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Research ArticlePediatrics
Open Access

MR Imaging Detection of CNS Lesions in Tuberous Sclerosis Complex: The Usefulness of T1WI with Chemical Shift Selective Images

H. Fujii, N. Sato, Y. Kimura, M. Mizutani, M. Kusama, N. Sumitomo, E. Chiba, Y. Shigemoto, M. Takao, Y. Takayama, M. Iwasaki, E. Nakagawa and H. Mori
American Journal of Neuroradiology August 2022, 43 (8) 1202-1209; DOI: https://doi.org/10.3174/ajnr.A7573
H. Fujii
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
eDepartment of Radiology (H.F., H.M.), Jichi Medical University, School of Medicine, Shimotsuke, Tochigi, Japan
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  • ORCID record for H. Fujii
N. Sato
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
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Y. Kimura
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
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M. Mizutani
bPathology and Laboratory Medicine (M.M., M.T.)
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M. Kusama
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
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N. Sumitomo
cChild Neurology (N.Sumitomo, E.N.)
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E. Chiba
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
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Y. Shigemoto
aFrom the Departments of Radiology (H.F., N.Sato, Y.K., M.K., E.C., Y.S.)
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M. Takao
bPathology and Laboratory Medicine (M.M., M.T.)
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Y. Takayama
dNeurosurgery (Y.T., M.I.), National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
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M. Iwasaki
dNeurosurgery (Y.T., M.I.), National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
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E. Nakagawa
cChild Neurology (N.Sumitomo, E.N.)
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H. Mori
eDepartment of Radiology (H.F., H.M.), Jichi Medical University, School of Medicine, Shimotsuke, Tochigi, Japan
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  • FIG 1.
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    FIG 1.

    Study flow chart. NCNP indicates National Center of Neurology and Psychiatry.

  • FIG 2.
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    FIG 2.

    Patient 19. A 26-month-old boy. Axial T2WI (A), FLAIR (B), T1WI (C), T1WI-MTC (D), and T1WI-CHESS (E). T2WI and FLAIR images demonstrate swelling of the cortex and subcortical hyperintensity, plus a linear hyperintensity extending to the ventricle, representing cortical tubers and RMLs, respectively (A and B, arrowheads and arrows, respectively). On T1WI, several RMLs can be seen as hyperintensity (C, arrows). The RMLs are more apparent on T1WI-MTC and especially on T1WI-CHESS (D and E, arrows).

  • FIG 3.
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    FIG 3.

    Patient 4. A 29-year-old woman. Axial T2WI (A), FLAIR (B), T1WI (C), T1WI-MTC (D), and T1WI-CHESS (E). T2WI and FLAIR images demonstrate swollen cortices, some of which are accompanied by subcortical hyperintensities, representing cortical tubers (A and B, arrowheads). Linear hyperintensities in the subcortical area extending to the ventricle are observed, representing the RMLs (A and B, arrows). On T1WI, an RML is found in the right parietal lobe (C, arrow). T1WI-TMC and T1WI-CHESS are able to detect more RMLs than T1WI, T2WI, and FLAIR (D and E, arrows). The RMLs are more apparent on T1WI-CHESS than on T1WI-MTC (D and E, arrows). Although no obvious cortical tuber is shown by T1WI, both T1WI-MTC and T1WI-CHESS detect a cortical tuber as a slight hyperintensity (D and E, arrowheads).

  • FIG 4.
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    FIG 4.

    Comparison of the CRtuber among T1WI, T1WI-MTC, and T1WI-CHESS.

  • FIG 5.
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    FIG 5.

    Comparison of the CRRML among T1WI, T1WI-MTC, and T1WI-CHESS.

  • FIG 6.
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    FIG 6.

    MR imaging findings of the surgical case, a 20-month-old boy with repeat epileptic seizures. A, Axial T2WI shows swelling of the cortex and subcortical hyperintensity (arrows), consistent with a cortical tuber. No other lesions are noted on T1WI, T2WI, and FLAIR (not shown); suggesting focal cortical dysplasia or a solitary cortical tuber. B, Axial T1WI-CHESS shows marked hyperintensity in the swollen cortex and subcortical white matter (arrows). C–E, Axial T1WI-CHESS shows several RMLs as linear hyperintense areas (arrows). On the basis of these findings, the patient was diagnosed with TSC.

