Cerebral White Matter Disruption in Creutzfeldt-Jakob Disease

Human prion diseases are known to cause gray matter degeneration in specific cerebral structures, but evidence for white matter involvement is scarce. We used DTI to test the hypothesis that white matter integrity is disrupted in human CJD during the early stages of the disease. Twenty-one patients...

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Bibliographic Details
Published in:American journal of neuroradiology : AJNR 2012-11, Vol.33 (10), p.1945-1950
Main Authors: LEE, H, COHEN, O. S, ROSENMANN, H, HOFFMANN, C, KINGSLEY, P. B, KORCZYN, A. D, CHAPMAN, J, PROHOVNIK, I
Format: Article
Language:English
Subjects:
Biological and medical sciences
Brain
Brain - pathology
Creutzfeldt-Jakob Syndrome - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Humans
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Imaging - methods
Male
Medical sciences
Middle Aged
Nerve Fibers, Myelinated - pathology
Nervous system
Neurology
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Reproducibility of Results
Sensitivity and Specificity
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Summary:Human prion diseases are known to cause gray matter degeneration in specific cerebral structures, but evidence for white matter involvement is scarce. We used DTI to test the hypothesis that white matter integrity is disrupted in human CJD during the early stages of the disease. Twenty-one patients with the E200K variant of CJD and 19 controls participated in DTI studies conducted on a 1.5T MR imaging scanner. The data were quantitatively analyzed and mapped with a voxelwise TBSS method. We found significant reductions of FA in patients with CJD in distinct and functionally relevant white matter pathways, including the corticospinal tract, internal capsule, external capsule, fornix, and posterior thalamic radiation. Moreover, these FA deficits increased with disease duration, and were mainly determined by increase of radial diffusivity, suggesting elevated permeability of axonal membranes. The findings suggest that some of the symptoms of CJD may be caused by a functional dysconnection syndrome, and that the leukoencephalopathy is progressive and detectable fairly early in the course of the disease.
ISSN:0195-6108
1936-959X
DOI:10.3174/ajnr.A3125