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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticleBrain
Open Access

Cerebral White Matter Disruption in Creutzfeldt-Jakob Disease

H. Lee, O.S. Cohen, H. Rosenmann, C. Hoffmann, P.B. Kingsley, A.D. Korczyn, J. Chapman and I. Prohovnik
American Journal of Neuroradiology November 2012, 33 (10) 1945-1950; DOI: https://doi.org/10.3174/ajnr.A3125
H. Lee
aFrom the Departments of Psychiatry (H.L., I.P.) and Radiology (I.P.), Mount Sinai School of Medicine, New York, New York
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O.S. Cohen
cDepartments of Neurology (O.S.C., J.C.) and Radiology (C.H.), Sheba Medical Center, and Sieratzky Chair of Neurology, Sackler Faculty of Medicine (A.D.K.), Tel Aviv University, Tel Aviv, Israel
dSackler Faculty of Medicine (O.S.C., J.C.), Tel Aviv University, Tel Aviv, Israel
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H. Rosenmann
bDepartment of Neurology (H.R.), Hadassah University Hospital, Jerusalem, Israel
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C. Hoffmann
cDepartments of Neurology (O.S.C., J.C.) and Radiology (C.H.), Sheba Medical Center, and Sieratzky Chair of Neurology, Sackler Faculty of Medicine (A.D.K.), Tel Aviv University, Tel Aviv, Israel
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P.B. Kingsley
eDepartment of Radiology (P.B.K.), North Shore University Hospital, Manhasset, New York.
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A.D. Korczyn
cDepartments of Neurology (O.S.C., J.C.) and Radiology (C.H.), Sheba Medical Center, and Sieratzky Chair of Neurology, Sackler Faculty of Medicine (A.D.K.), Tel Aviv University, Tel Aviv, Israel
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J. Chapman
cDepartments of Neurology (O.S.C., J.C.) and Radiology (C.H.), Sheba Medical Center, and Sieratzky Chair of Neurology, Sackler Faculty of Medicine (A.D.K.), Tel Aviv University, Tel Aviv, Israel
dSackler Faculty of Medicine (O.S.C., J.C.), Tel Aviv University, Tel Aviv, Israel
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I. Prohovnik
aFrom the Departments of Psychiatry (H.L., I.P.) and Radiology (I.P.), Mount Sinai School of Medicine, New York, New York
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  • Fig 1.
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    Fig 1.

    A flow diagram of voxelwise analysis performed by TBSS. After spatial normalization to a stereotactic space using a default FMRIB58_FA template, tracts are traced in FA, AD, and RD maps. Voxels surviving P < .05 (corrected for multiple comparisons) are overlaid onto a group averaged FA map and their anatomic correspondence is defined by ICBM-DTI-81 white matter and Harvard-Oxford subcortical structural atlases.

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    Fig 2.

    Pattern of reduced fractional anisotropy (green) in patients with CJD compared with healthy normal controls. Results are overlaid onto a representative anatomic scan in Montreal Neurological Institute standard brain (MNI152) stereotactic space. Arrows indicate corresponding anatomic regions. Voxels are considered significant at a threshold of P < .05 (corrected for multiple comparisons).

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    Fig 3.

    Group averaged DTI indices of the significant voxels shown in Fig 1. Group averaged values of (A) FA, (B) MD, (C) AD, and (D) RD are plotted by diagnosis. The error bars represent standard deviations.

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    Fig 4.

    Pearson product-moment correlation between duration of illness and averaged FA over the significant voxels. Open circles represent mean FA values of each patient. The filled circles and error bar represent the mean and standard deviations of FA in controls.

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    Table 1

    Patient characteristics (mean ± SD of numeric variables)

    Total number21
    Age (years)59.4 ± 7.4
    Male/Female13/8
    MMSE20.9 ± 6.7
    Clinical Neurological Scale12.1 ± 5.8
    Duration of illness (months)2.8 ± 2.0
    Survival after MRI (months)4.3 ± 4.7
    Symptoms (%)
        Brain stem dysfunction57%
        Frontal release signs86
        Cerebellar signs90
        Extrapyramidal signs86
        Pyramidal signs76
        Cognitive impairment (MMSE<24)60
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American Journal of Neuroradiology: 33 (10)
American Journal of Neuroradiology
Vol. 33, Issue 10
1 Nov 2012
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Cite this article
H. Lee, O.S. Cohen, H. Rosenmann, C. Hoffmann, P.B. Kingsley, A.D. Korczyn, J. Chapman, I. Prohovnik
Cerebral White Matter Disruption in Creutzfeldt-Jakob Disease
American Journal of Neuroradiology Nov 2012, 33 (10) 1945-1950; DOI: 10.3174/ajnr.A3125

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Cerebral White Matter Disruption in Creutzfeldt-Jakob Disease
H. Lee, O.S. Cohen, H. Rosenmann, C. Hoffmann, P.B. Kingsley, A.D. Korczyn, J. Chapman, I. Prohovnik
American Journal of Neuroradiology Nov 2012, 33 (10) 1945-1950; DOI: 10.3174/ajnr.A3125
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  • White matter abnormalities in healthy E200K carriers may serve as an early biomarker for genetic Creutzfeldt-Jakob disease (gCJD)
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