title: Extrapyramidal and cognitive signs in amyotrophic lateral sclerosis: A population based cross-sectional study.
abstract: Our objective was to assess the association between amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases such as Alzheimer's disease (AD), frontotemporal dementia (FTD) and Parkinson's disease (PD). From May 2007 through August 2012 we investigated 146 patients with newly diagnosed ALS and 146 age- and gender-matched controls. Each individual was screened for cardinal extrapyramidal signs (neurological examination) and cognitive dysfunction (Mini Mental State Examination, MMSE and Frontal Assessment Battery, FAB). Results demonstrated that rigidity was present in 8.2% of cases and 2.1% of controls (adjusted odds ratio, adjOR 5.7; 95% CI 1.5-22.0). The corresponding percentages for bradykinesia and postural instability were, respectively, 8.2 vs. 2.7% (adjOR 4.8; 95% CI 1.4-16.5) and 2.7 vs. 9.6% (adjOR 0.3; 95% CI 0.1-0.9). FAB ≤ 13.4 was recorded in 24.8 vs. 9.6%; adjOR 2.9; 95% CI 1.5-5.7). Tremor and abnormal FAB score were predicted by an older age at onset while an abnormal FAB score was associated with cramps and family history of neurodegenerative diseases. In conclusion, our data support the notion that newly diagnosed ALS carries a higher than expected risk of extrapyramidal signs and FTD.
authors.affiliation: a Laboratory of Neurological Disorders, IRCCS-Istituto di Ricerche Farmacologiche 'Mario Negri' , Milan , Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Pupillo
authors.affiliation: a Laboratory of Neurological Disorders, IRCCS-Istituto di Ricerche Farmacologiche 'Mario Negri' , Milan , Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Bianchi
authors.affiliation: a Laboratory of Neurological Disorders, IRCCS-Istituto di Ricerche Farmacologiche 'Mario Negri' , Milan , Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Messina
authors.affiliation: b Ospedale Valduce , Como , Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Chiveri
authors.affiliation: c Centro Clinico NEMO, Fondazione Serena Onlus , Milano , Italy.
authors.forename: Christian
authors.initials: C
authors.lastname: Lunetta
authors.affiliation: d Department of Neurorehabilitation Sciences , Casa Cura Policlinico , Milan , Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Corbo
authors.affiliation: e Spedali Civili di Brescia , Brescia , Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Filosto
authors.affiliation: f U.O. di Neurologia, A.O. Mellino Mellini, Chiari , Brescia , Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Lorusso
authors.affiliation: g INSERM UMR1094, Tropical Neuroepidemiology , Limoges , France.
authors.forename: Benoit
authors.initials: B
authors.lastname: Marin
authors.affiliation: h Sant'Agostino-Estense' Hospital, University of Modena and Reggio Emilia , Modena , Italy.
authors.forename: Jessica
authors.initials: J
authors.lastname: Mandrioli
authors.affiliation: i Department of Neurology and Institute of Experimental Neurology (INSPE) , IRCCS San Raffaele Scientific Institute , Milan , Italy.
authors.forename: Nilo
authors.initials: N
authors.lastname: Riva
authors.affiliation: j Azienda Ospedaliera di Melegnano, Vizzolo Predabissi , Milan , Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Sasanelli
authors.affiliation: k Ospedale San Gerardo, Monza, University of Milano-Bicocca , Italy.
authors.forename: Lucio
authors.initials: L
authors.lastname: Tremolizzo
authors.affiliation: a Laboratory of Neurological Disorders, IRCCS-Istituto di Ricerche Farmacologiche 'Mario Negri' , Milan , Italy.
authors.forename: Ettore
authors.initials: E
authors.lastname: Beghi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000453:epidemiology / Q000523:psychology; D001480:Basal Ganglia Diseases / Q000209:etiology*; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000209:etiology*; D018672:Community Health Planning; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D015999:Multivariate Analysis
keywords: Alzheimer's disease; Amyotrophic lateral sclerosis; Parkinson's disease; frontotemporal dementia; heterogeneity; population based