NEUROLOGICAL WILSONS-DISEASE STUDIED WITH MAGNETIC-RESONANCE-IMAGING AND WITH POSITRON EMISSION TOMOGRAPHY USING DOPAMINERGIC MARKERS

Four patients with neurological Wilson's disease were investigated usi ng magnetic resonance imaging (MRI) and positron emission tomography ( PET), All patients had dystonia as their major clinical manifestation but also had dysarthria and at the presentation of the disease had cho reoathetoid movements in at least one limb, A multitracer approach wit h PET was used to visualize various aspects of dopaminergic function; [C-11]-(+)nomifensine (NMF), [C-11]raclopride (RAG) and [C-11]-L-DOPA (one patient). Correlation analysis of RAC and NMF binding as well as putamen/caudate uptake ratios showed corresponding reductions. The pat ient investigated with [C-11]-L-DOPA had a normal striatal uptake. Gen erally, structural changes as shown by MRI corresponded to reductions both in NMF and RAC binding. There was no evident correspondence betwe en PET findings and the severity of clinical symptoms seen in the indi vidual patient, In two patients with discrete neurological impairment at the time of investigation, PET showed serious presynaptic dopaminer gic Lesions in the putamen. Our data suggest that the striatal degener ation seen in Wilson's disease comprises a complex pathology involving both afferent and efferent projections, The discrete neurological imp airment seen in some patients with gross striatal pathology might be d ue to concomitant lesions in functionally counteracting basal ganglia circuits.