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Abstract Details

Brain Metabolic Changes in Amyotrophic Lateral Sclerosis with SOD1 Mutations
Neuromuscular and Clinical Neurophysiology (EMG)
P1 - Poster Session 1 (9:00 AM-5:00 PM)
406
To evaluate brain metabolic changes characterizing ALS with SOD1 mutations (SOD1-ALS) compared to sporadic ALS (sALS), employing 18F-FDG-PET.
Neuropathological data suggest that SOD1-ALS is a distinct form of ALS. 
We included 18 SOD1-ALS patients, 40 healthy controls (HC), and 46 sALS patients without mutations in SOD1, TARDBP, FUS, and C9ORF72, randomly selected from subjects who underwent brain 18F-FDG-PET at diagnosis between 2009 and 2019 at the ALS Centre of Turin. We excluded patients with Frontotemporal Dementia. We used the full factorial design in SPM12 to evaluate whether differences among groups exist overall. In case the hypothesis was confirmed, group comparisons were performed through the two-sample t-test model of SPM12. In all the analyses the height threshold was P<0.001 (P<0.05 FWE-corrected at cluster level). 
The full factorial design resulted in a significant main effect of groups. We identified a relative hypometabolism in sALS patients compared to SOD1-ALS cases in right precentral and medial frontal gyrus, right paracentral lobule, and bilateral postcentral gyrus. SOD1 patients showed a relative hypermetabolism as compared to HC in right precentral gyrus and paracentral lobule. As compared to HC, sALS patients showed relative hypometabolism in frontal, temporal and occipital cortices. 
SOD1-ALS was characterized by a relative hypermetabolism in motor cortex as compared to sALS and HC. Since promising, targeted, therapeutic strategies are upcoming for SOD1-ALS, our data support the use of PET to study disease pathogenesis and to track its course in clinical trials, both in asymptomatic and symptomatic mutation carriers.
Authors/Disclosures
Antonio Canosa
PRESENTER 		Antonio Canosa has nothing to disclose.
Andrea Calvo, MD, PhD, FAAN (Dept. of Neuroscience, University of Turin) Dr. Calvo has nothing to disclose.
Cristina Moglia (University of Torino) Cristina Moglia has nothing to disclose.
Rosario Vasta, MD (University of Turin, Department of Neurosciences) Dr. Vasta has nothing to disclose.
Francesca Palumbo No disclosure on file
No disclosure on file
No disclosure on file
Sara Cabras No disclosure on file
No disclosure on file
No disclosure on file
Federico Casale No disclosure on file
Maura Brunetti No disclosure on file
No disclosure on file
No disclosure on file
Maurizio Grassano, MD (Dept. of Neuroscience, University of Turin) Dr. Grassano has received research support from American Brain Foundation, ALS Association and 好色先生.
Umberto Manera, MD (Department of Neuroscience "Rita Levi Montalcini" - University of Torino) Dr. Manera has nothing to disclose.
No disclosure on file
Adriano Chio, MD, FAAN (Dept. of Neuroscience, University of Turin) Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Corcept.