1.Management of advanced thymoma presenting with myasthenia gravis in a resource-limited setting: A case report
I Wayan Losen Adnyana ; Dian Daniella
Acta Medica Philippina 2024;58(Early Access 2024):1-6
Thymomas are rare tumours which generally account for only 0.2 – 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities.
A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient’s motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness.
The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the
importance of multidisciplinary management involving oncologists, surgeons, and neurologists.
Thymoma
;
Myasthenia Gravis
;
Drug Therapy
;
Thymectomy
2.Clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis: a retrospective analysis.
Rui-Yan WANG ; Hui CHEN ; Zhi-Xin HUANG ; Yong CHEN ; Jian-Min ZHONG
Chinese Journal of Contemporary Pediatrics 2023;25(10):1034-1039
OBJECTIVES:
To investigate the clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis (OMG).
METHODS:
A retrospective analysis was conducted on 130 children with OMG who were treated in the Department of Neurology, Jiangxi Children's Hospital, from February 2018 to February 2023. According to the treatment regimen, they were divided into four groups: glucocorticoid (GC) group (n=29), mycophenolate mofetil (MMF) group (GC+MMF; n=33), methotrexate (MTX) group (GC+MTX; n=30), and tacrolimus (FK506) group (GC+FK506; n=38). Treatment outcomes and adverse reactions were compared among the groups.
RESULTS:
After 3 months of treatment, the FK506 group had significantly lower scores of Myasthenia Gravis Quantitative Scale and Myasthenia Gravis-Specific Activities of Daily Living than the other three groups (P<0.05). After 3 months of treatment, the FK506 group had a significantly lower dose of prednisone than the GC group, and after 6 and 9 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower dose of prednisone than the GC group (P<0.05). After 12 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower incidence rate of GC-related adverse reactions than the GC group (P<0.05).
CONCLUSIONS
For children with OMG, the addition of various immunosuppressants can reduce the dosage of GC and adverse reactions. Among them, FK506 shows superior efficacy compared to other immunosuppressants in the early treatment of OMG.
Humans
;
Child
;
Prednisone/adverse effects*
;
Tacrolimus/adverse effects*
;
Retrospective Studies
;
Activities of Daily Living
;
Immunosuppressive Agents/adverse effects*
;
Myasthenia Gravis/drug therapy*
;
Glucocorticoids/therapeutic use*
;
Mycophenolic Acid/adverse effects*
3.Acupuncture combined with western medication for ocular myasthenia gravis: a randomized controlled trial.
Xian-Peng XU ; Yan-Bing JIANG ; Li-Hua GUAN ; Qing-Jie JI ; Ying JIN
Chinese Acupuncture & Moxibustion 2022;42(7):755-759
OBJECTIVE:
To compare the clinical efficacy between acupuncture combined with western medication and simple western medication for ocular myasthenia gravis (OMG), and to explore its possible mechanism.
METHODS:
A total of 60 patients of ocular myasthenia gravis were randomized into an acupuncture combined with western medication group (30 cases, 1 case dropped off) and a western medication group (30 cases, 2 cases dropped off). Oral pyridostigmine bromide tablet and prednisone acetate tablet were given in the western medication group. On the basis of the treatment in the western medication group, Tongdu Tiaoqi acupuncture (acupuncture for unblocking the governor vessel and regulating qi ) was applied at Baihui (GV 20), Fengfu (GV 16), Hegu (LI 4), Zusanli (ST 36), etc. in the acupuncture combined with western medication group, once a day, 6 days a week. The treatment was given 8 weeks in both groups. Before and after treatment, the OMG clinical absolute score was observed, electrophysiological indexes of orbicularis oculi (value of mean jitter, percentage of jitter >55 μs and percentage of blocks) were measured by single-fiber electromyography (SFEMG), serum levels of acetylcholine receptor antibody (AChR-Ab), interferon-gamma (IFN-γ) and interleukin-4 (IL-4) were detected by ELISA method.
RESULTS:
After treatment, the OMG clinical absolute scores, values of mean jitter, percentages of jitter >55 μs, percentages of blocks and serum levels of AChR-Ab, IFN-γ and IL-4 were decreased compared before treatment in both groups (P<0.05), and those in the acupuncture combined with western medication group were lower than the western medication group (P<0.05).
