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Research ArticleNeurodegenerative Disorder Imaging

Quantifying Brain Iron in Hereditary Hemochromatosis Using R2* and Susceptibility Mapping

S.K. Sethi, S. Sharma, S. Gharabaghi, D. Reese, Y. Chen, P. Adams, M.S. Jog and E.M. Haacke
American Journal of Neuroradiology July 2022, 43 (7) 991-997; DOI: https://doi.org/10.3174/ajnr.A7560
S.K. Sethi
aFrom the Department of Radiology (S.K.S., E.M.H.), Wayne State University, Detroit, Michigan
bSpinTech MRI Inc (S.K.S., S.G., E.M.H.), Bingham Farms, Michigan
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S. Sharma
cDepartment of Clinical Neurological Sciences (S.S., M.S.J.), London Health Sciences Centre
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S. Gharabaghi
bSpinTech MRI Inc (S.K.S., S.G., E.M.H.), Bingham Farms, Michigan
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D. Reese
eImaging Research Laboratories (D.R.), Robarts Research Institute, London, Ontario, Canada
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Y. Chen
fDepartment of Neurology (Y.C.), Wayne State University School of Medicine, Detroit, Michigan
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P. Adams
dDivision of Gastroenterology (P.A.), Department of Medicine, Western University, London, Ontario, Canada
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M.S. Jog
cDepartment of Clinical Neurological Sciences (S.S., M.S.J.), London Health Sciences Centre
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E.M. Haacke
aFrom the Department of Radiology (S.K.S., E.M.H.), Wayne State University, Detroit, Michigan
bSpinTech MRI Inc (S.K.S., S.G., E.M.H.), Bingham Farms, Michigan
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Article Figures & Data

Figures

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  • FIG 1.
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    FIG 1.

    Upper row: QSM showing 2 HC (A and B) and 2 subjects with HH (C and D). Lower row: Corresponding R2* maps. Elevated striatal and PT iron is depicted in both subjects with HH (C and D, F and G). For 1 subject (C and F), high iron can be seen in the cuneus and striatal cortex.

  • FIG 2.
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    FIG 2.

    Comparison of susceptibility (parts per billion) between patients with HH and HC. Asterisk indicates P < .05; double asterisks, P < .01; triple asterisks, P < .001.

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    FIG 3.

    Comparison of R2* (s−1) between patients with HH and HC. Double asterisks indicate P < .01; triple asterisks, P < .001.

Tables

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    Table 1:

    Mean age of subjects with HH and healthy controls

    Subjects with HH
    (n = 52)
    No.Mean Age (yr)95% CIP Value
    Men
     HH3058.23 (SD, 12.29)–12.29–2.52.19
     HC1953.32 (SD, 12.82)
    Women
     HH2258.00 (SD, 14.91)–11.59–4.52.38
     HC2854.46 (SD, 13.38)
    • View popup
    Table 2:

    Demographics of patients with HH who underwent MR imaging

    Demographics of Patients with HH
    Age at HH diagnosis (mean) (yr)51.09 (SD, 12.92) (n = 52)
    HandednessRight: 50, left: 2
    Family history of HHYes: 30, no: 21, unknown: 1
    Average duration from time of diagnosis to MR imaging (yr)7.29 (SD, 5.22) (n = 51)
    Genetic diagnosis (n = 60)C282Y homozygous: 41
    H63D homozygous: 2
    C282Y heterozygous: 2
    H63D heterozygous: 1
    Compound heterozygous: 3
    Unknown status: 3
    • View popup
    Table 3:

    Comparative analysis of mean susceptibility (parts per billion) in the DGM nuclei between patients with HH and HCa

    Group StatisticsT Test for Equality of Means
    StructureDiagnosisNo.MeanSDStandard Error of the MeantdfSignificant (2-Tailed)Mean DifferenceStandard Error Difference95% CI of the Difference
    LowerUpper
    CNHC4724.438.71.3−2.0897.040a−6.863.30−13.41−0.31
     HH5231.2921.02.9
    GPHC4785.4820.12.91.0997.2784.574.19−3.7412.89
     HH5280.9121.43.0
    PUTHC4733.0614.42.1−3.3797.001b−19.635.83−31.20−8.07
     HH5252.7037.55.2
    THAHC473.453.60.5−1.7897.079−1.580.89−3.360.19
     HH525.045.10.7
    PTHC4736.4013.52.0−2.6697.009b−9.793.68−17.09−2.48
     HH5246.1921.73.0
    RNHC4794.9124.13.5−4.3297<.001c−23.405.41−34.15−12.66
     HH52118.3229.24.1
    SNHC47129.6922.83.3−2.2697.026a−11.665.15−21.88−1.43
     HH52141.3527.93.9
    DNHC4792.5324.23.5−5.1097<.001c−32.096.3044.59−19.59
     HH52124.6236.55.1
    • ↵a P < .05.

    • ↵b P < .01.

    • ↵c P < .001.

    • View popup
    Table 4:

    Comparative analysis of R2* (s−1) in the deep gray matter nuclei between subjects with HH and HC

    Group StatisticsT Test for Equality of Means
    StructureDiagnosisNo.MeanSDStandard Error of the MeantdfSignificant (2-Tailed)Mean DifferenceStandard Error Difference95% Confidence Interval of the Difference
    LowerUpper
    CNHC4725.233.00.42.8997.005a−2.941.02−4.95−0.92
     HH5228.176.30.9
    GPHC4740.124.60.71.8897.064−2.221.18−4.570.13
     HH5242.356.80.9
    PUTHC4729.674.90.74.1997<.001b−5.981.43−8.81−3.15
     HH5235.658.61.2
    THAHC4721.011.50.2−3.5997.001a−1.310.37−2.04−0.59
     HH5222.322.10.3
    PTHC4724.153.00.4−4.3697<.001b−3.200.73−4.66−1.74
     HH5227.354.20.6
    RNHC4736.265.30.8−2.6597.009a−3.461.31−6.05−0.87
     HH5239.727.41.0
    SNHC4736.604.50.7−1.9497.056−2.021.04-4.080.05
     HH5238.625.70.8
    DNHC4732.235.20.8−3.9597<.001b−6.281.59−9.43−3.13
     HH5238.509.71.3
    • ↵a P <.01.

    • ↵b P <.001.

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American Journal of Neuroradiology: 43 (7)
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S.K. Sethi, S. Sharma, S. Gharabaghi, D. Reese, Y. Chen, P. Adams, M.S. Jog, E.M. Haacke
Quantifying Brain Iron in Hereditary Hemochromatosis Using R2* and Susceptibility Mapping
American Journal of Neuroradiology Jul 2022, 43 (7) 991-997; DOI: 10.3174/ajnr.A7560

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Quantifying Brain Iron in Hemochromatosis
S.K. Sethi, S. Sharma, S. Gharabaghi, D. Reese, Y. Chen, P. Adams, M.S. Jog, E.M. Haacke
American Journal of Neuroradiology Jul 2022, 43 (7) 991-997; DOI: 10.3174/ajnr.A7560
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