma were the commonest types among all potentially MG-associated thymoma. No differences of Osserman MG classification was found in thymomatous patients with different pathologic changes. CONCLUSIONS: The thymomatous MG patients had its distinctive clinical features: thymomas occurred in about 19.7% of MG patients with more men than women, more common in generalized, higher incidence of myasthenia crisis, with B2 type thymic pathology and Maosaoka I, II stages. No correlation was found between pathologic and clinical stages.

Photoredox-catalysed chloro-, bromo- and trifluoromethylthio-trifluoromethylation of unactivated alkenes with sodium triflinate.

A mild and transition-metal-free protocol is herein presented for chloro-, bromo- and trifluoromethylthiotrifluoromethylation of unactivated alkenes. The easy-handling Langlois reagent, as well as N-halophthalimide and N-trifluoromethylthiosaccharin, is used in this method. In the presence of an organic photoredox catalyst N-methyl-9-mesityl acridinium, a broad range of desired products were afforded in satisfactory yields upon visible-light irradiation via a radical process.

CD40 ligand as a potential biomarker for atherosclerotic instability.

OBJECTIVE: The signaling protein CD40 and its ligand, CD40L, are thought to contribute to atherosclerotic plaque formation and rupture. We sought to determine their utility as markers of cerebral atherosclerosis and neurological dysfunction. METHODS: We recruited 82 patients with acute cerebral infarction (ACI) and classified each as having large-artery atherosclerosis (LAA, 30), small-artery occlusion (36), or cardioaortic embolism (16). We also recruited 17 patients who had carotid artery stenosis (CAS) without stroke and 20 healthy individuals as controls. CD40L expression on peripheral blood monocytes (PBMCs) was detected by direct immunofluorescence with flow cytometry, and serum soluble CD40L (sCD40L) was measured by ELISA. RESULTS: CD40L expression on PBMCs was highest in the LAA group, followed by that in the CAS group (both P < 0·01 versus control). It was also higher in patients with atherosclerotic infarction than in those without atherosclerosis (P < 0·05). PBMC CD40L expression was sensitive and specific for detecting atherosclerosis and LAA cerebral infarction and was superior to serum C-reactive protein for predicting cerebral atherosclerosis (P < 0·01). Serum sCD40L was higher in patients with ACI than in healthy controls (P < 0·01) or patients with CAS (P < 0·01) and correlated with increased disability on three scales (all P < 0·01). CONCLUSIONS: We conclude that patients with ACI have an upregulated CD40-CD40L system, which could be used as a clinical biomarker for assessing atherosclerotic instability and severity of neurological dysfunction.

Hemichorea-hemiballismus associated with nonketotic hyperglycemia: a possible role of inflammation.

Three cases of hemichorea-hemiballismus (HC-HB) associated with nonketotic hyperglycemia were reported. Of them two patients presented as HC-HB and the remaining one as generalized chorea-ballismus (CB). Brain MRI showed characteristic T1-weighted high-intensity lesions in the contralateral or bilateral striatum without edema or mass effect. They all had a prior history of respiratory or urinary infection. Cerebrospinal fluid test in two patients showed an elevation of protein concentration with normal cell and an increased IgG content and elevated IgG index or 24 h IgG intrathecal synthesis rate. These results suggested that inflammation within the central nervous system may participate in the pathogenesis of chorea and ballismus induced by NKH.