Evaluation of parkinsonism and striatal dopamine transporter loss in patients with spinocerebellar ataxia type 6

Tao Xie, Daniel Appelbaum, Jacqueline Bernard, Mahesh Padmanaban, Yonglin Pu, Christopher Gomez

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

It is unclear whether patients with spinocerebellar ataxia type 6 (SCA6) have parkinsonism and striatal dopamine transporter (DAT) loss, based on previously small size studies without well-matched controls. A study with a larger number of patients and both age- and gender-matched healthy controls (HCs) is needed for a better answer to this question. Twelve genetically confirmed ataxic SCA6 patients (six male six female, age 65.3 ± 11.2 years), and eight age- and gender-matched HCs (five male three female, age 71.3 ± 8.6 years) were enrolled during 2013–2015 from tertiary movement disorders and ataxia clinics. Clinical assessment for parkinsonism, and qualitative and quantitative assessment of DAT level on DaTscan™ imaging were conducted in SCA6 patients compared to HCs. We found no convincing parkinsonism in SCA6 patients, given generalized bradykinesia in the context of significant ataxia in all, with mild symmetric rigidity in five without resting tremor. Furthermore, we found no striatal DAT loss in anterior, posterior, and total putamen and caudate on imaging, assessed independently by qualitative visual inspection in a blinded manner by the nuclear medicine specialist and movement disorder specialist (kappa = 1). Additional quantitative analysis on these areas did not reveal significant DAT loss either in SCA6 patients compared to HCs. We conclude that there is no convincing parkinsonism or DAT loss in SCA6 patients in this unique study with a larger than previously reported number of patients compared to both age- and gender-matched HCs, suggesting that dopaminergic dysfunction is not usually involved in SCA6.

Original languageEnglish (US)
Pages (from-to)2302-2307
Number of pages6
JournalJournal of Neurology
Volume263
Issue number11
DOIs
StatePublished - Nov 1 2016
Externally publishedYes

Fingerprint

Spinocerebellar Ataxias
Corpus Striatum
Dopamine Plasma Membrane Transport Proteins
Parkinsonian Disorders
Movement Disorders
Ataxia
Hypokinesia
Putamen
Nuclear Medicine
Tremor

Keywords

  • Dopamine transporter
  • Parkinsonism
  • SCA6
  • Spinocerebellar ataxia

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Evaluation of parkinsonism and striatal dopamine transporter loss in patients with spinocerebellar ataxia type 6. / Xie, Tao; Appelbaum, Daniel; Bernard, Jacqueline; Padmanaban, Mahesh; Pu, Yonglin; Gomez, Christopher.

In: Journal of Neurology, Vol. 263, No. 11, 01.11.2016, p. 2302-2307.

Research output: Contribution to journalArticle

Xie, Tao ; Appelbaum, Daniel ; Bernard, Jacqueline ; Padmanaban, Mahesh ; Pu, Yonglin ; Gomez, Christopher. / Evaluation of parkinsonism and striatal dopamine transporter loss in patients with spinocerebellar ataxia type 6. In: Journal of Neurology. 2016 ; Vol. 263, No. 11. pp. 2302-2307.
@article{d0b96bdcb8294e86a5d5ccb3bad4d298,
title = "Evaluation of parkinsonism and striatal dopamine transporter loss in patients with spinocerebellar ataxia type 6",
abstract = "It is unclear whether patients with spinocerebellar ataxia type 6 (SCA6) have parkinsonism and striatal dopamine transporter (DAT) loss, based on previously small size studies without well-matched controls. A study with a larger number of patients and both age- and gender-matched healthy controls (HCs) is needed for a better answer to this question. Twelve genetically confirmed ataxic SCA6 patients (six male six female, age 65.3 ± 11.2 years), and eight age- and gender-matched HCs (five male three female, age 71.3 ± 8.6 years) were enrolled during 2013–2015 from tertiary movement disorders and ataxia clinics. Clinical assessment for parkinsonism, and qualitative and quantitative assessment of DAT level on DaTscan™ imaging were conducted in SCA6 patients compared to HCs. We found no convincing parkinsonism in SCA6 patients, given generalized bradykinesia in the context of significant ataxia in all, with mild symmetric rigidity in five without resting tremor. Furthermore, we found no striatal DAT loss in anterior, posterior, and total putamen and caudate on imaging, assessed independently by qualitative visual inspection in a blinded manner by the nuclear medicine specialist and movement disorder specialist (kappa = 1). Additional quantitative analysis on these areas did not reveal significant DAT loss either in SCA6 patients compared to HCs. We conclude that there is no convincing parkinsonism or DAT loss in SCA6 patients in this unique study with a larger than previously reported number of patients compared to both age- and gender-matched HCs, suggesting that dopaminergic dysfunction is not usually involved in SCA6.",
keywords = "Dopamine transporter, Parkinsonism, SCA6, Spinocerebellar ataxia",
author = "Tao Xie and Daniel Appelbaum and Jacqueline Bernard and Mahesh Padmanaban and Yonglin Pu and Christopher Gomez",
year = "2016",
month = "11",
day = "1",
doi = "10.1007/s00415-016-8261-6",
language = "English (US)",
volume = "263",
pages = "2302--2307",
journal = "Journal of Neurology",
issn = "0340-5354",
publisher = "D. Steinkopff-Verlag",
number = "11",

