Arch Neurol 2012 Dec;69(12):1578-82
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Parkinsonism Relat Disord 2013 Jan 1;19(1):81-5. Epub 2012 Sep 1.
Department of Neuroscience, The Alfred Hospital, Commercial Road, Melbourne, Victoria 3004, Australia.
Background: Corticobasal syndrome (CBS), which encompasses cortical sensory loss, alien limb, bradykinesia, rigidity, limb apraxia and dystonia, is the classic presentation of corticobasal degeneration (CBD). It may occur in other neurodegenerative disorders including sporadic Creutzfeldt-Jakob disease (sCJD). Current CBD diagnostic criteria outline features of CBS but fail to distinguish CBD from other causative pathologies. Read More
JAMA Neurol 2016 Jan;73(1):76-84
Medical Research Council Prion Unit, Department of Neurodegenerative Diseases, University College London Institute of Neurology, London, England.
Importance: Prion diseases represent the archetype of brain diseases caused by protein misfolding, with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia. Diffusion-weighted imaging (DWI) has emerged as the most sensitive magnetic resonance imaging (MRI) sequence for the diagnosis of sCJD, but few studies have assessed the evolution of MRI signal as the disease progresses.
Objectives: To assess the natural history of the MRI signal abnormalities on DWI in sCJD to improve our understanding of the pathogenesis and to investigate the potential of DWI as a biomarker of disease progression, with histopathological correlation. Read More
J Alzheimers Dis 2013 ;34(1):231-8
Department of Neurosciences, SNPSRR, University of Padova, Via Giustiniani 5, Padua, Italy.
Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Read More
J Neurol 2014 May 26;261(5):877-83. Epub 2014 Feb 26.
Department of Neurology, National Reference Center for TSE, Clinical Dementia Center, University Medical School Göttingen, Göttingen, Germany,
In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fluid (CSF), electroencephalography (EEG), and magnetic resonance imaging (MRI) features of this group. Read More