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Changes in the clinical spectrum of pediatric moyamoya disease over 40 years

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Abstract

Objective

To investigate the chronological changes in the clinical presentation and long-term prognosis of pediatric-onset moyamoya disease in our institute over 40 years.

Methods

We evaluated 282 pediatric-onset (≤ 15 years old) moyamoya disease patients who visited our institute from 1981 to 2020 (divided into the former period, 1981–2000, and the latter period, 2001–2020). Differences in the clinical presentation and the long-term outcome were compared between the periods. Multivariate analysis was also performed to reveal the risk factors for poor long-term outcomes.

Results

Compared to the former period, the total number of patients, the onset age and both the number of patients with family history and relatively older patients without symptoms or with headache were greater in the latter period (p < 0.05). The number of patients with poor long-term outcomes was significantly lower in the latter period (24.9% vs. 6.7%, p < 0.01). Multivariate analysis revealed that stroke onset, late cerebrovascular events and postoperative complications were independent risk factors for poor long-term outcomes (odds ratio = 31.4, 40.8 and 5.4, respectively).

Conclusions

Over the last 40 years, the number of pediatric moyamoya disease patients has increased, especially in relatively older patients with mild presentation and favorable long-term outcomes. In clinical studies, these chronological changes and the inclusion period of the participants need to be accounted for. Whether the increased diagnostic rate in the recent era has led to a decrease in late cerebrovascular events and favorable outcomes throughout life remains unknown and should be evaluated in the future.

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Data availability

The full dataset is available from the corresponding author on reasonable request.

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Funding

This work was supported by Grants-in-Aid for Scientific Research “KAKENHI,” the Japan Society for the Promotion of Science, Grant #19K18406, given to Shoko Hara.

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Authors and Affiliations

  1. Department of Neurosurgery, Tokyo Medical and Dental University, Tokyo, Japan

    Mai Fujioka, Shoko Hara, Maki Mukawa, Jun Karakama, Motoki Inaji, Yoji Tanaka, Tadashi Nariai & Taketoshi Maehara

Authors
  1. Mai Fujioka
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  2. Shoko Hara
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  3. Maki Mukawa
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  4. Jun Karakama
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  5. Motoki Inaji
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  6. Yoji Tanaka
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  7. Tadashi Nariai
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  8. Taketoshi Maehara
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Contributions

Conceptualization: Shoko Hara and Tadashi Nariai; Methodology: Mai Fujioka, Maki Mukawa, and Shoko Hara; Formal analysis and investigation: Mai Fujioka, Maki Mukawa, Jun Karakama, and Shoko Hara; Writing—original draft preparation: Mai Fujioka; Writing—review and editing: Shoko Hara, Maki Mukawa, Motoki Inaji, Yoji Tanaka, Tadashi Nariai, and Taketoshi Maehara; Funding acquisition: Shoko Hara; Resources: Maki Mukawa, Shoko Hara, Motoki Inaji, Yoji Tanaka, Tadashi Nariai, and Taketoshi Maehara; Supervision: Tadashi Nariai and Taketoshi Maehara.

Corresponding author

Correspondence to Shoko Hara.

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Ethics approval and consent to participate

This study was approved by the ethical committee of the local institutional review board (M2000-1331), and informed consent was obtained from all of the participants.

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Consent for publication was obtained from all of the participants.

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No conflict of interest exists.

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Fujioka, M., Hara, S., Mukawa, M. et al. Changes in the clinical spectrum of pediatric moyamoya disease over 40 years. Childs Nerv Syst 39, 1215–1223 (2023). https://doi.org/10.1007/s00381-023-05852-0

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  • DOI: https://doi.org/10.1007/s00381-023-05852-0

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