Radiological assessment of Creutzfeldt-Jakob disease

• Published in: European radiology Vol. 17; no. 5; pp. 1200 - 1211
• Main Authors: Tschampa, Henriette J, Zerr, Inga, Urbach, Horst
• Format: Journal Article
• Language: English
• Published: Germany Springer Nature B.V 01-05-2007
• Subjects: Alzheimer's disease; Animals; Ataxia; Basal ganglia; Bovine spongiform encephalopathy; Cattle; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - epidemiology; Dementia; Dementia disorders; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Disease Progression; Dystonia; Electroencephalography; Ganglia; Humans; Hypotheses; Iatrogenesis; Insomnia; Laboratory animals; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Mutation; Neostriatum; Neurodegenerative diseases; Neuropathology; Patients; Proteins; Public concern; Pulvinar; United Kingdom > UK
• ISSN: 0938-7994; 1432-1084
• DOI: 10.1007/s00330-006-0456-2

Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The "pulvinar sign", defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.