Decrease of D2 receptors indicated by 123I-iodobenzamide single-photon emission computed tomography relates to neurological deficit in treated Wilson's disease
✍ Scribed by Dr W. H. Oertel; K. Tatsch; J. Schwarz; E. Kraft; C. Trenkwalder; J. Scherer; M. Weinzierl; T. Vogl; C. M. Kirsch
- Publisher
- John Wiley and Sons
- Year
- 1992
- Tongue
- English
- Weight
- 697 KB
- Volume
- 32
- Category
- Article
- ISSN
- 0364-5134
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✦ Synopsis
Abstract
Single‐photon emission computed tomography with ^123^I‐iodobenzamide, a dopamine D2 receptor antagonist, was employed to study dopamine D2 receptor densities in 17 patients with biochemically proved Wilson's disease and stable neurological status with therapy and in 5 age‐matched control subjects. Of the 17 patients with Wilson's disease, 5 were neurologically asymptomatic, 3 had cerebellar signs, 1 exhibited a mild parkinsonian syndrome, 7 showed a parkinsonian syndrome and cerebellar signs, and 1 had generalized dystonia and a parkinsonian syndrome. In 5 age‐matched control subjects specific isotope binding as calculated by the basal ganglia to frontal cortex ratio was 1.57 ± 0.04 (mean ± standard deviation). The ratio in patients with Wilson's disease ranged from 1.56 ± 0.05 (n = 5, asymptomatic patients) to 1.17 ± 0.02 (n = 4, marked neurological impairment). We observed an almost linear correlation between the reduction of ^123^I‐iodobenzamide (IBZM) binding and the severity of neurological signs at the time of IBZM‐SPECT (correlation coefficient, −0.84; p < 0.01). We suggest that the reduction of postsynaptic striatal dopamine D2 receptors as detected by IBZM‐SPECT reflects striatal neuronal damage in Wilson's disease.