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Prognostic predictors of remission in ocular myasthenia gravis

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Abstract

Background

Ocular myasthenia gravis (OMG) constitutes 15% of all myasthenia gravis patients.

Methods

One hundred eight patients with OMG followed-up for over 36 months were retrospectively evaluated regarding factors associated with remission. Demographic features, neuro-ophthalmologic findings at onset, acetylcholine receptor (AChR Ab) and muscle-specifc tyrosine kinase antibodies (MuSK Ab), thymic status, single fiber electromyography (SFEMG) results were the variables considered.

Results

Median age of disease onset was 57 years (range 18–82 years). Clinical features at onset was isolated ptosis in 55 (50.9%) and isolated diplopia in 33 (30.6%) patients. Combined ptosis and diplopia were present in 20 (18.5%) patients. Among 75 patients with ptosis, it was unilateral in 65 (86.7%) and bilateral in 10 (13.3%). AChR Abs were found in 66 (61.1%) and MuSK Abs in 2 (1.9%) patients. SFEMG abnormality was detected in 74 (68.5%) patients. Thymoma was present in 16 (14.8%) and thymic hyperplasia in 6 (5.6%) patients. Forty-one patients (37.9%) had been treated with pyridostigmine alone. Sixty-seven (62%) patients were given immunosupressive drugs. In 53 (49.1%) prednisone was used and in 14 (12.9%) patients it was combined with azathioprine. Thymectomy was performed in all 16 patients with thymoma. Complete stable remission (CSR) was achieved in 49 (45.4%) patients. Fifty-nine (54.6%) patients had reached minimal manifestation (MM) status; 32 (29.6%) having a status of MM-1 and 27 (25%) a status of MM-3.

Conclusions

The presence of AchR Abs (p = 0.034) and an abnormal SFEMG (p = 0.006) at onset as increased risk factors for the presence of ongoing signs necessitating medical treatment.

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Authors and Affiliations

  1. Department of Neurology, Ege University Medical School, 35100, Izmir, Turkey

    Neşe Çelebisoy, Asim Orujov, Fidan Balayeva, Hüseyin Nezih Özdemir & Figen Gökçay

  2. Department of Neurology, Celal Bayar University Medical School, 45000, Manisa, Turkey

    Ayşın Kısabay Ak

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  1. Neşe Çelebisoy
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Contributions

Study design and concept: NÇ, FG. Acquisition of the data: AO, FB, HNÖ. Data analysis and interpretation: AO, FB, AKA, NÇ. Manuscript preparation and revision: NÇ, AKA, FG.

Corresponding author

Correspondence to Ayşın Kısabay Ak.

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Conflict of ınterest

The authors have no conflicts of interest to declare.

Ethics approval and consent to participate

The study protocol was approved by Ege University Medical School Ethics Committee (reference number: 99166796–050.06.04) and was performed in accordance with the ethical standards outlined in the Declaration of Helsinki. Written informed consent was obtained from all participants.

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A written informed consent was obtained from each patient for the publication of this report.

Competing interests

The authors declare that they have no competing interests.

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The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Çelebisoy, N., Orujov, A., Balayeva, F. et al. Prognostic predictors of remission in ocular myasthenia gravis. Acta Neurol Belg 123, 1927–1932 (2023). https://doi.org/10.1007/s13760-022-02151-4

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