Complete stable remission and autoantibody specificity in myasthenia gravis

Baggi, Fulvio; Andreetta, Francesca; Maggi, Lorenzo; Confalonieri, Paolo; Morandi, Lucia; Salerno, Franco; Bernasconi, Pia; Montomoli, Cristina; Barberis, Massimo; Mantegazza, Renato; Antozzi, Carlo

doi:10.1212/wnl.0b013e31827b907b

Cited by 58 publications

(71 citation statements)

References 22 publications

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“…Furthermore, the effectiveness of thymectomy is questioned in MuSK-MG (Evoli et al, 2008), while thymic involvement in its pathogenesis is uncertain. However our results show a considerable 23% of CBA positive patients with thymic hyperplasia suggesting that thymic alterations may be more common in MuSK-MG than currently assumed (Baggi et al, 2013).…”

Section: Discussioncontrasting

confidence: 50%

“…Due to limited information with respect to the maximum severity of symptoms it remains uncertain whether these patients have an overall milder disease manifestation (Baggi et al, 2013). Nevertheless, much fewer of the MuSK-CBA positive patients (4.6%) presented with myasthenic crises compared to the 25-48% reported for typical AChR-MG and MuSK-MG (Oh, 2009;Evoli and Padua, 2013).…”

Section: Discussionmentioning

confidence: 99%

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MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

Tsonis

Zisimopoulou

Lazaridis

et al. 2015

Journal of Neuroimmunology

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Seronegative myasthenia gravis (MG) presents a serious gap in MG diagnosis and understanding. We applied a cell based assay (CBA) for the detection of muscle specific kinase (MuSK) antibodies undetectable by radioimmunoassay. We tested 633 triple-seronegative MG patients' sera from 13 countries, detecting 13% as positive. MuSK antibodies were found, at significantly lower frequencies, in 1.9% of healthy controls and 5.1% of other neuroimmune disease patients, including multiple sclerosis and neuromyelitis optica. The clinical data of the newly diagnosed MuSK-MG patients are presented. 27% of ocular seronegative patients were MuSK antibody positive. Moreover, 23% had thymic hyperplasia suggesting that thymic abnormalities are more common than believed.

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Section: Discussioncontrasting

confidence: 50%

Section: Discussionmentioning

confidence: 99%

MuSK autoantibodies in myasthenia gravis detected by cell based assay — A multinational study

Tsonis

Zisimopoulou

Lazaridis

et al. 2015

Journal of Neuroimmunology

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“…Our results were quite close to those of other large series [7, 8, 23]. However, our CSR percentage of 0.02% was much lower than the percentages given in previous studies [7, 9, 10, 23], probably because of our clinical practice of maintaining low dose CSs in distinct subgroups, including MuSK-MG.…”

Section: Discussionsupporting

confidence: 85%

“…Despite the challenges, about one half of the patients were reported to have a favorable outcome (MM or better according to the Myasthenia Gravis Foundation of America (MGFA) post-intervention scale [5]) at final follow-up, a percentage not different from that in anti-acetylcholine receptor (AChR) antibody positive MG [6-8]. However, complete stable remission (CSR) was observed to be lower [7, 9, 10] and the amount of required maintenance doses to be higher in MuSK-MG [6]. …”

Section: Introductionmentioning

confidence: 99%

Prompt Response to Prednisone Predicts Benign Course in MuSK-MG

Güngör-Tunçer¹,

Yılmaz²,

Toker³

et al. 2017

Eur Neurol

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Background: The difficult course of patients with myasthenia gravis (MG) with anti-muscle-specific tyrosine kinase antibodies (MuSK) has been emphasized. However, no clear information is available on patients who have a benign course. Methods: This study was aimed at comparing patients with favorable (minimal manifestations [MM] or better) and unfavorable outcomes to determine whether excellent response to corticosteroid (CS) treatment within 3 months (good response-3 months) has any predictive effect on the prognosis. Results: Forty-six percent of 46 patients had a favorable outcome at year 3 and 54% at final follow-up. The major finding of this study was its high predictive value with good response-3 months. Those with good response-3 months had significantly more favorable outcome as compared to those without at year 3. The positive predictive value of good response-3 months was high (89% at year 3 and 84% at final follow-up). The negative predictive value diminished from 85% at year 3 to 67% at final follow-up due to increasing number of patients improving in the long run. Overall, 33% of the patients had a benign course with good response-3 months and no major exacerbations until the end of follow-up. Conclusions: Excellent response to CSs within 3 months appears to predict a favorable outcome in MuSK-MG.

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“…Thymectomy does appear to have a positive impact in double-seronegative patients (7), an observation that may reflect the likelihood that many of these patients actually harbor anti-acetylcholine receptor antibodies when tested with cell-based assays that cluster the receptor, mimicking the architecture of the postsynaptic membrane (9,10). In most reports, thymectomy in MuSK-antibody positive patients seems to have little if any favorable impact (7,8), providing further emphasis to the need to individualize management approaches for patients with MG.…”

Section: To the Editormentioning

confidence: 99%