Marked reduction of striatal dopamine D2 receptors as detected by 123IBZM-SPECT in a Wilson's disease patient with generalized dystonia.
[123I]iodobenzamide-single photon emission computed tomography (IBZM-SPECT) was employed to study the distribution of dopamine D2 receptors in a patient with biochemically proven Wilson's disease presenting with generalized dystonia. IBZM is a dopamine D2 receptor antagonist with high affinity and specific binding to basal ganglia detectable by SPECT. IBZM-SPECT in this patient (age, 20 years) displayed a striatum to frontal cortex ratio of 1.2 compared to 1.55 +/- 0.05 (mean +/- SD) in normal controls (n = 7; mean age, 53.3 years). In parallel with this finding, MRI with heavily T2-weighted sequences showed atrophy and low signal intensity changes of the basal ganglia. There was no improvement of dystonia after a subcutaneous injection of apomorphine. In contrast, IBZM-SPECT of a neurologically asymptomatic Wilson's disease patient (age, 21 years) displayed a striatum to frontal cortex ratio of 1.6. The MRI scan of this patient was normal. It is suggested that the observed apomorphine-unresponsive generalized dystonia in this Wilson's disease patient is related to striatal lesions proven by IBZM-SPECT and MRI.
Schwarz
J
J
Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany.
Tatsch
K
K
Vogl
T
T
Kirsch
C M
CM
Trenkwalder
C
C
Arnold
G
G
Gasser
T
T
Oertel
W H
WH
D000328:Adult; D001549:Benzamides; D001921:Brain / Q000000981:diagnostic imaging*; D001931:Brain Mapping; D003342:Corpus Striatum / Q000000981:diagnostic imaging*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging*; D006801:Humans; D007457:Iodine Radioisotopes; D011759:Pyrrolidines; D011954:Receptors, Dopamine / Q000032:analysis*; D017448:Receptors, Dopamine D2; D015899:Tomography, Emission-Computed, Single-Photon
D001549:Benzamides; D007457:Iodine Radioisotopes; D011759:Pyrrolidines; D011954:Receptors, Dopamine; D017448:Receptors, Dopamine D2; C055743:3-iodo-2-hydroxy-6-methoxy-N-((1-ethyl-2-pyrrolidinyl)methyl)benzamide