Double‐seropositive myasthenia gravis

Zouvelou, Vasiliki; Kyriazi, Stavroula; Rentzos, Michael; Belimezi, Maria; Micheli, Maria-Angeliki; Tzartos, Socrates J.; Stamboulis, Eleftherios

doi:10.1002/mus.23645

Cited by 16 publications

(11 citation statements)

References 9 publications

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“…In fact, MuSK antibodies have been shown to act, at least in part, by preventing the MuSK-LRP4 interaction (Huijbers et al, 2013;Koneczny et al, 2013), suggesting that they target epitopes close to or at the interacting regions. On the other hand, MuSK/AChR double positives have rarely been reported (Ohta et al, 2004;Poulas et al, 2012;Zouvelou et al, 2013). However, the MuSK CBA detected a higher than expected rate of AChR-MG patients with concomitant presence of MuSK antibodies.…”

Section: Discussionmentioning

confidence: 84%

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: Discussionmentioning

confidence: 84%

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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“…MuSK‐Abs were tested subsequently, mostly because of a change in the disease course . Double antibody positivity has also been described in untreated patients or induced by penicillamine . Occasionally, patients may also have antibodies to both MuSK and LRP4 .…”

Section: Muscle‐specific Tyrosine Kinase Antibodymentioning

confidence: 99%

“…Currently in the literature, at least 19 patients have been reported to be double positive for AChR‐Abs and MuSK‐Abs, 12 of whom received thymectomy prior to MuSK‐Ab testing . In 2 patients, MuSK‐Abs were tested prior to thymectomy, and both were negative .…”

Section: Treatmentmentioning

confidence: 99%

Myasthenia gravis with muscle‐specific tyrosine kinase antibodies: A narrative review

Morren

2018

Muscle and Nerve

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Growing evidence provides new insights about myasthenia gravis (MG) with antibodies against muscle-specific tyrosine kinase (MuSK-MG), including its pathogenesis, clinical and electrophysiological manifestations, and treatment. Data now support the presence of both presynaptic and postsynaptic dysfunction in MuSK-MG. This is 1 of many key differences between MuSK-MG and acetylcholine receptor antibody-MG (AChR-MG), especially as it pertains to potential therapeutic implications. In comparison to AChR-MG, MuSK-MG is generally more refractory to treatment. However, because MuSK-MG is better understood and more readily recognized today, there are more reports of a relatively benign course. The most effective immunotherapies for MuSK-MG are corticosteroids, plasmapheresis, and rituximab. With appropriate therapy, most patients with MuSK-MG achieve minimal manifestation status or better on the postintervention status outlined by the Myasthenia Gravis Foundation of America. A minority of patients remain refractory to treatment, and optimal management for this group remains a considerable challenge. Muscle Nerve 58: 344-358, 2018.

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“…Vocal cord paralysis is seen in some patients with MuSK antibody-positive MG 91–93 . While most patients just have anti-MuSK antibodies, patients with antibodies to MuSK and ACHR 94,95 and voltage gated calcium channels (VGCC) 96,97 are described. Anti-MuSK antibody level is generally correlated with symptoms 98 .…”

Section: Musk Antibody-positive Mgmentioning

confidence: 99%

Muscle-Specific Tyrosine Kinase and Myasthenia Gravis Owing to Other Antibodies

et al. 2018

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Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors. Other antibodies associated with myasthenia gravis, including low-density lipoprotein receptor-related protein 4, are discussed.

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Double‐seropositive myasthenia gravis

Cited by 16 publications

References 9 publications

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

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

Myasthenia gravis with muscle‐specific tyrosine kinase antibodies: A narrative review

Muscle-Specific Tyrosine Kinase and Myasthenia Gravis Owing to Other Antibodies

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