Normal dopamine transporter binding in dopa responsive dystonia.
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 (99m)Tc-TRODAT-1 was performed in the 2 probands, 8 patients with young onset Parkinson disease (YOPD) and 16 normal controls. The ratios of (99m)Tc-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 (99m)Tc-TRODAT-1 is a reliable and convenient tool to study the function of the presynaptic dopaminergic axonal terminals.
Huang
Chin-Chang
CC
Department of Neurology, Chang Gung Memorial Hospital, 199, Tung Hwa North Road, Taipei, Taiwan. cch0537@adm.cgmh.org.tw
Yen
Tzu-Chen
TC
Weng
Yi-Hsin
YH
Lu
Chin-Song
CS
D000328:Adult; D001921:Brain / Q000378:metabolism; D003937:Diagnosis, Differential; D050483:Dopamine Plasma Membrane Transport Proteins; D020821:Dystonic Disorders / Q000175:diagnosis / Q000378:metabolism*; D005260:Female; D006801:Humans; D008562:Membrane Glycoproteins; D026901:Membrane Transport Proteins / Q000378:metabolism*; D008875:Middle Aged; D009419:Nerve Tissue Proteins; D015609:Organotechnetium Compounds; D010300:Parkinson Disease / Q000175:diagnosis / Q000378:metabolism*; D020734:Parkinsonian Disorders / Q000175:diagnosis / Q000378:metabolism*; D015899:Tomography, Emission-Computed, Single-Photon; D014326:Tropanes
D050483:Dopamine Plasma Membrane Transport Proteins; D008562:Membrane Glycoproteins; D026901:Membrane Transport Proteins; D009419:Nerve Tissue Proteins; D015609:Organotechnetium Compounds; C493214:SLC6A3 protein, human; D014326:Tropanes; C104196:technetium Tc 99m TRODAT-1