RT Journal Article
SR Electronic
T1 Concordant Pre- and Postsynaptic Deficits of Dopaminergic Neurotransmission in Neurologic Wilson Disease
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 234
OP 238
VO 24
IS 2
A1 Barthel, Henryk
A1 Hermann, Wieland
A1 Kluge, Regine
A1 Hesse, Swen
A1 Collingridge, David R.
A1 Wagner, Armin
A1 Sabri, Osama
YR 2003
UL http://www.ajnr.org/content/24/2/234.abstract
AB BACKGROUND AND PURPOSE: Although previous brain imaging studies of Wilson disease (WD) focused on the dopaminergic system, correlational data on the integrity of the pre- and postsynaptic compartments are lacking. The present study was initiated to intraindividually determine the integrity of these compartments in patients with WD.METHODS: A total of 46 patients with WD and 10 matched control subjects underwent [123I]2β-carbomethoxy-3β-(4[123I]iodophenyl)tropane ([123I]β-CIT) and [123I]iodobenzamide ([123I]IBZM) single photon emission CT (SPECT). For both radiotracers, specific striatal binding ratios (with the cerebellum as the reference region) were calculated after a standardized region-of-interest technique was applied. In addition, the severity of putative neurologic symptoms was evaluated by using a linear scoring system.RESULTS: In patients without neurologic symptoms, striatal binding ratios of both radiotracers did not differ from those of the control group (13.8 ± 3.1 vs 12.0 ± 3.4 and 2.00 ± 0.19 vs 1.90 ± 0.27; n.s.). In symptomatic patients, however, striatal binding ratios for both [123I]β-CIT and [123I]IBZM were significantly reduced (9.1 ± 2.3 and 1.64 ± 0.18; P < .001). In all patients with WD, the [123I]β-CIT and [123I]IBZM binding ratios were significantly correlated (r = 0.65, P < .001), as were SPECT parameters and the severity of the neurologic symptoms (r = −0.60 and −0.62; P < .001).CONCLUSION: These findings of a concordant bicompartmental dopaminergic deficit in neurologic WD provide in vivo evidence for assigning WD to the group of secondary Parkinsonian syndromes. These results could be relevant in therapeutic decision making in patients with this copper deposition disorder.