  • FIG 7.
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    FIG 7.

    Pathologic findings of the surgical case, a 20-month-old boy with repeat epileptic seizures. A, Many balloon cells are seen in the thickened cortex (arrowheads as examples). B, In subcortical WM, many balloon cells (arrowheads as examples) are seen in the left part of the white rectangle. Rare balloon cells (arrow) are present in the right part of the rectangle (star). C and D, Many balloon cells are observed in the subcortical white matter (arrowheads) and are immunoreactive for a monoclonal antibody against vimentin (1:400, M7020; Dako). A–C, Monoclonal antivimentin antibody. D, Hematoxylin-eosin staining. Scale bars: A and B, 500 μm. C, 100 μm. D, 10 μm.

Tables

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    Table 1:

    The number of CNS lesions in each sequencea

    T2WIFLAIRT1WIT1WI-MTCT1WI-CHESS
    Cortical tuber17.1 (SD, 10.8)17.5 (SD, 10.8)1.1 (SD, 2.3)10.2 (SD, 6.7)12.2 (SD, 7.7)
    RML3.56 (SD, 2.3)10.5 (SD, 8.1)1.4 (SD, 3.9)20.4 (SD, 12.1)24.8 (SD, 15.4)
    Subependymal nodule6.2 (SD, 4.3)4.5 (SD, 3.0)5.1 (SD, 3.2)6.4 (SD, 3.7)7.2 (SD, 3.9)
    • ↵a Data are mean (SD).

    • View popup
    Table 2:

    Interobserver reproducibility of the number of CNS lesions in each sequence

    T2WIFLAIRT1WIT1WI-MTCT1WI-CHESS
    Cortical tuber0.9590.9030.9840.7860.768
    RML0.6480.8660.8870.8830.799
    Subependymal nodule0.8260.6180.8220.9470.899
    • View popup
    Table 3:

    Comparison of the detection of CNS lesions among T1WI, T1WI-MTC, and T1WI-CHESS

    Multiple Comparison (P Value)
    T1WI vs. T1WI-MTCT1WI vs. T1WI-CHESST1WI-MTC vs. T1WI-CHESS
    Cortical tuber<.001a<.001a.198
    RML.001a<.001a.022a
    Subependymal nodule.007a<.001a.35
    • ↵a P  < .05.

    • View popup
    Table 4:

    Comparison of the detection of CNS lesions among T2WI, FLAIR, and T1WI-CHESS

    Multiple Comparison (P Value)
    T2WI vs FLAIRT2WI vs T1WI-CHESSFLAIR v. T1WI-CHESS
    Cortical tuber1.014a.011a
    RML.007a<.001a.014a
    Subependymal nodule.027a.085<.001a
    • ↵a P < .05.

    • View popup
    Table 5:

    Interobserver reproducibility of CRtuber and CRRML in each sequence

    T1WIT1WI-MTCT1WI-CHESS
    CRtuber0.7880.8630.797
    CRRML0.7130.8840.792
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American Journal of Neuroradiology: 43 (8)
American Journal of Neuroradiology
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1 Aug 2022
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H. Fujii, N. Sato, Y. Kimura, M. Mizutani, M. Kusama, N. Sumitomo, E. Chiba, Y. Shigemoto, M. Takao, Y. Takayama, M. Iwasaki, E. Nakagawa, H. Mori
MR Imaging Detection of CNS Lesions in Tuberous Sclerosis Complex: The Usefulness of T1WI with Chemical Shift Selective Images
American Journal of Neuroradiology Aug 2022, 43 (8) 1202-1209; DOI: 10.3174/ajnr.A7573

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MR Imaging of CNS Lesions in Tuberous Sclerosis
H. Fujii, N. Sato, Y. Kimura, M. Mizutani, M. Kusama, N. Sumitomo, E. Chiba, Y. Shigemoto, M. Takao, Y. Takayama, M. Iwasaki, E. Nakagawa, H. Mori
American Journal of Neuroradiology Aug 2022, 43 (8) 1202-1209; DOI: 10.3174/ajnr.A7573
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