Positron emission tomography with [18F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)

• Published in: Journal of neurology 2002-06, Vol.249 (6), p.699-705
• Main Authors: HENKEL, Karsten, ZERR, Inga, SPITZ, Jörg, POSER, Sigrid, HERTEL, Andreas, GRATZ, Klaus-F, SCHRÖTER, Andreas, TSCHAMPA, Henriette J, BIHL, Heiner, BÜLL, Udalrich, GRÜNWALD, Frank, DRZEZGA, Alexander
• Format: Article
• Language: English
• Subjects: Adult; Aged; Alzheimer's disease; Atrophy - etiology; Atrophy - pathology; Atrophy - physiopathology; Biological and medical sciences; Brain - diagnostic imaging; Brain - metabolism; Brain - pathology; Cerebrovascular Circulation - physiology; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnostic imaging; Creutzfeldt-Jakob Syndrome - metabolism; Creutzfeldt-Jakob Syndrome - physiopathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Down-Regulation - physiology; Electroencephalography; Energy Metabolism - physiology; Female; Fluorodeoxyglucose F18; Functional Laterality - physiology; Glucose; Glucose - metabolism; Humans; Magnetic Resonance Imaging; Male; Medical sciences; Metabolism; Middle Aged; Neurology; Nuclear medicine; Radiopharmaceuticals; Tomography; Tomography, Emission-Computed
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-002-0695-3

The aim of this study was to explore the sites of metabolic changes with [(18)F]2-fluoro-2-desoxy-D-glucose (FDG) and positron emission tomography (PET) in patients with Creutzfeldt-Jakob disease and to correlate the findings with clinical symptoms. Static [(18)F]FDG-PET studies of eight patients with the diagnosis of confirmed or probable CJD were retrospectively analysed by two physicians from departments of nuclear medicine independently with a strong interrater agreement (kappa=0,98). The clinical data of the patients, based on a standardized evaluation by physicians from the German Creutzfeldt-Jakob disease surveillance study, was correlated with the PET findings. [(18)F]FDG-PET shows widespread hypometabolism in CJD. All patients had a reduction of cerebral glucose metabolism in at least one temporal or parietal region. Additionally in 7 of our own 8 cases and 3 of 4 cases from the literature the occipital lobe, the cerebellum or the basal ganglia were involved. These findings differ from typical patterns of hypometabolism in Alzheimer's disease and other neurodegenerative disorders. In two thirds of the cases the distribution was markedly asymmetric. Myoclonus was present in five out of our eight own cases. Our data suggest that myoclonus might correlate with metabolic impairment of contralateral parietal and temporal lobes. In three of four patients with visual symptoms FDG uptake was reduced in the visual cortex bilaterally. Typical hyperintensities on MRI were only found in two of the eight cases at the time of PET-studies. Our results demonstrate that [(18)F]FDG-PET appears to be a sensitive investigation in CJD and could be useful to differentiate CJD from other neurodegenerative disorders.