Decrease of D2 receptors indicated by 123I-iodobenzamide single-photon emission computed tomography relates to neurological deficit in treated Wilson's disease.

W H Oertel; K Tatsch; J Schwarz; E Kraft; C Trenkwalder; J Scherer; M Weinzierl; T Vogl; C M Kirsch

doi:10.1002/ana.410320607

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Decrease of D2 receptors indicated by 123I-iodobenzamide single-photon emission computed tomography relates to neurological deficit in treated Wilson's disease.

W H Oertel·K Tatsch·J Schwarz·E Kraft·C Trenkwalder·J Scherer·M Weinzierl·T Vogl·C M Kirsch

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Abstract

Single-photon emission computed tomography with 123I-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 123I-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.

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Adolescent Adult Basal Ganglia Benzamides Dopamine D2 Receptor Antagonists Hepatolenticular Degeneration Humans Iodine Radioisotopes Pyrrolidines Receptors, Dopamine D2 Tomography, Emission-Computed, Single-Photon Journal Article Research Support, Non-U.S. Gov't

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W H Oertel, K Tatsch, J Schwarz, E Kraft, C Trenkwalder, J Scherer, M Weinzierl, T Vogl, C M Kirsch. 1992. Decrease of D2 receptors indicated by 123I-iodobenzamide single-photon emission computed tomography relates to neurological deficit in treated Wilson's disease.. https://doi.org/10.1002/ana.410320607

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