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Improved Turnaround Times | Median time to first decision: 12 days

Research ArticleBrain
Open Access

Metachromatic Leukodystrophy: A Scoring System for Brain MR Imaging Observations

F. Eichler, W. Grodd, E. Grant, M. Sessa, A. Biffi, A. Bley, A. Kohlschuetter, D.J. Loes and I. Kraegeloh-Mann
American Journal of Neuroradiology November 2009, 30 (10) 1893-1897; DOI: https://doi.org/10.3174/ajnr.A1739
F. Eichler
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W. Grodd
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E. Grant
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M. Sessa
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A. Biffi
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A. Bley
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A. Kohlschuetter
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D.J. Loes
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I. Kraegeloh-Mann
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  • Fig 1.
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    Fig 1.

    Scoring details of the metachromatic leukodystrophy (MLD) MR imaging severity score. Differences between scoring faint (1 point) versus dense appearance (2 points) are shown. A, Periventricular and central involvement in the frontal white matter is categorized as faint (1 point, thin arrow), whereas myelination is preserved in the subcortical U-fibers (0 points, thick arrow). B, Periventricular and central involvement in the frontal white matter is categorized as dense (2 points, thin arrow), and there are areas that lack subcortical U-fiber myelination (2 points, thick arrow). C, Inner atrophy as measured in the third ventricle (arrow).

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    Fig 2.

    Midline of the corpus callosum in advanced stages of MLD. T2 lesion hyperintensities in the midline are reduced compared with the adjacent supratentorial white matter lesion signal intensity (arrow).

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    Fig 3.

    Correlation of age and MR imaging severity score in patients with MLD. The greatest variability in MR imaging lesion severity is seen in the first decade of life. Thereafter, most patients have a severity score of >20. Patients with serial scans have scores with connecting lines.

Tables

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    Table 1:

    Brain MR imaging scoring system for MLD

    Brain AreasScore*Maximum per Area
    Frontal WM6†
        Periventricular012
        Central012
        U-fibers012
    Parieto-occipital WM6†
        Periventricular012
        Central012
        U-fibers012
    Temporal WM6†
        Periventricular012
        Central012
        U-fibers012
    Corpus callosum4†
        Genu012
        Splenium012
    Projection fibers6†
        Internal capsule posterior limb012
        Internal capsule anterior limb012
        Midline pons012
    Cerebral atrophy0122†
    Thalamus011†
    Basal ganglia011†
    Cerebellum2†
        WM01
        Atrophy01
    Total34†
    • Note:—MLD indicates metachromatic leukodystrophy; WM, white matter.

    • * 0 indicates normal; 1, faint hyperintensity; 2, dense hyperintensity.

    • † XXX.

    • View popup
    Table 2:

    Subtypes and initial MR imaging severity scores of patients with MLD

    Patient No.Phenotype*Age at MRIDeep Gray NucleiProjection FibersCerebellumAtrophyMRI Severity Score
    1Late infantile1.92NoNoNoNo1
    2Late infantile2.08YesNoNoNo17
    3Late infantile2.5NoNoNoNo4
    4Late infantile2.5NoNoNoNo4
    5Late infantile2.5NoNoNoNo4
    6Late infantile3NoNoNoNo6
    7Late infantile2.08NoNoNoNo0
    8Late infantile3.66YesNoNoNo21
    9Late infantile7.16YesYesYesYes30
    10Late infantile9.6YesYesYesYes32
    11Juvenile4.84NoNoNoNo12
    12Juvenile4.84NoNoNoNo12
    13Juvenile8.08YesNoNoNo13
    14Juvenile10.25NoNoNoNo3
    15Juvenile3NoNoNoNo2
    16Juvenile4.17NoNoNoNo6
    17Juvenile4.17NoNoNoNo10
    18Juvenile5.66YesYesNoYes26
    19Juvenile6.25YesYesNoYes27
    20Juvenile7NoNoNoNo18
    21Juvenile7.75NoNoNoYes19
    22Juvenile8.25YesYesYesYes30
    23Juvenile9.4NoNoNoNo12
    24Juvenile13.08YesYesNoYes26
    25Juvenile18.42YesYesYesYes28
    26Juvenile27NoYesNoYes24
    27Adult34.5YesYesNoYes22
    28Adult46YesYesNoYes26
    • Note:—MRI indicates MR imaging.

    • * The individual phenotypes, age as well as the MR imaging severity score with its subscores at initial MR imaging are provided.

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American Journal of Neuroradiology: 30 (10)
American Journal of Neuroradiology
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1 Nov 2009
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Metachromatic Leukodystrophy: A Scoring System for Brain MR Imaging Observations
F. Eichler, W. Grodd, E. Grant, M. Sessa, A. Biffi, A. Bley, A. Kohlschuetter, D.J. Loes, I. Kraegeloh-Mann
American Journal of Neuroradiology Nov 2009, 30 (10) 1893-1897; DOI: 10.3174/ajnr.A1739
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F. Eichler, W. Grodd, E. Grant, M. Sessa, A. Biffi, A. Bley, A. Kohlschuetter, D.J. Loes, I. Kraegeloh-Mann
Metachromatic Leukodystrophy: A Scoring System for Brain MR Imaging Observations
American Journal of Neuroradiology Nov 2009, 30 (10) 1893-1897; DOI: 10.3174/ajnr.A1739

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