TY - JOUR
T1 - Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism
AU - Horimoto, Yoshihiko
AU - Hayashi, Emi
AU - Ito, Yoshihiro
AU - Iida, Akihiko
AU - Goto, Yoji
AU - Kato, Shigenori
AU - Okita, Kenji
AU - Kako, Tetsuharu
AU - Sato, Chikako
AU - Tajima, Toshihisa
AU - Inagaki, Aki
AU - Nokura, Kazuya
AU - Hibino, Hiroaki
AU - Matsukawa, Noriyuki
AU - Yamada, Kazuo
AU - Kabasawa, Hidehiro
N1 - Funding Information:
This study was funded by The Rehabilitation Research Fund of Social Welfare Corporation Nagoya City Rehabilitation Agency.
Funding Information:
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.
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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.
AB - 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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U2 - 10.1007/s00415-020-09908-y
DO - 10.1007/s00415-020-09908-y
M3 - Article
C2 - 32440919
AN - SCOPUS:85085026948
VL - 267
SP - 2692
EP - 2696
JO - Journal of Neurology
JF - Journal of Neurology
SN - 0340-5354
IS - 9
ER -