NR AXYZ
AU Wroe,S.J.; Hyare,H.; Thornton,J.S.; Yousry,T.; Siddique,D.; Webb,T.; Collinge,J.
TI Regional and Global Apparent Diffusion Coefficient in Inherited Prion Disease: Correlation with Disease Severity
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Epidemiology, Risk Assessment and Transmission P04.50
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB Cerebral diffusion weighted MR imaging (DWI) has recently emerged as the most sensitive sequence for the diagnosis of prion diseases with reports of apparent diffusion coefficient (ADC) changes in specific anatomical regions. This study examined regional and global changes in cerebral ADC in a large cohort of patients with inherited prion disease, and by correlation with clinical indices investigated their potential as biomarkers of disease progression. Twenty five patients (14 female, mean age 45.2 years, range 32-69 years) with inherited prion disease underwent echo-planar DWI (b1000, TE101ms) and conventional T2W and FLAIR imaging at 1.5T. Mean region-of-interest (ROI) ADCs for the head of caudate, putamen and pulvinar nuclei were determined bilaterally and volume-normalised whole-brain ADC histograms computed following tissue segmentation. Clinical assessment included the Mini Mental State Examination (MMSE), Alzheimer's Disease Assessment Scale (ADAS-COG), Clinician's Dementia Rating (CDR) and the Rating of Global Severity (GS). The Spearman rank correlation coefficient was calculated for each of the ROI mean ADCs, the whole-brain mean and median ADCs, and histogram peak height and peak position, versus clinical score, with p<0.01 considered significant. For the whole-brain measures, significant correlations were with MMSE: whole-brain mean ADC vs. MMSE: r=-0.53, p=0.008, whole-brain ADC histogram peak height vs. MMSE: r=0.51, p=0.01 and median whole-brain ADC vs. MMSE: r=-0.52, p=0.009). Of the ROIs investigated, bilateral pulvinar mean ROI ADCs alone correlated significantly with each of the clinical scores, the most significant correlations being between the pulvinar mean ADC and ADAS-COG: right: r=0.77, p<0.001 (Fig 3) and left: r=0.56, p=0.009 (fig 4). No pathological signal changes were detected on conventional MR imaging. Both anatomically specific and whole-brain ADC metrics correlated with disease severity. Conventional MR imaging, including visual assessment of DWI, was relatively insensitive to cerebral pathology in this patient group. Despite this, we have shown that quantification of cerebral ADC provides both regional and global measures that correlate with clinical neurological status and therefore show promise as quantitative pathological biomarkers in inherited prion disease.
AD S. Wroe, D. Siddique, T. Webb, J. Collinge, National Hospital for Neurology and Neurosurgery, UK; H. Hyare, J.S. Thornton, T. Yousry, National Hospital for Neurology and Neurosurgery, UK
SP englisch
PO Schottland