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Hyperintensity in the pulvinar on T1-weighted images is a common finding in Fabry disease, likely reflecting the presence of calcification. Although other mineralizing abnormalities may result in calcification of deep gray nuclei, exclusive involvement of the pulvinar may be distinctively characteristic to Fabry disease. Increased CBF in the posterior circulation, particularly the thalamus, suggests that the dystrophic calcification is secondary to cerebral hyperfusion and selective vulnerability of the pulvinar and adjacent thalamic nuclei. The finding of isolated pulvinar hyperintensity on T1-weighted images should suggest Fabry disease, particularly when seen in conjunction with other nonspecific neuroradiologic manifestations of the disease. |
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acral sensory symptoms
angiokeratoma
calcification,intracranial
CAT scan,abnormal
cerebrovascular accident
Fabry's disease
MRI
MRI,abnormal
MRI,gradient-echo
MRI,high signal foci on
MRI,T1 weighted high signal foci
skin,lesions in neurologic disorders
thalamus,lesion of
thalamus,lesion of-bilateral
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