CONCLUSION
Acupuncture combined with western medication can effectively improve ptosis, palpebra superior fatigability, eye movement disorder and neuromuscular junction dysfunction in patients with ocular myasthenia gravis, the therapeutic effect is superior to simple western medication. Its mechanism may be related to down-regulating serum levels of AChR-Ab, IFN-γ and IL-4 and promoting the recovery of orbicularis oculi function.
Acupuncture Therapy
;
Facial Muscles
;
Humans
;
Interferon-gamma
;
Interleukin-4
;
Myasthenia Gravis/drug therapy*
4.Clinical effect of tacrolimus in the treatment of myasthenia gravis in children.
Jiu-Wei LI ; Fang FANG ; Xiao-Tun REN ; Wei-Hua ZHANG ; Xin-Ying YANG ; Chang-Hong REN ; Shuai GONG ; Jun-Lan LYU ; Xiao-Hui WANG ; Xu WANG ; Hu-Sheng WU ; Chang-Hong DING
Chinese Journal of Contemporary Pediatrics 2020;22(9):964-969
OBJECTIVE:
To evaluate the efficacy and safety of tacrolimus in the treatment of children with myasthenia gravis (MG).
METHODS:
A total of 28 children with MG were treated with tacrolimus. MG-Activities of Daily Living (MG-ADL) scale was used to assess clinical outcome and safety after 1, 3, 6, 9, and 12 months of treatment.
RESULTS:
After tacrolimus treatment, the MG-ADL score at 1, 3, 6, 9 and 12 months was lower than that at baseline (P<0.05), and the MG-ADL score showed a gradually decreasing trend. The response rates to tacrolimus treatment at 1, 3, 6, 9, and 12 months were 59%, 81%, 84%, 88%, and 88% respectively. At 6, 9, 12, and 18 months of treatment, 4, 13, 14, and 15 children respectively were withdrawn from prednisone. No recurrence was observed during treatment. Major adverse reactions/events were asymptomatic reduction in blood magnesium in 5 children and positive urine occult blood in 1 child, which turned negative without special treatment, and tacrolimus was not stopped due to such adverse reactions/events. One child was withdrawn from tacrolimus due to recurrent vomiting. According to CYP3A5 genotypes, all of the patients were divided into two groups: slow metabolic type (n=19) and non-slow metabolic type (fast metabolic type + intermediate type; n=9). The non-slow metabolism group received a higher dose of tacrolimus, but had a lower trough concentration of tacrolimus than the slow metabolism group (P<0.05). The slow metabolism group had a higher response rates to tacrolimus treatment than the non-slow metabolism group (P<0.05).
CONCLUSIONS
Tacrolimus appears to be effective and safe in the treatment of children with MG and is thus an option for immunosuppressive therapy. CYP3A5 genotyping has a certain guiding significance for determining the dosage of tacrolimus.
Activities of Daily Living
;
Child
;
Humans
;
Immunosuppressive Agents
;
Myasthenia Gravis
;
drug therapy
;
Neoplasm Recurrence, Local
;
Tacrolimus
;
therapeutic use
5.Acupuncture combined with Qigui decoction for 30 cases of myasthenia gravis.
Chinese Acupuncture & Moxibustion 2014;34(7):718-718
Acupuncture Therapy
;
Adolescent
;
Adult
;
Aged
;
Drugs, Chinese Herbal
;
administration & dosage
;
Female
;
Humans
;
Male
;
Middle Aged
;
Myasthenia Gravis
;
drug therapy
;
therapy
;
Young Adult
6.Clinical efficacy of Yiqi Qushi Recipe in treating myasthenia gravis and observation of its immunomodulatory effects.
Chinese Journal of Integrated Traditional and Western Medicine 2013;33(2):177-179
OBJECTIVETo observe the efficacy of Yiqi Qushi Recipe (YQR) in treating myasthenia gravis (MG) patients and its effects on their immune functions.
METHODSRecruited were 40 type I and II MG patients from clinics and wards of the Affiliated Hospital of Shandong University of Traditional Chinese Medicine from January 2009 to June 2011. They were randomly assigned to the treatment group (20 cases) and the control group (20 cases). Patients in the treatment group took YQR, one dose daily, while those in the control group took pyridostigmine 60 mg, three times a day. The therapeutic course consisted of eight weeks. The clinical efficacy, immunization indicators before and after treatment were observed. Meanwhile, the safety evaluation was performed.
