Journal of Neurology

, Volume 263, Issue 11, pp 2179–2187 | Cite as

Relationship between type 1 metabotropic glutamate receptors and cerebellar ataxia

  • Kenji IshibashiEmail author
  • Yoshiharu Miura
  • Kinya Ishikawa
  • Ming-Rong Zhang
  • Jun Toyohara
  • Kiichi Ishiwata
  • Kenji Ishii
Original Communication


Imaging of type 1 metabotropic glutamate receptor (mGluR1) has recently become possible using positron emission tomography (PET). We aimed to examine the relationship between mGluR1 and cerebellar ataxia. Families with spinocerebellar ataxia type 19/22 (SCA19/22) and SCA6, six patients with sporadic SCA, and 26 healthy subjects underwent PET using an mGluR1 radiotracer. Volumes-of-interest were placed on the anterior and posterior lobes and vermis. The binding potential (BPND) was calculated to estimate mGluR1 availability. A partial volume correction was applied to the BPND values. The Scale for the Assessment and Rating of Ataxia (SARA) score were measured. In each patient with SCA19/22 and SCA6, the anterior lobe showed the highest decrease rates in the BPND values, compared with healthy subjects. In the families with SCA19/22 and SCA6, the disease durations and SARA scores were shorter and lower, respectively, in the offspring, compared with the parents. However, the offspring paradoxically showed lower BPND values, especially in the anterior lobe, compared with the parents. The patients with sporadic SCA showed significantly lower BPND values in all subregions than healthy subjects. The BPND values significantly correlated with the SARA scores in all participants. In conclusion, these results showed a decrease in mGluR1 availability in patients with hereditary and sporadic SCA, a correlation between mGluR1 availability and degree of cerebellar ataxia, and paradoxical findings in two families. These results suggest the potential use of mGluR1 imaging as a specific biomarker of cerebellar ataxia.


Type 1 metabotropic glutamate receptor Spinocerebellar ataxia type 6 Spinocerebellar ataxia type 19/22 Sporadic spinocerebellar ataxia PET 11C-ITMM 



This study was supported by a Grant-in-Aid for Young Scientists (B) no. 15K19503 to KIshibashi and for Scientific Research (B) no. 24390298 to KIshiwata from the Japan Society for the Promotion of Science. The authors thank the people of Research Team for Neuroimaging at the Tokyo Metropolitan Institute of Gerontology.

Compliance with ethical standards

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard

The study was approved by the Ethics Committee of the Tokyo Metropolitan Institute of Gerontology (H26-49).


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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Kenji Ishibashi
    • 1
    • 2
    Email author
  • Yoshiharu Miura
    • 2
  • Kinya Ishikawa
    • 3
  • Ming-Rong Zhang
    • 4
  • Jun Toyohara
    • 1
  • Kiichi Ishiwata
    • 1
    • 5
    • 6
  • Kenji Ishii
    • 1
  1. 1.Research Team for NeuroimagingTokyo Metropolitan Institute of GerontologyTokyoJapan
  2. 2.Department of NeurologyTokyo Metropolitan Cancer and Infectious Diseases Center Komagome HospitalTokyoJapan
  3. 3.Department of Neurology and Neurological Science, and Predictive and Preventive MedicineTokyo Medical and Dental UniversityTokyoJapan
  4. 4.Molucular Probe Program, Molecular Imaging CenterNational Institute of Radiological SciencesChibaJapan
  5. 5.Institute of Cyclotron and Drug Discovery ResearchSouthern Tohoku Research Institute for NeuroscienceKoriyamaJapan
  6. 6.Department of Biofunctional ImagingFukushima Medical UniversityFukushimaJapan

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