Journal of Neurology

, Volume 249, Issue 8, pp 1016–1020

Normal dopamine transporter binding in dopa responsive dystonia

  • Chin-Chang Huang
  • Tzu-Chen Yen
  • Yi-Hsin Weng
  • Chin-Song Lu
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-002-0776-3

Cite this article as:
Huang, CC., Yen, TC., Weng, YH. et al. J Neurol (2002) 249: 1016. doi:10.1007/s00415-002-0776-3

Abstract

We report the clinical manifestations of dopa responsive dystonia (DRD) in 2 patients from the same family. The brain magnetic resonance images (MRI) were normal. The dopamine transporter (DAT) imaging with 99mTc-TRODAT-1 was performed in the 2 probands, 8 patients with young onset Parkinson disease (YOPD) and 16 normal controls. The ratios of 99mTc-TRODAT-1 brain SPECT in the striatum were 2.40 ± 0.12 (right) and 2.30 ± 0.17 (left) in these 2 DRD patients as compared with 1.38 ± 0.18 (right), 1.41 ± 0.20 (left) in YOPD patients, and 2.15 ± 0.35 (right), 2.14 ± 0.32 (left) in normal controls respectively. A normal DAT uptake was found in DRD suggesting a normal presynaptic nigrostriatal dopaminergic terminal. We conclude that a normal DAT in parkinsonian patients can differentiate DRD from YOPD. In addition, DAT with 99mTc-TRODAT-1 is a reliable and convenient tool to study the function of the presynaptic dopaminergic axonal terminals.

Key words dopa responsive dystonia Segawa's disease parkinsonism dopamine transporter 99mTc-TRODAT-1 

Copyright information

© Steinkopff Verlag 2002

Authors and Affiliations

  • Chin-Chang Huang
    • 1
  • Tzu-Chen Yen
    • 2
  • Yi-Hsin Weng
    • 1
  • Chin-Song Lu
    • 1
  1. 1.Department of Neurology Chang Gung Memorial Hospital 199, Tung Hwa North Road Taipei, Taiwan Tel.: +886-3/3281200, ext 8420 Fax: +886-3/3287226 E-Mail: cch0537@adm.cgmh.org.twTW
  2. 2.Department of Nuclear Medicine Chang Gung Memorial Hospital and University Taipei, TaiwanTW