Prevalence, clinical features and treatment outcomes of patients with myasthenia gravis positive for antibodies to muscle-specific kinase in Thailand

Witoonpanich, Rawiphan; Dejthevaporn, Charungthai; Pulkes, Teeratorn; Tunlayadechanont, Supoch; Boonkongchuen, Pairoj; Pongpakdee, Sunsanee; Vincent, Angela

doi:10.1016/j.jocn.2012.03.047

Cited by 5 publications

(7 citation statements)

References 19 publications

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“…6,30 In the current study, 10.25% Clinical features peculiar to MUSK-antibody MG include focal involvement of facial, bulbar, neck and respiratory muscles, lack of thymic abnormalities, worse outcome, lower remission rates, poor response to cholinesterase inhibitors and immunosuppressants and good response to plasmapheresis. 37 Tsonis et al, in a multi-national study, reported a relatively mild disease, with majority responding to treatment. 37 Tsonis et al, in a multi-national study, reported a relatively mild disease, with majority responding to treatment.…”

Section: Discussionmentioning

confidence: 98%

“…37 Tsonis et al, in a multi-national study, reported a relatively mild disease, with majority responding to treatment. 30,37 Antibodies against intracellular components of striated muscle viz titin and ryanodine receptor (RYR) also play a role in governing the disease severity. 30,37 Antibodies against intracellular components of striated muscle viz titin and ryanodine receptor (RYR) also play a role in governing the disease severity.…”

Section: Discussionmentioning

confidence: 98%

“…4,5,20,30,34,35 Outcome in MUSK-antibody MG is poor compared to AChR-antibody MG. 20,27,36 In contrast, a good response to treatment in all MUSK-antibody MG was reported among the Thai-Chinese. 37 Tsonis et al, in a multi-national study, reported a relatively mild disease, with majority responding to treatment. 10 In the current study, 62.5% (5/8) of patients with MUSK-antibody MG had one or more episodes of myasthenic crisis.…”

Section: Discussionmentioning

confidence: 98%

“…10 Our patients of MUSK-antibody MG improved following thymectomy, and previous studies also have reported improvement or remission in a significant proportion of subjects who underwent thymectomy, but the caveat was that beneficial effect of concomitantly administered immunosuppressants could not be ruled out. 30,37 Antibodies against intracellular components of striated muscle viz titin and ryanodine receptor (RYR) also play a role in governing the disease severity. 4 Titin antibodies are directed against a 30 kDa segment which is considered to be the main immunogenic region within the large protein.…”

Section: Discussionmentioning

confidence: 99%

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Autoantibodies in acquired myasthenia gravis: Clinical phenotype and immunological correlation

et al. 2019

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Background: Data on antibody profile in myasthenia gravis (MG) from India are limited. Objectives: To investigate antibody profile in patients with MG and their clinical correlates. Patients and Methods: Patients of MG (n = 85, M:F::1.1:1, mean age: 39.29 ± 17.3 years, mean symptom duration: 72.94 ± 91.8 months) were evaluated for clinical features, MG foundation of America (MGFA) score, response to treatment, and outcome at last follow-up. Antibodies to acetylcholine receptor (AChR), muscle-specific kinase (MUSK), titin and ryanodine receptor (RYR) were analysed using ELISA.Results: Based on the regional distribution of weakness, the cohort could be categorized as: generalized: 60, ocular: 16 and oculo-bulbar: 9. Sixty patients were followed up for a mean duration of 26.74 ± 13.8 months. Outcome at last follow-up was as follows: remission-22, no remission-33 and dead-5. AChR and MUSK antibodies were detected in 58 and 8 patients, respectively. Frequency of generalized MG, worse MGFA score during the disease course and thymomatous histology significantly correlated with presence of AChR-antibodies, though outcome at last followup was comparable between AChR-antibody positive and negative groups. Patients with MUSK antibodies had oculo-bulbar or generalized MG and frequent respiratory crisis, but majority improved or remitted with treatment. Titin antibodies were detected in 31.8% and RYR antibodies in 32.9%. Their presence did not correlate with age at onset of MG, severity or presence of thymoma. Conclusion: This report highlights the spectrum of antibodies in MG in an Indian cohort. AChR-antibody positivity correlated with clinical severity. Outcome was good in majority of MUSK antibody-positive MG. The role of other antibodies, complementary vs epiphenomenon, remains open. K E Y W O R D S acetylcholine receptor, muscle-specific kinase, myasthenia gravis, ryanodine receptor, titin | 429 NAGAPPA et Al.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

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Autoantibodies in acquired myasthenia gravis: Clinical phenotype and immunological correlation

et al. 2019

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“…MuSK‐MG treatment has been the object of recent studies . On the whole, they agree on the disease being difficult‐to‐treat, though the current prognosis appears to be more favorable than in earlier reports, owing to the extensive use of aggressive immunosuppression and the availability of effective treatment for refractory disease.…”

Section: Diseasesmentioning

confidence: 93%

Characteristics of myasthenia gravis with antibodies to muscle‐specific kinase and low‐density lipoprotein‐related receptor protein 4

Evoli

Iorio

2015

Clinical & Exp Neuroim

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Myasthenia gravis (MG) is characterized by fatigable weakness of voluntary muscles caused by immunoglobulin G autoantibodies binding to post‐synaptic proteins at the neuromuscular junction. Antibodies specific for the acetylcholine receptor are detected in the great majority (85%) of MG patients. Serum immunoglobulin G binding to the muscle‐specific kinase (MuSK) are found in approximately 40% of anti‐acetylcholine receptor‐negative patients. Recently, the low‐density lipoprotein receptor‐related protein 4 (Lrp4), which is the MuSK co‐receptor for agrin, has been recognized as a new antigen in MG. Specific antibodies bind to the extracellular domains of MuSK and Lrp4 and, as experimental models have shown, interfere with the protein function. The present review describes the MuSK‐Lrp4 complex, and summarizes the effects of MuSK and Lrp4 antibodies at the neuromuscular junction. Furthermore, the epidemiological and clinical characteristics, the response to therapy and the controversies in management of the specific diseases are discussed.

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Juvenile and Neonatal Myasthenia Gravis

Kang

Wkm.

Oskoui

et al. 2015

Neuromuscular Disorders of Infancy, Childhood, and Adolescence

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Prevalence, clinical features and treatment outcomes of patients with myasthenia gravis positive for antibodies to muscle-specific kinase in Thailand

Cited by 5 publications

References 19 publications

Autoantibodies in acquired myasthenia gravis: Clinical phenotype and immunological correlation

Autoantibodies in acquired myasthenia gravis: Clinical phenotype and immunological correlation

Characteristics of myasthenia gravis with antibodies to muscle‐specific kinase and low‐density lipoprotein‐related receptor protein 4

Juvenile and Neonatal Myasthenia Gravis

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