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Research ArticlePEDIATRICS
Open Access

Volumetric Description of Brain Atrophy in Neuronal Ceroid Lipofuscinosis 2: Supratentorial Gray Matter Shows Uniform Disease Progression

U. Löbel, J. Sedlacik, M. Nickel, S. Lezius, J. Fiehler, I. Nestrasil, A. Kohlschütter and A. Schulz
American Journal of Neuroradiology October 2016, 37 (10) 1938-1943; DOI: https://doi.org/10.3174/ajnr.A4816
U. Löbel
aFrom the Departments of Diagnostic and Interventional Neuroradiology (U.L., J.S., J.F.)
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  • ORCID record for U. Löbel
J. Sedlacik
aFrom the Departments of Diagnostic and Interventional Neuroradiology (U.L., J.S., J.F.)
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M. Nickel
bPediatrics (M.N., A.K., A.S.)
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S. Lezius
cMedical Biometry and Epidemiology (S.L.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany
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J. Fiehler
aFrom the Departments of Diagnostic and Interventional Neuroradiology (U.L., J.S., J.F.)
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I. Nestrasil
dDepartment of Pediatrics (I.N.), University of Minnesota, Minneapolis, Minnesota.
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A. Kohlschütter
bPediatrics (M.N., A.K., A.S.)
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A. Schulz
bPediatrics (M.N., A.K., A.S.)
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    Fig 1.

    Progressive brain atrophy in CLN2 disease. Baseline 3D-T1-weighted MPRAGE images in axial, coronal, and sagittal planes (left to right) of patient 457–1 (A), segmented brain volumes (B), and segmented brain volumes 4 years later (C).

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    Fig 2.

    Age dependence of segmented brain volumes, logarithmic fit functions, and confidence intervals.

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    Table 1:

    Correlation of brain volumes with patient age and clinical motor-language-visual scores

    Patient AgeClinical Score
    Correlation Coefficient rP ValueaCorrelation Coefficient rP Valuea
    ROI
        Lateral ventricles0.606.001−0.692<.001
        Supratentorial cortical GM−0.913<.0010.862<.001
        Supratentorial WM−0.865<.0010.735<.001
        Basal ganglia/thalamus−0.832<.0010.758<.001
        Cerebellar GM−0.659<.0010.609.001
        Cerebellar WM−0.830<.0010.638<.001
    Clinical score−0.781<.001NA
    • Note:—NA indicates not applicable.

    • ↵a All P values are statistically significant.

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    Table 2:

    Correlation of brain volumes with patient age and clinical motor-language-visual scores for patients with very low clinical scores (<1)

    Patient AgeClinical Scores <1
    Correlation Coefficient rP ValueCorrelation Coefficient rP Value
    ROI
        Lateral ventricles−0.068.810NANA
        Supratentorial cortical GM−0.903<.001aNANA
        Supratentorial WM0.111.695NANA
        Basal ganglia/thalamus−0.822<.001aNANA
        Cerebellar GM−0.548.035aNANA
        Cerebellar WM−0.795<.001aNANA
    Clinical scoreNANANANA
    • Note:—NA indicates not applicable.

    • ↵a Statistically significant.

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American Journal of Neuroradiology: 37 (10)
American Journal of Neuroradiology
Vol. 37, Issue 10
1 Oct 2016
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Cite this article
U. Löbel, J. Sedlacik, M. Nickel, S. Lezius, J. Fiehler, I. Nestrasil, A. Kohlschütter, A. Schulz
Volumetric Description of Brain Atrophy in Neuronal Ceroid Lipofuscinosis 2: Supratentorial Gray Matter Shows Uniform Disease Progression
American Journal of Neuroradiology Oct 2016, 37 (10) 1938-1943; DOI: 10.3174/ajnr.A4816

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Volumetric Description of Brain Atrophy in Neuronal Ceroid Lipofuscinosis 2: Supratentorial Gray Matter Shows Uniform Disease Progression
U. Löbel, J. Sedlacik, M. Nickel, S. Lezius, J. Fiehler, I. Nestrasil, A. Kohlschütter, A. Schulz
American Journal of Neuroradiology Oct 2016, 37 (10) 1938-1943; DOI: 10.3174/ajnr.A4816
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