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From the Departments of Neurology (Drs. Meissner, Körtner, Bartl, Jastrow, Mollenhauer, Poser, and Zerr) and Genetic Epidemiology (K. Köhler), University of Göttingen; Department of Neurology (Dr. Schröter), University of Würzburg; Department of Neuroradiology (Dr. Finkenstaedt), Magnetic Resonance/Computed Tomography Institute Hamburg; and Institute of Neuropathology (Drs. Windl and Kretzschmar), University of Munich, Germany.
Address correspondence and reprint requests to Dr. Inga Zerr, Department of Neurology, University of Göttingen, Robert-Koch-Str. 40, D-37073 Göttingen, Germany; e-mail: IngaZerr{at}aol.com
Objective: To assess if clinical features, prion protein codon 129, and molecular subtype correlate with MRI basal ganglia hyperintensity in sporadic Creutzfeldt-Jakob disease (CJD).
Methods: The authors studied 219 patients including 153 confirmed CJD cases for their neurologic symptoms and MRI findings. The MRI was assessed by a blinded investigator for the presence of high signal intensity on T2-weighted images in the basal ganglia.
Results: Patients with basal ganglia high signal on T2-weighted images were more likely to present with rapid progressive dementia in an early stage and shorter disease duration (median 6.7 months and 8.6 months). Surprisingly, among the CJD cases, patients without signal increase of the basal ganglia were shown to have a higher frequency of extrapyramidal disturbances (82% vs 70%). More striking differences were found for symptoms such as depression and sensory disturbances, which were more frequent among cases without signal increase. MRI was more likely to be diagnostic in patients with MV2 molecular subtype.
Conclusions: Selected clinical and pathologic features correlate with the presence of basal ganglia high signal on T2-weighted MRI in patients with definite or probable CJD.
Received January 30, 2004. Accepted in final form June 2, 2004.
See also pages 410, 436, and 443
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Neurology 2004 63: 410-411.
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