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Marked reduction of striatal dopamine D 2 receptors as detected by 123 IBZM‐SPECT in a Wilson's disease patient with generalized dystonia
Author(s) -
Schwarz J.,
Tatsch K.,
Vogl T.,
Kirsch C. M.,
Trenkwalder C.,
Arnold G.,
Gasser T.,
Oertel W. H.
Publication year - 1992
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870070111
Subject(s) - dystonia , basal ganglia , striatum , dopamine receptor d2 , apomorphine , dopamine , single photon emission computed tomography , spect imaging , medicine , nuclear medicine , psychology , endocrinology , neuroscience , dopaminergic , central nervous system
[ 123 I]iodobenzamide‐single photon emission computed tomography (IBZMSPECT) was employed to study the distribution of dopamine D 2 receptors in a patient with biochemically proven Wilson's disease presenting with generalized dystonia. IBZM is a dopamine D 2 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 T 2 ‐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.