| Summary: | Creutzfeldt-Jakob disease (CJD) is a human prion disease found in four forms: sporadic, familial, iatrogenic and variant. Clinical and radiological presenting features of the various forms of prion disease are well documented, but the natural history is less well described. Knowledge of the imaging appearances in human prion diseases is increasing and quantitative magnetic resonance imaging (MRI) techniques have been developec;l to analyse progression of cerebral atrophy. As part of a clinical trial, patients with different types of CJD were assessed regularly using a standardised videoed clinical examination (including cognitive and motor tests) and MRI including a volumetric sequence. Each clinical video was scored by an independent neurologist blinded to the patients' diagnosis, date of examination and arm of trial. The corresponding volumetric MRI was also analysed blindly to measure brain volume, the brain boundary shift integral (a more accurate means of assessing volume change between scans) and a visual representation of the sites of atrophy. . Rates of brain atrophy were correlated with baseline clinical scores and clinical decline in scores. Regional analysis of cerebellar volume was performed and the changes in the different types of human prion disease were compared. Greater understanding of disease course and rates of brain atrophy will aid clinicians in diagnosis and prognosis and provide the basis for surrogate markers of disease progression. Use of the most appropriate markers will optimise measurement of therapeutic outcomes in clinical trials in human prion disease.
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