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Research ArticleBrain
Open Access

Sensorimotor, Visual, and Auditory Cortical Atrophy in Unverricht-Lundborg Disease Mapped with Cortical Thickness Analysis

P. Koskenkorva, E. Niskanen, J. Hyppönen, M. Könönen, E. Mervaala, H. Soininen, R. Kälviäinen and R. Vanninen
American Journal of Neuroradiology May 2012, 33 (5) 878-883; DOI: https://doi.org/10.3174/ajnr.A2882
P. Koskenkorva
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E. Niskanen
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J. Hyppönen
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M. Könönen
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E. Mervaala
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H. Soininen
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R. Kälviäinen
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R. Vanninen
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    Fig 1.

    Group analysis of CTH. Brain regions demonstrate significantly (P < .0001, FDR corrected) reduced CTH in patients with EPM1 compared with healthy controls.

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    Fig 2.

    Brain regions with significant (P < .01, FDR-corrected) negative correlations between age and CTH in patients with EPM1 (A) and controls (B). Scatterplots indicate correlations between age and mean CTH in individual subjects. The individual mean thicknesses have been calculated on the basis of the regions surviving the statistical threshold.

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    Fig 3.

    Brain regions with significant (P < .05, FDR-corrected) negative correlations between the “Myoclonus with Action” score and CTH in patients with EPM1. Scatterplots indicate correlations between the “Myoclonus with Action” score and mean CTH in individual subjects. The individual mean thicknesses have been calculated on the basis of the regions surviving the statistical threshold.

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    Table 1:

    Basic clinical characteristics and demographics of 53 patients with EPM1 and 70 controlsa)

    Patients with EPM1Controls
    Age (yr)35 ± 11.533 ± 10.7
    Sex (M/F)29/2434/36
    Handedness (R/L/B/unknown)35/6/1/1162/4/3/1
    Age at EPM1 onset (yr)10.7 ± 2.9 (6–25)
    Duration of the disease (yr)24.2 ± 10.4 (4–44)
    UMRS: stimulus sensitivity1.92 ± 1.9 (0–8)
    UMRS: myoclonus with action48.5 ± 28.4 (2–120)
    UMRS: functional tests10.35 ± 7 (0–28)
    • Note:—R indicates right; L, left; B, bilateral.

    • ↵a a Data are presented as means unless otherwise indicated.

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    Table 2:

    Areas of significant difference in CTH between the EPM1 group and controls

    Brain RegionHemisphereMaximum T ValueMNI Coordinates, (x, y, z)a
    Central sulcusRight8.4242, −16, 36
    Left6.69−39, −19, 46
    Precentral sulcusLeft6.05−40, 7, 27
    Paracentral lobuleRight8.115, −27, 50
    Postcentral gyrusRight7.326, −49, 65
    Cingulate sulcusLeft8.06−10, −19, 46
    Postcentral sulcusLeft6.79−24, −40, 60
    Superior frontal gyrusRight6.487, 45, 29
    Calcarine sulcusLeft8.99−20, −70, 6
    Lingual gyrusRight6.525, −68, 3
    Left8.20−3, −76, 5
    Superior temporal sulcusRight5.3254, −16, −14
    Transverse temporal gyrusRight5.3343, −28, 10
    Middle temporal gyrusLeft5.98−48, −66, 4
    Inferior occipital gyrusLeft7.39−27, −89, −17
    • Note:—MNI indicates Montreal Neurological Institute.

    • ↵a MNI coordinates are based on a standard brain template defined by using multiple MR images of healthy controls.

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American Journal of Neuroradiology: 33 (5)
American Journal of Neuroradiology
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Cite this article
P. Koskenkorva, E. Niskanen, J. Hyppönen, M. Könönen, E. Mervaala, H. Soininen, R. Kälviäinen, R. Vanninen
Sensorimotor, Visual, and Auditory Cortical Atrophy in Unverricht-Lundborg Disease Mapped with Cortical Thickness Analysis
American Journal of Neuroradiology May 2012, 33 (5) 878-883; DOI: 10.3174/ajnr.A2882

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Sensorimotor, Visual, and Auditory Cortical Atrophy in Unverricht-Lundborg Disease Mapped with Cortical Thickness Analysis
P. Koskenkorva, E. Niskanen, J. Hyppönen, M. Könönen, E. Mervaala, H. Soininen, R. Kälviäinen, R. Vanninen
American Journal of Neuroradiology May 2012, 33 (5) 878-883; DOI: 10.3174/ajnr.A2882
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