Andrea Calvo1, Adriano Chiò2, Marco Pagani3,4, Stefania Cammarosano2, Francesca Dematteis5, Cristina Moglia2, Luca Solero2, Umberto Manera2, Tiziana Martone6, Maura Brunetti2, Michele Balma7, Giancarlo Castellano7, Marco Barberis2, Angelina Cistaro8, Carlo Alberto Artusi6, Rosario Vasta2, Elisa Montanaro6, Alberto Romagnolo6, Barbara Iazzolino2, Antonio Canosa2, Giovanna Carrara9, Consuelo Valentini9, Tie-Qiang Li10,11, Flavio Nobili12, Leonardo Lopiano6, Mario G Rizzone6. 1. Department of Neuroscience "Rita Levi Montalcini", ALS Center, University of Turin, Via Cherasco 15, 1026, Turin, Italy. andrea.calvo@unito.it. 2. Department of Neuroscience "Rita Levi Montalcini", ALS Center, University of Turin, Via Cherasco 15, 1026, Turin, Italy. 3. Institute of Cognitive Sciences and Technologies, CNR, Rome, Italy. 4. Department of Nuclear Medicine, Karolinska Hospital Stockholm, Solna, Sweden. 5. Neurology Unit, Ospedali Riuniti di Rivoli, Rivoli, Italy. 6. Department of Neuroscience "Rita Levi Montalcini", Movement Disorders Center, University of Turin, Turin, Italy. 7. Nuclear Medicine Clinic, University of Turin, Turin, Italy. 8. Positron Emission Tomography Centre IRMET S.p.A., Affidea, Turin, Italy. 9. Neuroradiology Unit, AOU Città della Salute e della Scienza di Torino, Turin, Italy. 10. Division of Function and Technology, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 141 86, Stockholm, Sweden. 11. Division of MR Physics, Department of Medical Radiation and Nuclear Medicine, Karolinska University Hospital, 141 86, Stockholm, Sweden. 12. Clinical Neurology, Department of Neuroscience (DINOGMI), University of Genoa and Polyclinic San Martino Hospital, Genoa, Italy.
Abstract
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is characterized by a spectrum of phenotypes, but only a few studies have addressed the presence of parkinsonian (PK) symptoms. The aim of our study was to investigate the occurrence of PK features in a prospective population-based cohort of ALS patients, determining their demographic, clinical, neuropsychological and genetic characteristics, and identifying their morphological and functional imaging correlates. METHODS: A consecutive series of ALS patients were enrolled and prospectively followed for 2 years. Patients were classified according to the presence (ALS-PK) or absence (ALS) of PK signs, and they underwent neuropsychological testing, genetic analysis for the main ALS and PD genes, brain MRI and 18F-FDG-PET. ALS-PK patients underwent 123I-ioflupane SPECT. RESULTS: Out of 114 eligible patients, 101 (64 men; mean age at onset 65.1 years) were recruited. Thirty-one patients (30.7%) were classified as ALS-PK. Compared to ALS patients, ALS-PK patients were more frequently male, but did not differ for any other clinical, demographic or neuropsychological factors. 123I-ioflupane SPECT was normal in all but two ALS-PK patients. At 18F-FDG-PET, ALS-PK patients showed a relative hypometabolism in left cerebellum and a relatively more preserved metabolism in right insula and frontal regions; MRI fractional anisotropy was reduced in the sagittal stratum and increased in the retrolenticular part of the internal capsule. CONCLUSIONS: In our study, about 30% of ALS patients showed PK signs. Neuroimaging data indicate that PK signs are due to the involvement of brain circuitries other than classical nigrostriatal ones, strengthening the hypothesis of ALS as a complex multisystem disease.
BACKGROUND:Amyotrophic lateral sclerosis (ALS) is characterized by a spectrum of phenotypes, but only a few studies have addressed the presence of parkinsonian (PK) symptoms. The aim of our study was to investigate the occurrence of PK features in a prospective population-based cohort of ALS patients, determining their demographic, clinical, neuropsychological and genetic characteristics, and identifying their morphological and functional imaging correlates. METHODS: A consecutive series of ALS patients were enrolled and prospectively followed for 2 years. Patients were classified according to the presence (ALS-PK) or absence (ALS) of PK signs, and they underwent neuropsychological testing, genetic analysis for the main ALS and PD genes, brain MRI and 18F-FDG-PET. ALS-PK patients underwent 123I-ioflupane SPECT. RESULTS: Out of 114 eligible patients, 101 (64 men; mean age at onset 65.1 years) were recruited. Thirty-one patients (30.7%) were classified as ALS-PK. Compared to ALS patients, ALS-PK patients were more frequently male, but did not differ for any other clinical, demographic or neuropsychological factors. 123I-ioflupane SPECT was normal in all but two ALS-PK patients. At 18F-FDG-PET, ALS-PK patients showed a relative hypometabolism in left cerebellum and a relatively more preserved metabolism in right insula and frontal regions; MRI fractional anisotropy was reduced in the sagittal stratum and increased in the retrolenticular part of the internal capsule. CONCLUSIONS: In our study, about 30% of ALS patients showed PK signs. Neuroimaging data indicate that PK signs are due to the involvement of brain circuitries other than classical nigrostriatal ones, strengthening the hypothesis of ALS as a complex multisystem disease.
Authors: Colin J Mahoney; Rebekah M Ahmed; William Huynh; Sicong Tu; Jonathan D Rohrer; Richard S Bedlack; Orla Hardiman; Matthew C Kiernan Journal: CNS Drugs Date: 2021-05-15 Impact factor: 5.749
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