Parkinsonian traits in amyotrophic lateral sclerosis (ALS): a prospective population-based study

Andrea Calvo, Adriano Chiò, Marco Pagani, Stefania Cammarosano, Francesca Dematteis, Cristina Moglia, Luca Solero, Umberto Manera, Tiziana Martone, Maura Brunetti, Michele Balma, Giancarlo Castellano, Marco Barberis, Angelina Cistaro, Carlo Alberto Artusi, Rosario Vasta, Elisa Montanaro, Alberto Romagnolo, Barbara Iazzolino, Antonio CanosaGiovanna Carrara, Consuelo Valentini, Tie Qiang Li, Flavio Nobili, Leonardo Lopiano, Mario G. Rizzone

Research output: Contribution to journalArticlepeer-review

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 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.

Original languageEnglish
Pages (from-to)1633-1642
Number of pages10
JournalJournal of Neurology
Volume266
Issue number7
DOIs
Publication statusPublished - Jul 1 2019

Keywords

  • Amyotrophic lateral sclerosis
  • Parkinsonian
  • Population-based study
  • Positron emission tomography

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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