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Recurrence and long-term outcomes of Tolosa-Hunt syndrome

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Abstract

Background and purpose

Tolosa-Hunt Syndrome (THS) is a rare disorder, and detailed clinical information and treatment outcomes have yet to be fully elucidated. This study aims to investigate the clinical features and factors associated with the treatment outcomes of THS, as defined by the established diagnostic criteria.

Methods

This study retrospectively recruited 91 patients with a diagnosis of THS from 2003 to 2020. We analyzed the clinical features and outcomes, the initial treatment response, recurrences, and the final treatment response.

Results

Isolated ocular motor nerve palsy was the most common (82.4%) finding of ophthalmoplegia, involving the oculomotor nerve in more than half of the cases (52.0%). The MRI lesions were mostly observed in the cavernous sinus (94.5%) with an extracavernous extension in about one-third of them. Five patients showed only extracavernous lesions. A total of 25 (27.5%) patients experienced recurrence. Recurrence occurred during steroid tapering as part of the initial treatment in seven, while in 18 patients, it happened after the successful termination of the initial treatment. However, all patients achieved complete remission at the final. Age was associated with a decrease in initial symptom duration (HR = 1.023, CI = 1.004–1.044) as well as an increase in recurrence-free duration (HR = 0.944, CI = 0.911–0.978). High-dose corticosteroid treatment was associated with a decrease in initial symptom duration (HR = 1.642, CI = 1.001–2.695) and total treatment duration (HR = 2.203 CI = 1.302–3.730).

Conclusions

THS can recur frequently especially in younger but have a favorable prognosis. High-dose corticosteroids can be an effective initial treatment and reduce the total treatment duration.

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Data availability statement

Anonymized data will be shared by request from any qualified investigator.

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Funding

This study was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science and ICT (2020R1A2C4002281).

Author information

Authors and Affiliations

  1. Department of Neurology, Chungbuk National University Hospital, Cheongju, Republic of Korea

    Hyun-Jae Kim

  2. Neurotology Laboratory, Korea University Anam Hospital, Seoul, Republic of Korea

    Sun-Uk Lee

  3. Department of Neurology, Korea University Medical Center, Seoul, Republic of Korea

    Sun-Uk Lee

  4. Department of Neurology, Soonchunhyang University Bucheon Hospital, Bucheon, Republic of Korea

    Eek-Sung Lee

  5. Dizziness Center, Clinical Neuroscience Center and Department of Neurology, Seoul National University Bundang Hospital, 173-82 Gumi-ro, Bundang-gu, Seongnam-si, Gyeonggi-do, 13620, Republic of Korea

    Jeong-Yoon Choi & Ji-Soo Kim

  6. Department of Neurology, Seoul National University College of Medicine, Seoul, Republic of Korea

    Jeong-Yoon Choi & Ji-Soo Kim

Authors
  1. Hyun-Jae Kim
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Contributions

HJK analyzed and interpreted the data and wrote the manuscript. SL, ESL, HKY, JMH, JSK analyzed and interpreted the data and revised the manuscript. JYC designed and conceptualized the study, interpreted the data, and revised the manuscript.

Corresponding author

Correspondence to Jeong-Yoon Choi.

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Conflict of interest

Dr. J. S. Kim serves as an associate editor of Frontiers in Neuro-otology and on the editorial boards of Frontiers in Neuro-ophthalmology, Journal of Neuro-ophthalmology, Journal of Vestibular Research, and Clinical and Translational Neuroscience. The other authors have nothing to disclose.

Ethical approval

This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of the Institutional Review Board of Seoul National University Bundang Hospital (B‑2109–707‑001).

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Kim, HJ., Lee, SU., Lee, ES. et al. Recurrence and long-term outcomes of Tolosa-Hunt syndrome. J Neurol 271, 935–943 (2024). https://doi.org/10.1007/s00415-023-12044-y

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  • DOI: https://doi.org/10.1007/s00415-023-12044-y

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