Jordi A Matías-Guiu1, Carmen Guerrero-Márquez2, María Nieves Cabrera-Martín3, Ulises Gómez-Pinedo1,4, María Romeral1, Diego Mayo1, Jesús Porta-Etessam1, Teresa Moreno-Ramos1, José Luis Carreras3, Jorge Matías-Guiu1. 1. a Department of Neurology, Hospital Clínico San Carlos, San Carlos Institute for Health Research (IdISSC) , Universidad Complutense , Madrid , Spain. 2. b Laboratory of Neuropathology, Brain Bank, Department of Pathology , Hospital Universitario Fundación Alcorcón , Madrid , Spain. 3. c Department of Nuclear Medicine, Hospital Clínico San Carlos, San Carlos Institute for Health Research (IdISSC) , Universidad Complutense , Madrid , Spain. 4. d Laboratory of Regenerative Medicine, Hospital Clínico San Carlos, San Carlos Institute for Health Research (IdISSC) , Universidad Complutense , Madrid , Spain.
Abstract
INTRODUCTION: The role of positron emission tomography (PET) in Creutzfeldt-Jakob disease is less defined than in other neurodegenerative diseases. We studied the correlation between the uptake of 18F-florbetaben and 18F-fluorodeoxyglucose with pathological prion protein deposition in histopathology in a case. METHODS: A patient with 80 y old with a rapid neurological deterioration with a confirmed diagnosis of CJD was studied. PET and MRI studies were performed between 13-20 d before the death. A region of interest analysis was performed using Statistical Parametric Mapping. RESULTS: MRI showed atrophy with no other alterations. FDG-PET showed extensive areas of hypometabolism including left frontoparietal lobes as well as bilateral thalamus. Correlation between uptake of 18F-florbetaben and pathological prion protein deposition was r = 0.786 (p < 0.05). Otherwise, correlation between uptake of 18F-FDG and pathological prion protein was r = 0.357 (p = 0.385). Immunohistochemistry with β-amyloid did not show amyloid deposition or neuritic plaques. CONCLUSIONS: Our study supports the use of FDG-PET in the assessment of CJD. FDG-PET may be especially useful in cases of suspected CJD and negative MRI. Furthermore, this case report provides more evidence about the behavioral of amyloid tracers, and the possibility of a low-affinity binding to other non-amyloid proteins, such as the pathological prion protein, is discussed.
INTRODUCTION: The role of positron emission tomography (PET) in Creutzfeldt-Jakob disease is less defined than in other neurodegenerative diseases. We studied the correlation between the uptake of 18F-florbetaben and 18F-fluorodeoxyglucose with pathological prion protein deposition in histopathology in a case. METHODS: A patient with 80 y old with a rapid neurological deterioration with a confirmed diagnosis of CJD was studied. PET and MRI studies were performed between 13-20 d before the death. A region of interest analysis was performed using Statistical Parametric Mapping. RESULTS: MRI showed atrophy with no other alterations. FDG-PET showed extensive areas of hypometabolism including left frontoparietal lobes as well as bilateral thalamus. Correlation between uptake of 18F-florbetaben and pathological prion protein deposition was r = 0.786 (p < 0.05). Otherwise, correlation between uptake of 18F-FDG and pathological prion protein was r = 0.357 (p = 0.385). Immunohistochemistry with β-amyloid did not show amyloid deposition or neuritic plaques. CONCLUSIONS: Our study supports the use of FDG-PET in the assessment of CJD. FDG-PET may be especially useful in cases of suspected CJD and negative MRI. Furthermore, this case report provides more evidence about the behavioral of amyloid tracers, and the possibility of a low-affinity binding to other non-amyloid proteins, such as the pathological prion protein, is discussed.
Entities:
Keywords:
Creutzfeldt-Jakob disease; amyloid; fluorodeoxyglucose; magnetic resonance imaging; positron emission tomography; prion diseases; prion protein
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