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Pantothenate Kinase-Associated Neurodegeneration: MR Imaging, Proton MR Spectroscopy, and Diffusion MR Imaging Findings

R. Nuri Senera

a From the Department of Radiology, Ege University Hospital, Bornova, Izmir, Turkey



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  		FIG 1. T2-weighted and fluid-attenuated inversion recovery images from the initial examination.

A, T2-weighted image reveals marked hypointensity in the globus pallidi with high signal intensity foci (eye-of-the-tiger appearance).

B, Fluid-attenuated inversion recovery image reveals marked hypointensity of the globus pallidi. Note that the eye-of-the-tiger appearance cannot be detected.

C, Fluid-attenuated inversion recovery image reveals high signal intensity lesions in the deep cerebral white matter and dentate nuclei.



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  		FIG 2. Spectra from the initial examination.

A, Proton MR spectroscopy (1500/40) reveals markedly decreased NAA in the globus pallidi.

B, Spectrum from deep cerebral white matter reveals decreased NAA and increased myoinositol peaks.

C, Spectrum from a normal region in the cerebral parenchyma.



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  		FIG 3. Images obtained at the 10-month follow-up examination.

A, T2-weighted image reveals further hypointense appearance of the globus pallidi. Note loss of the eye-of-the-tiger appearance.

B, b=1000 s/mm2 image from trace diffusion MR imaging sequence (5700/139) reveals low signal intensity in the globus pallidi.

C, Corresponding ADC map (same section as that shown in B) reveals low ADC values (0.45–0.54 x 10-3 mm2/s) in the globus pallidi, compared with the unaffected thalamus (0.77 to 0.83 x 10-3 mm2/s).

D, b=1000 s/mm2 image is negative (normal appearing) for the deep cerebral white matter lesions.

E, Corresponding ADC map (same section as that shown in D) reveals slightly increased ADC values (1.08–1.12 x 10-3 mm2/s) compared with the ADC values from the normal white matter regions (0.78–0.87 x 10-3 mm2/s).





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X