Teaching File Series

On CT Scan

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14 Y Female referred  to CT with chorea.

Findings :
1. Diffuse mild cerebral atrophy

2. Bilateral symmetrical hypodense area in the Mid brain, Basal ganglia, Thalamus,  Parietal white matter.

Diagnosis Wilson Disease.

Discussion :
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

Reference :
Diagnostic neuro-radiology by ANNE G. OSBORN.

Submitted by : DR.VIJAYA D.M.(NEURO.)