Arianna Sala1,2, Leonardo Iaccarino1,2, Piercarlo Fania3, Emilia G Vanoli4, Federico Fallanca4, Caterina Pagnini2, Chiara Cerami2,5, Andrea Calvo6, Antonio Canosa6, Marco Pagani7,8, Adriano Chiò6,7,9, Angelina Cistaro10, Daniela Perani11,12,13. 1. Vita-Salute San Raffaele University, Milan, Italy. 2. In Vivo Human Molecular and Structural Neuroimaging Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy. 3. Positron Emission Tomography Centre IRMET, Affidea, Turin, Italy. 4. Nuclear Medicine Unit, IRCCS San Raffaele Hospital, Via Olgettina, 60, Milan, Italy. 5. Clinical Neuroscience Department, San Raffaele Turro Hospital, Milan, Italy. 6. ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy. 7. Institute of Cognitive Sciences and Technologies, C.N.R, Rome, Italy. 8. Department of Nuclear Medicine, Karolinska Hospital, Stockholm, Sweden. 9. Neuroscience Institute of Turin, Turin, Italy. 10. Department of Neuroscience, Advisor Nuclear Medicine for Amiotrophic Lateral SclerosisRegional Expert Center, University of Turin, Turin, Italy. 11. Vita-Salute San Raffaele University, Milan, Italy. perani.daniela@hsr.it. 12. In Vivo Human Molecular and Structural Neuroimaging Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy. perani.daniela@hsr.it. 13. Nuclear Medicine Unit, IRCCS San Raffaele Hospital, Via Olgettina, 60, Milan, Italy. perani.daniela@hsr.it.
Abstract
PURPOSE: The role for [18F]FDG-PET in supporting amyotrophic lateral sclerosis (ALS) diagnosis is not fully established. In this study, we aim at evaluating [18F]FDG-PET hypo- and hyper-metabolism patterns in spinal- and bulbar-onset ALS cases, at the single-subject level, testing the diagnostic value in discriminating the two conditions, and the correlations with core clinical symptoms severity. METHODS: We included 95 probable-ALS patients with [18F]FDG-PET scan and clinical follow-up. [18F]FDG-PET images were analyzed with an optimized voxel-based-SPM method. The resulting single-subject SPM-t maps were used to: (a) assess brain regional hypo- and hyper-metabolism; (b) evaluate the accuracy of regional hypo- and hyper metabolism in discriminating spinal vs. bulbar-onset ALS; (c) perform correlation analysis with motor symptoms severity, as measured by ALS-FRS-R. RESULTS: Primary motor cortex showed the most frequent hypo-metabolism in both spinal-onset (∼57%) and bulbar-onset (∼64%) ALS; hyper-metabolism was prevalent in the cerebellum in both spinal-onset (∼56.5%) and bulbar-onset (∼55.7%) ALS, and in the occipital cortex in bulbar-onset (∼62.5%) ALS. Regional hypo- and hyper-metabolism yielded a very low accuracy (AUC < 0.63) in discriminating spinal- vs. bulbar-onset ALS, as obtained from single-subject SPM-t-maps. Severity of motor symptoms correlated with hypo-metabolism in sensorimotor cortex in spinal-onset ALS, and with cerebellar hyper-metabolism in bulbar-onset ALS. CONCLUSIONS: The high variability in regional hypo- and hyper-metabolism patterns, likely reflecting the heterogeneous pathology and clinical phenotypes, limits the diagnostic potential of [18F]FDG-PET in discriminating spinal and bulbar onset patients.
PURPOSE: The role for [18F]FDG-PET in supporting amyotrophic lateral sclerosis (ALS) diagnosis is not fully established. In this study, we aim at evaluating [18F]FDG-PET hypo- and hyper-metabolism patterns in spinal- and bulbar-onset ALS cases, at the single-subject level, testing the diagnostic value in discriminating the two conditions, and the correlations with core clinical symptoms severity. METHODS: We included 95 probable-ALSpatients with [18F]FDG-PET scan and clinical follow-up. [18F]FDG-PET images were analyzed with an optimized voxel-based-SPM method. The resulting single-subject SPM-t maps were used to: (a) assess brain regional hypo- and hyper-metabolism; (b) evaluate the accuracy of regional hypo- and hyper metabolism in discriminating spinal vs. bulbar-onset ALS; (c) perform correlation analysis with motor symptoms severity, as measured by ALS-FRS-R. RESULTS: Primary motor cortex showed the most frequent hypo-metabolism in both spinal-onset (∼57%) and bulbar-onset (∼64%) ALS; hyper-metabolism was prevalent in the cerebellum in both spinal-onset (∼56.5%) and bulbar-onset (∼55.7%) ALS, and in the occipital cortex in bulbar-onset (∼62.5%) ALS. Regional hypo- and hyper-metabolism yielded a very low accuracy (AUC < 0.63) in discriminating spinal- vs. bulbar-onset ALS, as obtained from single-subject SPM-t-maps. Severity of motor symptoms correlated with hypo-metabolism in sensorimotor cortex in spinal-onset ALS, and with cerebellar hyper-metabolism in bulbar-onset ALS. CONCLUSIONS: The high variability in regional hypo- and hyper-metabolism patterns, likely reflecting the heterogeneous pathology and clinical phenotypes, limits the diagnostic potential of [18F]FDG-PET in discriminating spinal and bulbar onset patients.
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