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Metabolic patterns in prion diseases: an FDG PET voxel-based analysis

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European Journal of Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Purpose

Clinical diagnosis of human prion diseases can be challenging since symptoms are common to other disorders associated with rapidly progressive dementia. In this context, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) might be a useful complementary tool. The aim of this study was to determine the metabolic pattern in human prion diseases, particularly sporadic Creutzfeldt-Jakob disease (sCJD), the new variant of Creutzfeldt-Jakob disease (vCJD) and fatal familial insomnia (FFI).

Methods

We retrospectively studied 17 patients with a definitive, probable or possible prion disease who underwent FDG PET in our institution. Of these patients, 12 were diagnosed as sCJD (9 definitive, 2 probable and 1 possible), 1 was diagnosed as definitive vCJD and 4 were diagnosed as definitive FFI. The hypometabolic pattern of each individual and comparisons across the groups of subjects (control subjects, sCJD and FFI) were evaluated using a voxel-based analysis.

Results

The sCJD group exhibited a pattern of hypometabolism that affected both subcortical (bilateral caudate, thalamus) and cortical (frontal cortex) structures, while the FFI group only presented a slight hypometabolism in the thalamus. Individual analysis demonstrated a considerable variability of metabolic patterns among patients, with the thalamus and basal ganglia the most frequently affected areas, combined in some cases with frontal and temporal hypometabolism.

Conclusion

Patients with a prion disease exhibit a characteristic pattern of brain metabolism presentation in FDG PET imaging. Consequently, in patients with rapidly progressive cognitive impairment, the detection of these patterns in the FDG PET study could orient the diagnosis to a prion disease.

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Acknowledgments

The authors would like to acknowledge the clinical and pathological data provided by the following collaborators: María del Carmen Guerrero (Department of Pathology, Hospital Universitario Fundación Alcorcón, Madrid, Spain), Alberto Rábano (Biobank, Fundación CIEN, Madrid, Spain), Juan José Zarranz (Department of Neurology, Hospital Universitario de Cruces, Baracaldo, Spain), Teresa Tuñón (Department of Pathology, Complejo Hospitalario de Navarra, Pamplona, Spain) and the staff of the Department of Neurology at Clínica Universidad de Navarra: Pablo Irimia, Jaime Gállego, Pau Pastor and César Viteri.

Compliance with ethical standards

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Conflicts of interest

None.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Authors and Affiliations

  1. Nuclear Medicine Department, Clínica Universidad de Navarra, 36, Pío XII Avenue, 31008, Pamplona, Spain

    Elena Prieto, Inés Domínguez-Prado, María Jesús Ribelles & Javier Arbizu

  2. Neurology Department, Clínica Universidad de Navarra, 36, Pío XII Avenue, 31008, Pamplona, Spain

    Mario Riverol, Sara Ortega-Cubero, María Rosario Luquin & Purificación de Castro

Authors
  1. Elena Prieto
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  2. Inés Domínguez-Prado
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  3. Mario Riverol
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  4. Sara Ortega-Cubero
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  5. María Jesús Ribelles
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  6. María Rosario Luquin
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  7. Purificación de Castro
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  8. Javier Arbizu
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Corresponding author

Correspondence to Javier Arbizu.

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Prieto, E., Domínguez-Prado, I., Riverol, M. et al. Metabolic patterns in prion diseases: an FDG PET voxel-based analysis. Eur J Nucl Med Mol Imaging 42, 1522–1529 (2015). https://doi.org/10.1007/s00259-015-3090-x

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  • DOI: https://doi.org/10.1007/s00259-015-3090-x

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