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Research ArticleBRAIN

T1 Hyperintensity in the Pulvinar: Key Imaging Feature for Diagnosis of Fabry Disease

Jun-ichi Takanashi, A. James Barkovich, William P. Dillon, Elliott H. Sherr, Kimberly A. Hart and Seymour Packman
American Journal of Neuroradiology May 2003, 24 (5) 916-921;
Jun-ichi Takanashi
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A. James Barkovich
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William P. Dillon
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Elliott H. Sherr
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Kimberly A. Hart
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Seymour Packman
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  • Fig 1.
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    Fig 1.

    Patient 10.

    A, Axial T1-weighted spin-echo image at the level of basal ganglia shows hyperintense lesions in the bilateral peripheral globus pallidus, lateral pulvinar (arrowhead), and medial occipital cortex.

    B, Axial T2-weighted image spin-echo showing hypointensity in the bilateral globus pallidus (small arrowhead) and putamen (arrowhead).

    C, Noncontrast axial CT scan reveals calcification in the bilateral globus pallidus and medial occipital cortex; however, no abnormal attenuation exists in the pulvinar.

    D and E, Coronal gradient echo images show hypointensity in the putamen and substantia nigra (D, arrowhead) but normal SI in pulvinar (E).

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    Fig 2.

    Patient 8.

    A, T1-weighted spin-echo image at the level of basal ganglia shows hyperintensity in right globus pallidus and bilateral lateral pulvinar (arrowhead).

    B, T2-weighted spin-echo image shows a small hyperintense foci in right frontal white matter and hypointensity in the bilateral lateral putamen (arrowhead) and pulvinar (small arrowhead).

Tables

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    TABLE 1:

    Clinical and MR imaging findings in Fabry disease

    Patient (No.)Age (y)/SexCNS SymptomsIschemic LesionMR Imaging Findings Small Foci of WMDGM Lacunes
    119/MDizziness, syncopeNoneNoneNone
    228/MVertigo, vision lossNoneNoneNone
    330/FNoneNoneNoneNone
    431/MDizziness, decreased visionNoneD, P
    534/MVertigo, double visionNoneS, D, PLeft medial Th
    636/MVisual loss, hemiplegia, seizureNoneD, PBilateral BG
    742/MNoneNoneS, DNone
    846/MNoneNoneS, D, PNone
    950/MVisual disturbanceNoneDNone
    1059/MAphasiaBilateral F, O, left PS, D, PNone
    • Note.—Pt signifies patient; WM, white matter; DGM, deep gray matter; M, male; F, female; TIA, transient ischemic attack; Th, thalamus; BG, basal ganglia; S, subcortical; D, deep; P, periventricular; F, frontal; O, occipital; P, parietal.

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    TABLE 2:

    Signal intensity of deep gray matter

    Patient (No.)GPPutThSNRNDN
    QSI/CSFSI/CCQSI/CSFSI/CCQSI/CSFSI/CCQSI/CSFSI/CCQ/CSF/CCQ/CSF/CC
    A. On T1WI
    1NNNNNNNNNNNNNNNNNN
    2HNNNNNHNNNNNNNNHNN
    3NNNNNNNNNNNNNNNNNN
    4NNNNNNHHHNNNNNNNNN
    5HHNNNHNHHHHHNNHNHH
    6NNNNNHHHNNNNHHNNNN
    7HHHNHNHHHNHNNHNNHN
    8HNNNNNHHHNNNHNNNNN
    9NNNNNNHHHHNNNNNHNN
    10HHNNNNHHHHNNHHHHNN
    B. On T2WI
    1
    2LLLNNNNLLLLNNLLLLN
    3NLNNNNNNNNLNNLNLLN
    4NLNNLNNNNLLNNLNLLN
    5NLNNLNNLNNNNNNNLLN
    6NLLLLNNLNLLNNLLLNN
    7LLLNLNLLLLLLNNNLLL
    8NLLLLNLLNLLNNNNLNN
    9NLLNLNNLNNNNNNNLNN
    10LLLLLLLNNLLLNNNNNN
    • Note.—Pt signifies patient; T1WI, T1-weighted image; T2WI, T2-weighted image; GP, globus pallidus; Put, putamen; Th, thalamus; SN, substantia nigra; RN, red nucleus; DN, dentate nucleus; Q, qualitative analysis; SI/CSF, signal intensity ratio relative to CSF in the lateral ventricle; SI/CC, signal intensity ratio relative to the genu of corpus callosum; H, high; L, low; and N, normal range.

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American Journal of Neuroradiology: 24 (5)
American Journal of Neuroradiology
Vol. 24, Issue 5
1 May 2003
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Cite this article
Jun-ichi Takanashi, A. James Barkovich, William P. Dillon, Elliott H. Sherr, Kimberly A. Hart, Seymour Packman
T1 Hyperintensity in the Pulvinar: Key Imaging Feature for Diagnosis of Fabry Disease
American Journal of Neuroradiology May 2003, 24 (5) 916-921;

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T1 Hyperintensity in the Pulvinar: Key Imaging Feature for Diagnosis of Fabry Disease
Jun-ichi Takanashi, A. James Barkovich, William P. Dillon, Elliott H. Sherr, Kimberly A. Hart, Seymour Packman
American Journal of Neuroradiology May 2003, 24 (5) 916-921;
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  • Pulvinar: Associative role in cortical function and clinical correlations
  • Cerebrovascular Involvement in Fabry Disease: Current Status of Knowledge
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