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Research ArticlePediatric Neuroimaging

Acute and Chronic Kernicterus: MR Imaging Evolution of Globus Pallidus Signal Change during Childhood

J. Gburek-Augustat, I. Sorge, M. Stange, J. Kern, A. Merkenschlager, T. Nägele and I. Krägeloh-Mann
American Journal of Neuroradiology August 2023, DOI: https://doi.org/10.3174/ajnr.A7948
J. Gburek-Augustat
aFrom the Division of Neuropaediatrics (J.G.-A., A.M.), Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany
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I. Sorge
bDepartment of Pediatric Radiology (I.S.), University Hospital for Children and Adolescents, University Leipzig, Germany
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M. Stange
cDepartment of Pediatrics (M.S.), University Hospital Halle, Halle, Germany
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J. Kern
dDepartment of Paediatric Neurology (J.K., I.K.-M.), University Children's Hospital, Tübingen, Germany
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A. Merkenschlager
aFrom the Division of Neuropaediatrics (J.G.-A., A.M.), Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany
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T. Nägele
eDepartment of Diagnostic and Interventional Neuroradiology (T.N.), Tübingen University Hospital, Tübingen, Germany.
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I. Krägeloh-Mann
dDepartment of Paediatric Neurology (J.K., I.K.-M.), University Children's Hospital, Tübingen, Germany
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    FIG 1.

    Examples illustrating the various patterns observed with respect to bilateral signal change in the GP in kernicterus. The upper row displays pattern I, characterized by a diffuse signal increase on T1WI (arrows), while T2WI shows no abnormalities (sample case one, 2 weeks). The middle row represents pattern IIa, where T1WI appears normal, but there is a diffuse signal increase on T2WI (arrows) and FLAIR (arrows), (sample case three, 11 months of age). The lower row shows pattern IIb, featuring normal T1WI findings and a signal increase on T2WI (arrows) and FLAIR (arrows), limited to the borders (sample case two, 2 years 10 months of age).

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    FIG 2.

    The temporal progression of the GP signal changes in 8 patients. A bilateral signal change in the GP is the characteristic sign of kernicterus. In the neonatal period, hyperintensity is observed on T1WI (arrows). However, at approximately 2 months of age, a “blind diagnostic window” is encountered, where neither T1WI nor T2WI/FLAIR show abnormal findings. Subsequently, during infancy, there is signal hyperintensity of the entire GP on T2WI/FLAIR (arrows). After around 2 years, the signal hyperintensity on T2/FLAIR is limited to the borders of the GP (arrows). IR indicates inversion recovery; corr., corrected age related to gestational age.

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    FIG 3.

    Case 2 demonstrates the typical temporal sequence in a single patient (patient 2). The initial MR imaging was performed at approximately 2 months of age, revealing no abnormalities (illustrated on T1WI, left). At 9 months of age (middle), the entire GP exhibited a diffuse signal increase on T2WI (pattern IIa, arrows). At 34 months of age (right), a signal increase on T2WI was observed solely at the borders of the GP (pattern IIb, arrows).

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    FIG 4.

    Hippocampal volume loss is illustrated in patient 8 at the age of 11.5 years. The coronal T1WI (left) displays the head of the hippocampus only as filiform structures (arrows), resulting in enlarged temporal horns, as also depicted in the axial T2WI (arrows, middle). Both the external and internal spaces are globally enlarged (axial T2WI, right). The right images highlights the bilateral signal changes in the GP (arrows), indicative of pattern IIb.

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    Table 1:

    Absolute inclusion and exclusion criteria

    Inclusion CriteriaExclusion Criteria
    Dyskinetic CPMovement disorder with onset after infancy
    Developmental delay since neonatal periodProgressive disorder
    Hyperbilirubinemia in neonatal periodOther explanatory causes
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    Table 2:

    Relative inclusion criteria

    Hearing impairment
    Vertical gaze palsy
    Dental enamel dysplasia
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J. Gburek-Augustat, I. Sorge, M. Stange, J. Kern, A. Merkenschlager, T. Nägele, I. Krägeloh-Mann
Acute and Chronic Kernicterus: MR Imaging Evolution of Globus Pallidus Signal Change during Childhood
American Journal of Neuroradiology Aug 2023, DOI: 10.3174/ajnr.A7948

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Acute and Chronic Kernicterus: MR Imaging Evolution of Globus Pallidus Signal Change during Childhood
J. Gburek-Augustat, I. Sorge, M. Stange, J. Kern, A. Merkenschlager, T. Nägele, I. Krägeloh-Mann
American Journal of Neuroradiology Aug 2023, DOI: 10.3174/ajnr.A7948
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