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OtherPediatric Neuroimaging

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

R. Nuri Sener
American Journal of Neuroradiology September 2003, 24 (8) 1690-1693;
R. Nuri Sener
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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.

  • Fig 2.
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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 × 10−3 mm2/s) in the globus pallidi, compared with the unaffected thalamus (0.77 to 0.83 × 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 × 10−3 mm2/s) compared with the ADC values from the normal white matter regions (0.78–0.87 × 10−3 mm2/s).

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American Journal of Neuroradiology: 24 (8)
American Journal of Neuroradiology
Vol. 24, Issue 8
1 Sep 2003
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R. Nuri Sener
Pantothenate Kinase-Associated Neurodegeneration: MR Imaging, Proton MR Spectroscopy, and Diffusion MR Imaging Findings
American Journal of Neuroradiology Sep 2003, 24 (8) 1690-1693;

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Pantothenate Kinase-Associated Neurodegeneration: MR Imaging, Proton MR Spectroscopy, and Diffusion MR Imaging Findings
R. Nuri Sener
American Journal of Neuroradiology Sep 2003, 24 (8) 1690-1693;
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