Prepubertal anti-Musk positive myasthenia gravis with long remission

Güngör-Tunçer, Özlem; Orhan, Elif Kocasoy; Yılmaz, Vuslat; Parman, Yeşim; Oflazer, Piraye; Saruhan‐Direskeneli, Güher; Deymeer, Feza

doi:10.1016/j.nmd.2013.07.012

Cited by 5 publications

(2 citation statements)

References 21 publications

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“…At that age, it is often with atypical presentation, so it could be easily misdiagnosed, as was in the presented patient. Also, another charactestic of childhood onset MuSK MG is the presence of spontaneous and some time long remissions, present also in our patient and other published cases [3][4][5] . On the other hand, such spontaneous remissions are extremly rare in adult onset MG associated with anti-MuSK antibodies.…”

Section: Discussionsupporting

confidence: 85%

A misdiagnosed myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies with possible childhood onset

et al. 2015

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Section: Discussionsupporting

confidence: 85%

A misdiagnosed myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies with possible childhood onset

et al. 2015

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“…Similarly to adult patients, most pediatric patients with MuSK‐MG are female . Prepubertal patients with MuSK‐MG with long‐term remission without treatment have been frequently reported, and these patients usually have predominantly oculobulbar features . Spontaneous improvement in childhood MuSK‐MG could be related to favorable developmental changes in NMJs; otherwise, this is currently poorly understood.…”

Section: Special Populationsmentioning

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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