RESULTSThe cured and effective rate was 75% and the total effective rate was 95% in the treatment group. They were 45% and 85% in the control group. Better results were obtained in the treatment group. Compared with the same group before treatment, IgA, IgG, and CD8 increased, IgM, CD4, and CD4/CD8 decreased in the treatment group, showing statistical difference (P < 0.05). There was no obvious change in each index of the control group after treatment (P > 0.05). Compared with the control group after treatment, IgA, IgG, and CD8 increased, CD4 and CD4/CD8 decreased in the treatment group, showing statistical difference (P < 0.05). During the course of treatment, mild diarrhea, nausea, and vomit occurred in two patients of the control group, while no adverse reaction occurred in those of the treatment group.
CONCLUSIONYQR could significantly improve clinical symptoms of MG patients, regulate their immune functions, with no obvious adverse reaction.
Adult ; CD4-CD8 Ratio ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Immunoglobulin A ; immunology ; Immunoglobulin G ; immunology ; Male ; Middle Aged ; Myasthenia Gravis ; drug therapy ; immunology ; Phytotherapy ; Young Adult
7.Myasthenia Gravis, Schizophrenia, and Colorectal Cancer in A Patient: Long-Term Follow-Up with Medication Complexity.
Haebin KIM ; Minha HONG ; Geon Ho BAHN
Psychiatry Investigation 2013;10(3):300-302
In a case of 46-year-old woman suffering from schizophrenia for over 20 years, she experienced frequent episodes of dyspnea and confirmed as superimposed with myasthenia gravis (MG). Throughout the seven-year follow-up period, after diagnosed as MG, she has been hospitalized 6 times and also diagnosed as colorectal cancer. Authors experienced various conditions associated with untoward effects of medication for myasthenia, schizophrenia, and colorectal cancer. Therefore, authors reported considerations for the pharmacotherapy of schizophrenia with myasthenia gravis.
Colorectal Neoplasms*
;
Drug Therapy
;
Dyspnea
;
Female
;
Follow-Up Studies*
;
Humans
;
Middle Aged
;
Myasthenia Gravis*
;
Schizophrenia*
8.Role of miR-155 in myasthenia gravis and effect of dexamethasone on miR-155.
Xiaoli CHEN ; Yuqian CHEN ; Yuzhong WANG ; Mei YAN ; Junmei ZHANG ; Qun LIU ; Huan YANG ; Jing LI
Journal of Central South University(Medical Sciences) 2012;37(8):777-782
OBJECTIVE:
To determine the role of miR-155 in the pathogenesis of generalized myasthenia gravis (GMG) and the effect of dexamethasone (DXM) on miR-155.
METHODS:
The expression of miR-155 in B cells from the GMG patients and healthy controls was analyzed by qPCR. The B cells were cultured with DXM and PBS. The B cell proliferation was examined by MTT; CD80 and CD86 frequencies were detected by flow cytometry; and anti-AChRIgG and isotypes anti-AChR-IgG1, 2, 3 in the supernatant were detected by ELISA.
RESULTS:
qPCR revealed that the expression of miR-155 in the B cells was much higher than that in the controls, and the miR155 expression decreased after DXM treatment. flow cytometry showed that there was no significant difference in the proliferation and the expressions of CD80 and CD86 in the B cells between the DXM group and the PBS group. The concentration of anti-AChR-IgG1 was obviously lower in the DXM group than in the PBS group, but the concentration of anti-AChRIgG, anti-AChR-IgG2, and anti-AchR-IgG3 was similar.
CONCLUSION
high expression of miR-155 may be associated with myasthenia gravis progression. DXM may disturb the antibody class switch of B cells by suppressing the expression of miR-155 and improve the symptom of MG patients.
Adult
;
B-Lymphocytes
;
cytology
;
immunology
;
metabolism
;
B7-1 Antigen
;
metabolism
;
Cell Proliferation
;
Cells, Cultured
;
Dexamethasone
;
therapeutic use
;
Female
;
Humans
;
Immunoglobulin G
;
immunology
;
Male
;
MicroRNAs
;
genetics
;
metabolism
;
Middle Aged
;
Myasthenia Gravis
;
drug therapy
;
genetics
;
immunology
;
Receptors, Cholinergic
;
immunology
;
Tetraspanin 28
;
metabolism
;
Young Adult
9.Comparison of Clinical Manifestations between Patients with Ocular Myasthenia Gravis and Generalized Myasthenia Gravis.
