TY - JOUR
T1 - Parkinsonian traits in amyotrophic lateral sclerosis (ALS)
T2 - a prospective population-based study
AU - Calvo, Andrea
AU - Chiò, Adriano
AU - Pagani, Marco
AU - Cammarosano, Stefania
AU - Dematteis, Francesca
AU - Moglia, Cristina
AU - Solero, Luca
AU - Manera, Umberto
AU - Martone, Tiziana
AU - Brunetti, Maura
AU - Balma, Michele
AU - Castellano, Giancarlo
AU - Barberis, Marco
AU - Cistaro, Angelina
AU - Artusi, Carlo Alberto
AU - Vasta, Rosario
AU - Montanaro, Elisa
AU - Romagnolo, Alberto
AU - Iazzolino, Barbara
AU - Canosa, Antonio
AU - Carrara, Giovanna
AU - Valentini, Consuelo
AU - Li, Tie Qiang
AU - Nobili, Flavio
AU - Lopiano, Leonardo
AU - Rizzone, Mario G.
PY - 2019/7/1
Y1 - 2019/7/1
N2 -
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
18
F-FDG-PET. ALS-PK patients underwent
123
I-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.
123
I-ioflupane SPECT was normal in all but two ALS-PK patients. At
18
F-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.
AB -
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
18
F-FDG-PET. ALS-PK patients underwent
123
I-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.
123
I-ioflupane SPECT was normal in all but two ALS-PK patients. At
18
F-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.
KW - Amyotrophic lateral sclerosis
KW - Parkinsonian
KW - Population-based study
KW - Positron emission tomography
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U2 - 10.1007/s00415-019-09305-0
DO - 10.1007/s00415-019-09305-0
M3 - Article
AN - SCOPUS:85064203142
VL - 266
SP - 1633
EP - 1642
JO - Journal of Neurology
JF - Journal of Neurology
SN - 0340-5354
IS - 7
ER -