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

Research ArticleBrain
Open Access

Combined Diffusion Imaging and MR Spectroscopy in the Diagnosis of Human Prion Diseases

D. Galanaud, S. Haik, M.G. Linguraru, J.-P. Ranjeva, B. Faucheux, E. Kaphan, N. Ayache, J. Chiras, P. Cozzone, D. Dormont and J.-P. Brandel
American Journal of Neuroradiology August 2010, 31 (7) 1311-1318; DOI: https://doi.org/10.3174/ajnr.A2069
D. Galanaud
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S. Haik
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M.G. Linguraru
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J.-P. Ranjeva
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B. Faucheux
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E. Kaphan
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N. Ayache
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J. Chiras
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P. Cozzone
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D. Dormont
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J.-P. Brandel
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Abstract

BACKGROUND AND PURPOSE: The physiopathologic bases underlying the signal intensity changes and reduced diffusibility observed in prion diseases (TSEs) are still poorly understood. We evaluated the interest of MRS combined with DWI both as a diagnostic tool and a way to understand the mechanism underlying signal intensity and ADC changes in this setting.

MATERIALS AND METHODS: We designed a prospective study of multimodal MR imaging in patients with suspected TSEs. Forty-five patients with a suspicion of TSE and 11 age-matched healthy volunteers were included. The MR imaging protocol included T1, FLAIR, and DWI sequences. MRS was performed on the cerebellum, pulvinar, right lenticular nucleus, and frontal cortex. MR images were assessed visually, and ADC values were calculated.

RESULTS: Among the 45 suspected cases, 31 fulfilled the criteria for probable or definite TSEs (19 sCJDs, 3 iCJDs, 2 vCJDs, and 7 genetic TSEs); and 14 were classified as AltDs. High signals in the cortex and/or basal ganglia were observed in 26/31 patients with TSEs on FLAIR and 29/31 patients on DWI. In the basal ganglia, high DWI signals corresponded to a decreased ADC. Metabolic alterations, increased mIns, and decreased NAA were observed in all patients with TSEs. ADC values and metabolic changes were not correlated; this finding suggests that neuronal stress (vacuolization), neuronal loss, and astrogliosis do not alone explain the decrease of ADC.

CONCLUSIONS: MRS combined with other MR imaging is of interest in the diagnosis of TSE and provides useful information for understanding physiopathologic processes underlying prion diseases.

Abbreviations

ADC
apparent diffusion coefficient
AltD
alternative diagnosis
Avg
average
C (or c)
control
Cho
choline
CJD
Creutzfeldt-Jakob disease
Cr
creatine
DWI
diffusion-weighted imaging
EEG
electroencephalograph
FFI
fatal familial insomnia
FLAIR
fluid-attenuated inversion recovery
GABA
gamma-aminobutyric acid
gCJD
genetic CJD
Glx
glutamine-glutamate-GABA
GSS
Gerstmann Strausser Sheinker syndrome
iCJD
iatrogenic CJD
mIns
myo-inositol
MM
methionine homozygosity (PRNP: genotype at codon 129)
MRS
MR spectroscopy
MRI
MR imaging
MV
methionine-valine heterozygosity (PRNP: genotype at codon 129)
NA
data not available
NAA
N-acetylaspartate
NS
not significant or nonspecific slow waves
P (or p)
patient
PRNP
genotype at codon 129
PrP
prion protein or persistent plexus gene
S
sum of metabolites
sCJD
sporadic CJD
TSE
human transmissible spongiform encephalopathy
vCJD
variant CJD
VV
valine homozygosity (PRNP: genotype at codon 129)
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American Journal of Neuroradiology: 31 (7)
American Journal of Neuroradiology
Vol. 31, Issue 7
1 Aug 2010
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Cite this article
D. Galanaud, S. Haik, M.G. Linguraru, J.-P. Ranjeva, B. Faucheux, E. Kaphan, N. Ayache, J. Chiras, P. Cozzone, D. Dormont, J.-P. Brandel
Combined Diffusion Imaging and MR Spectroscopy in the Diagnosis of Human Prion Diseases
American Journal of Neuroradiology Aug 2010, 31 (7) 1311-1318; DOI: 10.3174/ajnr.A2069

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Combined Diffusion Imaging and MR Spectroscopy in the Diagnosis of Human Prion Diseases
D. Galanaud, S. Haik, M.G. Linguraru, J.-P. Ranjeva, B. Faucheux, E. Kaphan, N. Ayache, J. Chiras, P. Cozzone, D. Dormont, J.-P. Brandel
American Journal of Neuroradiology Aug 2010, 31 (7) 1311-1318; DOI: 10.3174/ajnr.A2069
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