Wilson's disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents.
Sixty patients with Wilson's disease have been studied by means of computerized cranial tomography (CT). The findings are described and analysed with particular reference to their value, both diagnostic and prognostic, in the management of this disease. The commonest abnormalities were ventricular dilation, 73 per cent; cortical atrophy, 63 per cent; brain-stem atrophy, 55 per cent. Characteristic hypodense areas in the regions of the basal ganglia were present in 45 per cent and almost invariably these were accompanied by one or more of the other CT abnormalities. This combination of findings is considered specific for Wilson's disease in the appropriate clinical context. CT abnormalities were most common and most marked in patients with a neurological presentation, only 2 out of 40 having a normal scan. Similar changes were also demonstrated in three-quarters of the patients with an hepatic presentation and nearly half of those who were presymptomatic. Nineteen patients were scanned on more than one occasion to assess the influence of treatment with penicillamine or triethylene tetramine on the abnormalities recorded initially. Fourteen showed basal ganglia hypodensities on first scanning and in ten of these there was a moderate to marked improvement in this abnormality in response to therapy. Corresponding with this there was considerable clinical improvement. Despite the findings of quite severe CT abnormalities in patients with Wilson's disease. suggesting considerable loss of neurons, patients will still respond well to treatment. We conclude that although the CT. Examination of patients with Wilson's disease is of value both in diagnosis and management, it is of no great help in prognosis.
D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D004108:Dilatation, Pathologic; D005029:Ethylenediamines / Q000627:therapeutic use*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D008297:Male; D009048:Motor Skills / Q000187:drug effects; D010396:Penicillamine / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D014266:Trientine / Q000627:therapeutic use*
D005029:Ethylenediamines; D010396:Penicillamine; D014266:Trientine