Deep white matter lesion with bilateral deep bright thalamic appearance is suggestive of ?

Correct Answer: Krabbe's disease
Description: Ans. is 'c' i.e., Krabbe's disease C.T. scan of leucodystrophies ? o C.T. scan of the leucodystrophies shows low density areas in the white matter which are bilateral but not necessary symmetric and becomes more extensive as the disease process progresses. Krabbe's disease On C.T, scan it shows increased density in the thalamus and low density areas in the white matter. Metachromatic leucodystrophy C. T shows diffuse symmetric attenuation of the cerebellar and cerebral white matter. Alexander's disease It is one of the few leucodystrophies that occurs sporadically and is characterized histopathologically by the abundant presence of Rosenthal fibres in the affected brain. Definitive diagnosis thus depends on brain biopsy. o It occurs due to mutation in glial fibrillary acidic protein (GFAP). C.T. scan demonstrates degeneration of the white matter most prominent in the frontal lobes. Canavan's disease o C.T. scan shows diffuse white matter degeneration primarily in the cerebral hemispheres with less involvement in the cerebellum and brainstem.
Category: Pediatrics
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