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Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism

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Abstract

Background

Although pure cerebellar ataxia is usually emphasized as the characteristic clinical feature of spinocerebellar ataxia type 6 (SCA6), parkinsonism has been repeatedly described in patients with genetically confirmed SCA6.

Methods

We conducted a positron emission tomography study using a combination of [18F]fluoro-l-dopa for dopamine synthesis and [11C]raclopride for dopamine D2 receptor function on six genetically confirmed SCA6 patients, both with and without parkinsonism. To the best of our knowledge, this is the first dopamine receptor imaging study of patients with SCA6.

Results

Most patients had somewhat decreased dopaminergic function, and this decrease was significant in the caudate nucleus. In addition, one SCA6 patient with parkinsonism had whole striatal dysfunction of both dopamine synthesis and dopamine D2 receptor function.

Conclusions

The pathology of SCA6 may not be restricted to the cerebellum, but may also be distributed across various regions, including in both presynaptic and postsynaptic dopaminergic neurons to some degree. Patients with SCA6 may show apparent parkinsonism after the progression of neurodegeneration.

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Acknowledgements

This study was funded by The Rehabilitation Research Fund of Social Welfare Corporation Nagoya City Rehabilitation Agency. We are especially grateful to the late Dr. Michitaka Matsubara, former Director of Department of Planning and Research, Nagoya City Rehabilitation Center, and to the late Professor Tomoyuki Shirai, former President of Nagoya City Rehabilitation Center and Professor Emeritus of Nagoya City University, for their kind suggestions during their lifetimes. We also thank Mr. Shin Hibino, Department of Planning and Research, Nagoya City Rehabilitation Center, for his excellent technical assistance.

Funding

This study was funded by The Rehabilitation Research Fund of Social Welfare Corporation Nagoya City Rehabilitation Agency.

Author information

Authors and Affiliations

  1. Department of Neurology, Nagoya City Rehabilitation Center, 1-2, Mikan-yama, Yatomi-cho, Mizuho, Nagoya, 467-8622, Japan

    Yoshihiko Horimoto, Chikako Sato, Toshihisa Tajima, Aki Inagaki, Hiroaki Hibino & Hidehiro Kabasawa

  2. Department of Radiology, Nagoya City Rehabilitation Center, Nagoya, Japan

    Emi Hayashi, Yoshihiro Ito & Akihiko Iida

  3. Department of Planning and Research, Nagoya City Rehabilitation Center, Nagoya, Japan

    Emi Hayashi, Yoshihiro Ito & Toshihisa Tajima

  4. Department of Neurology, Japanese Red Cross Nagoya Daiichi Hospital, Nagoya, Japan

    Yoji Goto

  5. Department of Neurology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan

    Shigenori Kato

  6. Department of Neurology, Nagoya City University, Nagoya, Japan

    Kenji Okita & Noriyuki Matsukawa

  7. Department of Neurology, Bantane Hospital, Fujita Health University, Nagoya, Japan

    Tetsuharu Kako & Kazuya Nokura

  8. Department of Neurosurgery, Nagoya City Rehabilitation Center, Nagoya, Japan

    Kazuo Yamada

Authors
  1. Yoshihiko Horimoto
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  2. Emi Hayashi
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  3. Yoshihiro Ito
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  4. Akihiko Iida
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  5. Yoji Goto
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  6. Shigenori Kato
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  7. Kenji Okita
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  8. Tetsuharu Kako
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  9. Chikako Sato
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  10. Toshihisa Tajima
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  11. Aki Inagaki
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  12. Kazuya Nokura
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  14. Noriyuki Matsukawa
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  15. Kazuo Yamada
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  16. Hidehiro Kabasawa
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Contributions

All of the authors have read the manuscript and have approved this submission. YH: Recruitment of patients. Assessment of parkinsonism. EH: Scanning and analyzing of images. YI: Scanning of images. AI: Scanning and analyzing of images. YG: Recruitment of patients. Assessment of parkinsonism. SK: Recruitment of patients. Assessment of parkinsonism. KO: Recruitment of patients. Assessment of parkinsonism. TK: Recruitment of patients. Assessment of parkinsonism. CS: Recruitment of patients. TT: Recruitment of patients. AI: Recruitment of patients. KN: Recruitment of patients. HH: Recruitment of patients. NM: Recruitment of patients. KY: Recruitment of patients. HK: Recruitment of patients.

Corresponding author

Correspondence to Yoshihiko Horimoto.

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Conflicts of interest

There are no conflicts of interest or competing interests to declare.

Ethics approval

This study was approved by The Ethics Committee of Nagoya City Rehabilitation Center.

Informed Consent

All participants gave written informed consent to participate.

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All authors have read and agree with the contents of the manuscript for publication.

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Horimoto, Y., Hayashi, E., Ito, Y. et al. Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism. J Neurol 267, 2692–2696 (2020). https://doi.org/10.1007/s00415-020-09908-y

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  • DOI: https://doi.org/10.1007/s00415-020-09908-y

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