RT Journal Article
SR Electronic
T1 Combined Diffusion Imaging and MR Spectroscopy in the Diagnosis of Human Prion Diseases
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1311
OP 1318
DO 10.3174/ajnr.A2069
VO 31
IS 7
A1 Galanaud, D.
A1 Haik, S.
A1 Linguraru, M.G.
A1 Ranjeva, J.-P.
A1 Faucheux, B.
A1 Kaphan, E.
A1 Ayache, N.
A1 Chiras, J.
A1 Cozzone, P.
A1 Dormont, D.
A1 Brandel, J.-P.
YR 2010
UL http://www.ajnr.org/content/31/7/1311.abstract
AB 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. ADCapparent diffusion coefficientAltDalternative diagnosisAvgaverageC (or c)controlChocholineCJDCreutzfeldt-Jakob diseaseCrcreatineDWIdiffusion-weighted imagingEEGelectroencephalographFFIfatal familial insomniaFLAIRfluid-attenuated inversion recoveryGABAgamma-aminobutyric acidgCJDgenetic CJDGlxglutamine-glutamate-GABAGSSGerstmann Strausser Sheinker syndromeiCJDiatrogenic CJDmInsmyo-inositolMMmethionine homozygosity (PRNP: genotype at codon 129)MRSMR spectroscopyMRIMR imagingMVmethionine-valine heterozygosity (PRNP: genotype at codon 129)NAdata not availableNAAN-acetylaspartateNSnot significant or nonspecific slow wavesP (or p)patientPRNPgenotype at codon 129PrPprion protein or persistent plexus geneSsum of metabolitessCJDsporadic CJDTSEhuman transmissible spongiform encephalopathyvCJDvariant CJDVVvaline homozygosity (PRNP: genotype at codon 129)