Hyun Seok ROH ; Sang Yeul LEE ; Jin Sook YOON
Korean Journal of Ophthalmology 2011;25(1):1-7
PURPOSE: To compare the clinical manifestations between patients with ocular myasthenia gravis and those with generalized myasthenia gravis (MG). METHODS: The medical records of 71 patients diagnosed with MG between January 1995 and December 2007 were reviewed. Demographics, sensitivities of diagnostic methods, the presence of systemic autoimmune diseases, ophthalmic complications caused by MG, and treatments were evaluated and compared. RESULTS: Fourteen patients (20%) were diagnosed with ocular MG and 57 patients (80%) with generalized MG. Sensitivities of anti-acetylcholine receptor antibody and repetitive nerve stimulation tests were significantly higher in the generalized MG group (84%, 89%) compared to those in the ocular MG group (50%, 54%) (p = 0.011, p = 0.008). The sensitivity of the neostigmine test was the highest in both groups (98% of generalized MG, 79% of ocular MG), and the difference between the two groups was borderline significant (p = 0.058). The most common symptoms were ptosis and diplopia, and both groups presented with pain, blurred vision, and tearing. Systemic autoimmune disease was more prominent in the generalized MG group (21%) than in the ocular MG group (14%), and steroid therapy was used more frequently in the generalized MG group (82%) than in the ocular MG group (57%). Ophthalmic complications associated with long-term steroid treatment were more profound in the generalized MG (30%) compared to those of the ocular MG (21%). CONCLUSIONS: The generalized MG group was associated with higher sensitivities to diagnostic tests, more systemic steroid use, higher ophthalmic complications caused by systemic autoimmune disease, and long-term steroid treatment compared to those of the ocular MG group.
Adolescent
;
Adult
;
Aged
;
Aged, 80 and over
;
Autoimmune Diseases/complications
;
Child
;
Drug Administration Schedule
;
Eye Diseases/chemically induced/immunology
;
Female
;
Humans
;
Male
;
Middle Aged
;
Myasthenia Gravis/complications/*diagnosis/*drug therapy
;
Steroids/*administration & dosage/adverse effects
;
Young Adult
10.Clinical study of strengthening pi and nourishing shen therapy combined with Western medicine on patients with glucocorticoid resistant myasthenia gravis.
Xiao-yun LIU ; Song-lin CHEN ; Wei-xi ZHANG
Chinese Journal of Integrated Traditional and Western Medicine 2010;30(3):271-274
OBJECTIVETo study the effect of strengthening Pi and nourishing Shen therapy (SPNST) in treating patients with glucocorticoid resistant myasthenia gravis (GR-MG).
METHODSTwenty-seven patients with MG were enrolled, who were relapse cases after treated by cholinesterase inhibitor with systemic glucocorticoid treatment and showed resistance to glucocorticoid. All were treated by Western medicines, methylprednisolone (MP) and pyridostigmine bromide (PSB), together with Chinese medicine (CM) given according to their syndrome types, namely, for the 15 patients of Pi-Shen qi-yin deficiency type, Buzhong Yiqi Pill and Liuwei Dihuang Pill, and for the 12 patients of Pi-Shen yang-deficiency type, Buzhong Yiqi Pill and Zishen Yutai Pill. The dosages of medicines were reduced gradually in MP-PSB-CM order along with the progressing of the therapy in 4 stages (symptom curing, choline receptor restoration, immune regulation, and functional strengthening). Muscle strength and overall state of patients were re-examined before and after each of the 4 stages.
RESULTSAfter 1-year treatment, the therapeutic effect in 9 patients was judged as completely remitted; in 7 as remitted with continuous medication; in 5, 1 and 3 as significantly improved, moderately improved and unchanged respectively, while 2 patients died.
CONCLUSIONIntegrative medicine shows definite effects in treating GR-MG, and it is worthy of further studying.
Adolescent ; Adult ; Aged ; Drug Resistance ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Integrative Medicine ; methods ; Male ; Middle Aged ; Myasthenia Gravis ; drug therapy ; Yin Deficiency ; drug therapy ; Young Adult


Result Analysis
Print
Save
E-mail