14 Y Female referred to CT with chorea.
1. Diffuse mild cerebral atrophy
2. Bilateral symmetrical hypodense area
in the Mid brain, Basal ganglia, Thalamus, Parietal white matter.
Wilson disease is autosomal recessive inherited disorder .
Deficiency of copper transport serum protein ceruloplasmin resulting in accumulation
of Copper in various tissues.WD can occur at any age but symptomatic between
ages of 8 &16years. Neurological
symptoms are due to accumulation of copper in basal ganglia,
indirectly as an encephalopathy complicating
progressive liver disease.
Neuro-imaging studies in some cases are normal or show only mild
generalized atrophy. Other show bilateral putaminal low density lesions on NECT
Scans and high signal in the thalami, putamen, dentate nuclei,
brainstem ont2- weighted MR scans
Diagnostic neuro-radiology by ANNE G. OSBORN.
by : DR.VIJAYA