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Radiology · Neuroimaging: eye of the tiger sign
Sample itemA child has progressive dystonia, dysarthria and pigmentary retinopathy. T2 MRI shows bilateral globus pallidus hypointensity with a central region of hyperintensity. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Pantothenate kinase-associated neurodegeneration
In PKAN, iron deposition darkens the globus pallidus while central gliosis and oedema stay bright, giving the eye of the tiger sign. It is the classic imaging marker of this neurodegeneration with brain iron accumulation.
Option by option
A. Huntington disease
Huntington disease shows caudate atrophy.
B. Tuberous sclerosis
Tuberous sclerosis shows tubers and subependymal nodules.
C. Leigh syndrome
Leigh syndrome shows symmetric basal ganglia and brainstem lesions without iron hypointensity.
D. Pantothenate kinase-associated neurodegeneration (correct)
Correct. Pallidal iron with a central bright spot is the eye of the tiger.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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