A 14 years old male child presented with abnormal body movements with MRI showing signal changes in corpus striatum, thalami, pons, medulla, centrum semiovale and asymmetric diffuse white matter involvement. Most likely diagnosis is:
Parkinsonism
Nigrostriatal degeneration
Wilson's disease
Hallervorden-Spatz disease
Answer- C. Wilson's diseaseWilson disease:Wilson disease usually presents with tremors, rigidity, dementia, and pseudobulbar features and has an autosomal recessive mode of inheritance.Slit-lamp examination of the eyes may reveal a Kayser-Fleischer ring.MRI in Wilson disease exhibits the characteristic changes consisting of high-intensity lesions in the basal ganglia, thalami, and mid brain on T2-weighted images. The normal low intensity of red nuclei and SN surrounded by abnormal high-signal intensity in the tegmentum of the mid brain gives rise to the typical 'face-of-the-giant-panda' sign.Best diagnostic clue is symmetrical T2 hyperintensity.