Pure ocular anti‐musk myasthenia under no immunosuppressive treatment

Zouvelou, Vasiliki; Παπαθανασίου, Αθανάσιος; Koros, Christos; Rentzos, Michael; Stamboulis, Eleftherios

doi:10.1002/mus.23847

Cited by 16 publications

(16 citation statements)

References 14 publications

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“…Decreasing muscle responses to a series of low frequency repetitive nerve stimuli were observed at the orbicularis oculi in patient 6, and at the nasalis, orbicularis oculi and deltoid in patient 7 . Therefore, myasthenic involvement was not limited to the ocular muscles of patient 7 . Edrophonium tests were positive in two of four patients tested (50%).…”

Section: Resultsmentioning

confidence: 86%

“…Patient 7 had pure fluctuating ocular symptoms and signs for 3.5 years after the onset, which were classified as ocular MG. However, the myasthenic involvement might not be limited to the ocular muscles, because decreasing muscle responses to a series of low frequency repetitive nerve stimuli were observed at the deltoid muscles . Spontaneous clinical remission with decreased antibody titers was another atypical finding.…”

Section: Resultsmentioning

confidence: 99%

“…27,28 Therefore, myasthenic involvement was not limited to the ocular muscles of patient 7. 28 Edrophonium tests were positive in two of four patients tested (50%).…”

Section: Literature Reviewmentioning

confidence: 93%

“…The data from all nine patients (seven published cases and our two cases) are collated in Table 1. [22][23][24][25][26][27][28] The MuSK-OMG patients consisted of three men and four women; the mean age at onset was 35.0 AE 17.3 years, and the mean disease duration (from ocular MG onset to report) was 32.0 AE 16.4 months. All patients had ptosis, and six patients (85.7%) had diplopia or ophthalmoplegia.…”

Section: Literature Reviewmentioning

confidence: 99%

“…However, the myasthenic involvement might not be limited to the ocular muscles, because decreasing muscle responses to a series of low frequency repetitive nerve stimuli were observed at the deltoid muscles. 28 Spontaneous clinical remission with decreased antibody titers was another atypical finding. In cases reported by Hanisch et al and Hosaka et al, antibody titers declined along with improvements of clinical symptoms.…”

Section: Literature Reviewmentioning

confidence: 99%

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Ocular myasthenia gravis with anti‐muscle‐specific tyrosine kinase antibodies: Two new cases and a systematic literature review

Kamada

Nakane

Matsui

et al. 2016

Clinical & Exp Neuroim

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Objective Many myasthenia gravis (MG) patients with anti‐muscle‐specific tyrosine kinase (MuSK) antibodies have prominent oculobulbar symptoms or weaknesses of the neck and respiratory muscles. An ocular form of MG having anti‐MuSK antibodies (MuSK‐OMG) is very rare. We review the clinical features of two such cases. Methods We reviewed cases of patients with an ocular form of MG having anti‐MuSK antibodies, including two new cases. Results We found seven published cases, plus two cases described in this report. The mean age at onset of these nine patients was 37.0 ± 17.7 years, and the mean disease duration (from ocular MG onset to report) was 47.0 ± 43.1 months. The clinical courses appeared benign, but heterogeneous. Five of the patients responded to pyridstigmine. Four patients received immunotherapy, which resulted in improvement. Our two patients had mild ocular symptoms. One patient was stable with no immunosuppressive treatment, and the other patient was treated with prednisolone and tacrolimus. Conclusions Cases of an ocular form of MG having anti‐MuSK antibodies do exist. The benign clinical courses and pharmacological responses to cholinesterase inhibitors imply that the antibodies might have different pathogenicities and specificities from those of MuSK‐generalized MG. The presence of anti‐MuSK antibodies should therefore be determined in ocular MG patients negative for anti‐acetylcholine receptor antibodies.

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Section: Resultsmentioning

confidence: 86%

Section: Resultsmentioning

confidence: 99%

“…27,28 Therefore, myasthenic involvement was not limited to the ocular muscles of patient 7. 28 Edrophonium tests were positive in two of four patients tested (50%).…”

Section: Literature Reviewmentioning

confidence: 93%

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

See 3 more Smart Citations

Ocular myasthenia gravis with anti‐muscle‐specific tyrosine kinase antibodies: Two new cases and a systematic literature review

Kamada

Nakane

Matsui

et al. 2016

Clinical & Exp Neuroim

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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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Autoantibodies in Japanese patients with ocular myasthenia gravis

et al. 2020

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Introduction The majority of patients with myasthenia gravis (MG) initially present with ocular symptoms, but it is difficult to predict which cases will remain as ocular MG (OMG) or will progress to generalized MG. Herein we evaluated the serologic profile of Japanese OMG and its relationship with clinical features. Methods Seventy‐three patients with OMG from five Japanese myasthenia gravis (MG) centers were enrolled. Live cell‐based assays (CBAs) were used to determine the presence of autoantibodies (Abs) to clustered adult (2α, β, δ, ε) and fetal (2α, β, δ, γ) acetylcholine receptor (AChR) isoforms, muscle‐specific receptor tyrosine kinase (MuSK), and lipoprotein receptor–related protein‐4 (LRP4). Results Thirty‐four of 73 (46.5%) serum samples were positive for Abs against both the adult‐type and fetal‐type AChR, as expected, but 7 (9.6%) and 2 (2.7%) were positive only for fetal or adult AChR‐Abs, respectively. Four (5.4%) samples were positive for MuSK‐Abs, but two of these also contained antibodies to fetal AChR or LRP4. Twenty‐six (35.6%) samples were seronegative. Discussion Abs against fetal‐specific AChR, MuSK, and LRP4 are found in some patients with OMG. Future studies attempting to predict conversion from ocular symptoms to generalized MG may benefit from measurement of these antibodies.

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Pure ocular anti‐musk myasthenia under no immunosuppressive treatment

Cited by 16 publications

References 14 publications

Ocular myasthenia gravis with anti‐muscle‐specific tyrosine kinase antibodies: Two new cases and a systematic literature review

Ocular myasthenia gravis with anti‐muscle‐specific tyrosine kinase antibodies: Two new cases and a systematic literature review

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

Autoantibodies in Japanese patients with ocular myasthenia gravis

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