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Research ArticlePediatric Neuroimaging
Open Access

Diffusion Tensor MR Imaging in Children with Pantothenate Kinase−Associated Neurodegeneration with Brain Iron Accumulation and Their Siblings

R. Awasthi, R.K. Gupta, R. Trivedi, J.K. Singh, V.K. Paliwal and R.K.S. Rathore
American Journal of Neuroradiology March 2010, 31 (3) 442-447; DOI: https://doi.org/10.3174/ajnr.A1849
R. Awasthi
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R.K. Gupta
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R. Trivedi
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J.K. Singh
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V.K. Paliwal
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R.K.S. Rathore
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    Fig 1.

    A, Color-coded FA map overlaid on an MD map at the level of the basal ganglia shows placement of regions of interest at the CN (square), putamen (solid rectangle), GP (hollow rectangle), and ALIC (ellipse). B, Color-coded FA map overlaid on the MD map at the level of the SN shows region-of-interest placement on the SN. Note that the cutoff value for the color-coded FA map overlaid on the MD map for display is kept at 0. 20.

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    Fig 2.

    A–C, MR imaging of a 6-year-old male healthy control shows normal distribution of white and gray matter on the T2-weighted image (A), the T2*-weighted GRE image (B), and the color-coded FA map overlaid on the MD map (C). D–F, A 6-year-old male sibling of the patient with PKAN G–J. Findings of T2-weighted (D) and T2*-weighted GRE (E) images appear normal in the sibling; however, the quantified color-coded FA map overlaid on the MD map (F) shows intermediate FA values between patients and controls. G–I, In a 10-year-old male patient with PKAN, T2-weighted (G) and T2*-weighted GRE (H) images show the eye-of-the-tiger sign. The color-coded FA map overlaid on the MD map (I) shows high FA values (arrow) in the GP (0.20) compared with those in both the control (0.10) and his sibling (0.12). J, Segmented FA map of the patient overlaid on T2. The hypointense regions are shown in yellow.

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    Fig 3.

    A–C, Imaging of a 6-year-old male healthy control shows normal distribution of white and gray matter on the T2-weighted image (A), the T2*-weighted GRE image (B), and the color-coded FA map (C) at the level of the SN. D–F, In a 6-year-old male sibling of the patient with PKAN shown in Fig G–I, findings on T2-weighted (D) and T2*-weighted GRE (E) images appear normal; however, the color-coded FA map overlaid on the MD map (F) shows higher FA values (0.22, arrow) than those in the control (0.18). G–I, In imaging of a 10-year-old male patient with PKAN, findings on T2-weighted (G) and T2*-weighted GRE (H) images are normal-appearing. The color-coded FA map overlaid on the MD map (I) from the patient shows high FA values (0.30) in the SN (arrow) compared with those in both the control and his sibling.

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    Table 1:

    Summary of FA and MD (×10−3 mm2s−1) values quantified in patients with PKAN, their siblings, and age-matched healthy controls in deep gray matter nuclei and ALIC

    SubjectCNPutamenGPSNALIC
    FAMDFAMDFAMDFAMDFAMD
    Control (n = 5)0.10 ± 0.010.76 ± 0.020.08 ± 0.010.70 ± 0.010.10 ± 0.010.70 ± 0.010.19 ± 0.010.69 ± 0.110.35 ± 0.010.71 ± 0.02
    Sibling (n = 5)0.10 ± 0.010.77 ± 0.020.08 ± 0.010.71 ± 0.010.11 ± 0.020.72 ± 0.020.22 ± 0.020.69 ± 0.120.35 ± 0.010.72 ± 0.01
    Patient (n = 7)0.10 ± 0.010.77 ± 0.030.08 ± 0.010.70 ± 0.020.18 ± 0.030.75 ± 0.060.32 ± 0.020.67 ± 0.100.36 ± 0.010.71 ± 0.03
    P valuea.17.23.31.10<.001<.001<.001.71.83.16
    • a ANOVA.

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    Table 2:

    Multiple comparisons, using the Bonferroni test for FA and MD valuesa of deep gray matter nuclei, among the subject groups

    RegionVariableGroup A vs Group BMean Diff.95% Confidence IntervalP Value
    ABLower BoundUpper Bound
    GPFAControlSibling−0.020.03−0.003.009
    ControlPatient−0.08−0.10−0.07<.001
    SiblingPatient−0.07−0.08−0.06<.001
    MDControlPatient−0.04−0.06−0.02<.001
    SiblingPatient−0.02−0.04−0.004.013
    SNFAControlSibling−0.04−0.05−0.03<.001
    ControlPatient−0.13−0.14−0.13<.001
    SiblingPatient−0.09−0.10−0.09<.001
    • a Only in the region that showed a significant difference on the Bonferroni post hoc test.

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American Journal of Neuroradiology: 31 (3)
American Journal of Neuroradiology
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Cite this article
R. Awasthi, R.K. Gupta, R. Trivedi, J.K. Singh, V.K. Paliwal, R.K.S. Rathore
Diffusion Tensor MR Imaging in Children with Pantothenate Kinase−Associated Neurodegeneration with Brain Iron Accumulation and Their Siblings
American Journal of Neuroradiology Mar 2010, 31 (3) 442-447; DOI: 10.3174/ajnr.A1849

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Diffusion Tensor MR Imaging in Children with Pantothenate Kinase−Associated Neurodegeneration with Brain Iron Accumulation and Their Siblings
R. Awasthi, R.K. Gupta, R. Trivedi, J.K. Singh, V.K. Paliwal, R.K.S. Rathore
American Journal of Neuroradiology Mar 2010, 31 (3) 442-447; DOI: 10.3174/ajnr.A1849
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