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21.05.2020 | Original Communication

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

verfasst von: Yoshihiko Horimoto, Emi Hayashi, Yoshihiro Ito, Akihiko Iida, Yoji Goto, Shigenori Kato, Kenji Okita, Tetsuharu Kako, Chikako Sato, Toshihisa Tajima, Aki Inagaki, Kazuya Nokura, Hiroaki Hibino, Noriyuki Matsukawa, Kazuo Yamada, Hidehiro Kabasawa

Erschienen in: Journal of Neurology | Ausgabe 9/2020

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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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Titel: Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism
verfasst von: Yoshihiko Horimoto
Emi Hayashi
Yoshihiro Ito
Akihiko Iida
Yoji Goto
Shigenori Kato
Kenji Okita
Tetsuharu Kako
Chikako Sato
Toshihisa Tajima
Aki Inagaki
Kazuya Nokura
Hiroaki Hibino
Noriyuki Matsukawa
Kazuo Yamada
Hidehiro Kabasawa
Publikationsdatum: 21.05.2020
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 9/2020
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-09908-y

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

Abstract

Background

Methods

Results

Conclusions

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Update Neurologie

Live-Webinar: Aktuelle Leitlinien bei Herz-Kreislauf-Erkrankungen

Springer Medizin

Abstract

Background

Methods

Results

Conclusions

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