}

TY - JOUR

T1 - Evaluation of parkinsonism and striatal dopamine transporter loss in patients with spinocerebellar ataxia type 6

AU - Xie, Tao

AU - Appelbaum, Daniel

AU - Bernard, Jacqueline

AU - Padmanaban, Mahesh

AU - Pu, Yonglin

AU - Gomez, Christopher

PY - 2016/11/1

Y1 - 2016/11/1

N2 - It is unclear whether patients with spinocerebellar ataxia type 6 (SCA6) have parkinsonism and striatal dopamine transporter (DAT) loss, based on previously small size studies without well-matched controls. A study with a larger number of patients and both age- and gender-matched healthy controls (HCs) is needed for a better answer to this question. Twelve genetically confirmed ataxic SCA6 patients (six male six female, age 65.3 ± 11.2 years), and eight age- and gender-matched HCs (five male three female, age 71.3 ± 8.6 years) were enrolled during 2013–2015 from tertiary movement disorders and ataxia clinics. Clinical assessment for parkinsonism, and qualitative and quantitative assessment of DAT level on DaTscan™ imaging were conducted in SCA6 patients compared to HCs. We found no convincing parkinsonism in SCA6 patients, given generalized bradykinesia in the context of significant ataxia in all, with mild symmetric rigidity in five without resting tremor. Furthermore, we found no striatal DAT loss in anterior, posterior, and total putamen and caudate on imaging, assessed independently by qualitative visual inspection in a blinded manner by the nuclear medicine specialist and movement disorder specialist (kappa = 1). Additional quantitative analysis on these areas did not reveal significant DAT loss either in SCA6 patients compared to HCs. We conclude that there is no convincing parkinsonism or DAT loss in SCA6 patients in this unique study with a larger than previously reported number of patients compared to both age- and gender-matched HCs, suggesting that dopaminergic dysfunction is not usually involved in SCA6.

AB - It is unclear whether patients with spinocerebellar ataxia type 6 (SCA6) have parkinsonism and striatal dopamine transporter (DAT) loss, based on previously small size studies without well-matched controls. A study with a larger number of patients and both age- and gender-matched healthy controls (HCs) is needed for a better answer to this question. Twelve genetically confirmed ataxic SCA6 patients (six male six female, age 65.3 ± 11.2 years), and eight age- and gender-matched HCs (five male three female, age 71.3 ± 8.6 years) were enrolled during 2013–2015 from tertiary movement disorders and ataxia clinics. Clinical assessment for parkinsonism, and qualitative and quantitative assessment of DAT level on DaTscan™ imaging were conducted in SCA6 patients compared to HCs. We found no convincing parkinsonism in SCA6 patients, given generalized bradykinesia in the context of significant ataxia in all, with mild symmetric rigidity in five without resting tremor. Furthermore, we found no striatal DAT loss in anterior, posterior, and total putamen and caudate on imaging, assessed independently by qualitative visual inspection in a blinded manner by the nuclear medicine specialist and movement disorder specialist (kappa = 1). Additional quantitative analysis on these areas did not reveal significant DAT loss either in SCA6 patients compared to HCs. We conclude that there is no convincing parkinsonism or DAT loss in SCA6 patients in this unique study with a larger than previously reported number of patients compared to both age- and gender-matched HCs, suggesting that dopaminergic dysfunction is not usually involved in SCA6.

KW - Dopamine transporter

KW - Parkinsonism

KW - SCA6

KW - Spinocerebellar ataxia

UR - http://www.scopus.com/inward/record.url?scp=84982299349&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84982299349&partnerID=8YFLogxK

U2 - 10.1007/s00415-016-8261-6

DO - 10.1007/s00415-016-8261-6

M3 - Article

C2 - 27544504

AN - SCOPUS:84982299349

VL - 263

SP - 2302

EP - 2307

JO - Journal of Neurology

JF - Journal of Neurology

SN - 0340-5354

IS - 11

ER -