[Show abstract][Hide abstract] ABSTRACT: To evaluate the efficacy and safety of tacrolimus in patients with generalized myasthenia gravis (MG).
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 tacromus for one month.
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.
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.
No preview · Article · Jul 2013 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
[Show abstract][Hide abstract] ABSTRACT: 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.
No preview · Article · Feb 2013 · Neurological Sciences
[Show abstract][Hide abstract] ABSTRACT: To investigation the clinical characteristics in myasthenia gravis (MG) patients with thymomas.
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.
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.
The thymomatous MG patients had its distinctive clinical features: thymomas occured 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.
No preview · Article · Aug 2012 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
[Show abstract][Hide abstract] ABSTRACT: To investigate the immunoregulatory role of Th17 cell and the related cytokines in myasthenia gravis.
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.
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-1β, 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-1β were always increased in TM group versus healthy controls.
The elevated levels of IL-17 and other Th17 related cytokines in thymomas may aggravate the autoimmunity disorder.
No preview · Article · Jul 2012 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
[Show abstract][Hide abstract] ABSTRACT: To compare the clinical characteristics of early-onset and late-onset myasthenia gravis (MG).
A total of 819 MG patients admitted in our department during the same period were reviewed retrospectively. The patients with MG were divided into two groups by the age of onset, which were the early-onset MG (< 49 years) and late-onset MG (≥ 50 years). Several clinical features were compared in the two groups including the percentage, initial symptoms, MG types, the positive rates of neostigmine test and repetitive nerve stimulation, thyroid function and thymic abnormalities.
The more common onset age of MG was 0 - 49 years (early-onset). In both groups the male and female ratio was closer to 1:1 with the extra ocular muscles weakness presented as the most frequent symptom and Type IIB presented as the most common type in both groups (41.51% and 51.37% respectively in early-onset and late-onset groups). Type I was more common in early-onset group while type IIB was more common in late-onset group (P < 0.05). There was no significant difference in the positive rates of neostigmine test and repetitive nerve stimulation between the two groups. The thyroid function abnormality ratio was higher in early-onset group. Hyperplastic thymus was common in early-onset group (67.57%) while both hyperplastic thymus and thymoma were common in late-onset group (48.68% and 47.37%).
The clinical characteristics of early-onset and late-onset myasthenia gravis (MG) was different in MG type, the positive rate of thyroid function abnormalities and thymic pathologic type.
No preview · Article · Jun 2011 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
[Show abstract][Hide abstract] ABSTRACT: To explore the value of low-frequency repetitive nerve stimulation (RNS) in the diagnosis of myasthenia gravis (MG).
A total of 436 MG patients admitted during the period of July 2008 and August 2010 and undergoing the repetitive nerve stimulation test were reviewed retrospectively. We analyzed the positive rates of RNS in MG patients and compared the abnormalities in different nerves including facial, accessory, axillary and ulnar nerves.
Among them, 73.85% had abnormal recordings on low-frequency RNS test. The highest abnormality was in facial nerves (82.30%), then axillary nerves (52.17%) and the lowest in ulnar nerves (27.64%). The positive rates of RNS in ocular MG were significant lower than those in general MG patients. And there were no significant statistical differences of RNS abnormal rates in types IIa, IIb, III and IV MG patients with 89.66%, 82.56%, 91.67% and 83.33%, respectively. In ocular MG, 16.34% patients were positive in RNS test under the stimulation of accessory, axillary and ulnar nerves. And 79.50% patients with general MG had two or more nerves with abnormal results.
The low-frequency RNS test is of great value in the diagnosis of MG. And it shows a higher sensitivity in general MG patients than that in ocular ones. The stimulation of facial and axillary nerves increases the positive rates of RNS in MG patients.
No preview · Article · May 2011 · Zhonghua yi xue za zhi
[Show abstract][Hide abstract] ABSTRACT: To analyze the clinical characteristics of myasthenia gravis (MG) associated with thyroid abnormalities.
A total of 300 MG patients admitted to the department of neurology from July 2008 to September 2009 were reviewed retrospectively. Based on the thyroid function and abnormality of thyroid related antibodies (thyroglobulin antibody and peroxidase antibody), the patients with MG were divided into two groups (260 cases without thyroid disease and 40 cases with thyroid abnormalities). The different clinical features, the relationship between the antithyroid antibodies and anti-acetylcholine receptor antibody (AChRAb) and the distribution of T cell subsets between the two groups were compared.
(1) Among the 300 MG patients, 13.3% of them was accompanied with thyroid disease and the most common abnormality was positive thyroid antibody. (2) Between the groups of MG with and without thyroid abnormalities, no significant differences on the sex percentage, age of onset, duration of disease, clinical classification and thymic abnormalities were found (P>0.05). (3) The blood levels of AChRAb in postsynaptic membrane in the thyroid antibody-positive patients were 1.15±0.11, being much higher than those in the antibody-negative patients (1.01±0.11). (4) The percentage of CD8+ T cells was significantly decreased [(21.63±5.17)% vs (24.28±5.79)%] and the ratio of CD4+/CD8+ T cells was increased (2.10±0.67 vs 1.81±0.61, P<0.05) in the group of MG with thyroid abnormality as compared with the group of MG with normal thyroid.
In MG patients the incidence positive thyroid related antibodies was much higher than that of other thyroid abnormalities. It is suggested that there are higher levels of AChRAb in MG coexisting with positive thyroid antibodies and more abnormalities of T lymphocyte subset distribution in MG patients with thyroid disease.
No preview · Article · Jul 2010 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]