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Research ArticlePEDIATRICS

Thiamine Deficiency in Infants: MR Findings in the Brain

Liora Kornreich, Efrat Bron-Harlev, Chen Hoffmann, Michael Schwarz, Osnat Konen, Tommy Schoenfeld, Rachel Straussberg, Elhanan Nahum, Abu-Kishk Ibrahim, Gideon Eshel and Gadi Horev
American Journal of Neuroradiology August 2005, 26 (7) 1668-1674;
Liora Kornreich
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Efrat Bron-Harlev
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Chen Hoffmann
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Michael Schwarz
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Osnat Konen
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Tommy Schoenfeld
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Rachel Straussberg
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Elhanan Nahum
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Abu-Kishk Ibrahim
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Gideon Eshel
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Gadi Horev
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    Fig 1.

    Patient 1. Images at presentation (A) and follow-up 5 (B–E) and 45 (F) days later.

    A, Axial T2-weighted image shows abnormal hyperintensity in the mammillary bodies (arrow) and tectum.

    B–D, Axial T2-weighted images show abnormal hyperintensity in the periaqueductal region, thalami, basal ganglia, and frontal area. Lesions are bilateral and symmetric.

    E, DWI (ADC) shows restricted diffusion in the basal ganglia. Thalami have hypointensity and hyperintensity, which are presumed to represent cytotoxic and vasogenic edema, respectively.

    F, Axial T2-weighted image shows diffuse parenchymal loss, severe atrophy of the caudate nuclei, and necrosis of the putamina.

  • Fig 2.
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    Fig 2.

    Patient 2. Axial T2-weighted images.

    A, At presentation, large area of hyperintensity is present in the pons.

    B, Involvement of the frontal region, up to the motor cortex, is extensive.

  • Fig 3.
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    Fig 3.

    Patient 4. Axial images at presentation.

    A, T2-weighted image.

    B, T1-weighted contrast-enhanced image shows extensive frontal injury. Note enhancement of both cortex and white matter.

  • Fig 4.
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    Fig 4.

    Patient 5.

    A, T2-weighted image shows subtle abnormal findings, slight hyperintensity of the anterior frontal region, and localized areas of blurring of the cortical stripe.

    B, DWI (ADC) indicates restricted diffusion compatible with cytotoxic edema.

  • Fig 5.
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    Fig 5.

    Patient 6. Proton MRS image from the periaqueductal region (TR/TE = 1500/144).

    A, At presentation. Note the negative doublet of lactate. NAA/Cr ratio is reduced 1.11.

    B, Five weeks later, the lactate doublet is no longer seen. NAA peak is higher than before.

Tables

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    TABLE 1:

    Clinical findings in six infants with thiamine deficiency

    Pt.No.Age(months)GenderLactate LevelsOutcomeLength of Follow-up (months)
    BloodCSF
    18.5F↑↑Dysphagia; severe DD4
    24.5F↑↑Dysphagia; mild DD5
    310M↑↑Dysphagia; moderate DD4
    44.5FN↑Mild DD5
    52F↑↑Complete recovery2
    65F↑↑Mild DD; ataxia4
    • Note.—↑ indicates elevated; N, normal; DD, developmental delay.

    • View popup
    TABLE 2:

    Imaging findings in six children with thiamine deficiency

    PatientNo.Date ofMRPeriaqueductalDWBrain stemDWTectumMammillaryBodiesThalamiDWCaudateDWPutamenDWFrontal Cortexand WMDWVolumeLoss
    125.9.03−N−N++−N−N−N−N−
    1.10.03+C,V+N+++C,V+C+C+C−
    7.11.03+ImpN−NNecrosisAtrophy+NecrosisNHem Atrophy NecrosisNHem Atrophy NecrosisNLaminar necrosis LeukomalaciaN+
    25.11.03+N+C−++C−C−N+C−
    B1→
    10.11.03−NA−NA−++ImpNA−NA−NAImp cortexNA−
    26.11.03−N+N−Atrophy−N−N−NLaminar necrosis LeukomalaciaN+
    33.11.03+NA−NA−−+NA−NA+NA+NA+
    B1→
    17.11.03+ImpN−N−Atrophy+ImpN+WorseC+C+ImpNWorse
    425.9.03+V+N−++V−N+N+V+
    8.11.03−N−N−−ImpN+N+N+ImpNWorse
    56.11.03−N−N−NA−N−N−N−C−
    613.10.03+N−N−++N−N+N+N−
    B1→
    17.11.03−N−N−−−N−NNecrosisN+N−
    9.2.04−N−N−−−N−NAtrophyN+N+
    • Note.—+ indicates high signals on T2, FLAIR, or PD; −, normal signals; DW, diffusion-weighted images; C, cytotoxic edema; V, vasogenic edema; WM, white matter; NA, not available. N, normal or no signs of acute edema; Imp, improvement; Hem, hemorrhage; B1, start of treatment with B1.

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American Journal of Neuroradiology: 26 (7)
American Journal of Neuroradiology
Vol. 26, Issue 7
1 Aug 2005
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Liora Kornreich, Efrat Bron-Harlev, Chen Hoffmann, Michael Schwarz, Osnat Konen, Tommy Schoenfeld, Rachel Straussberg, Elhanan Nahum, Abu-Kishk Ibrahim, Gideon Eshel, Gadi Horev
Thiamine Deficiency in Infants: MR Findings in the Brain
American Journal of Neuroradiology Aug 2005, 26 (7) 1668-1674;

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Thiamine Deficiency in Infants: MR Findings in the Brain
Liora Kornreich, Efrat Bron-Harlev, Chen Hoffmann, Michael Schwarz, Osnat Konen, Tommy Schoenfeld, Rachel Straussberg, Elhanan Nahum, Abu-Kishk Ibrahim, Gideon Eshel, Gadi Horev
American Journal of Neuroradiology Aug 2005, 26 (7) 1668-1674;
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