Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy
- 25 April 2014
- journal article
- neurodegeneration
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 86 (2), 168-173
- https://doi.org/10.1136/jnnp-2013-307223
Abstract
Background There is less data available regarding the characteristics of cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) in a population-based series. Methodology Patients with ALS incident in Piemonte, Italy, between 2009 and 2011 underwent an extensive neuropsychological battery. Cognitive status was classified as follows: normal cognition, frontotemporal dementia (ALS-FTD), executive cognitive impairment (ALS-ECI), non-executive cognitive impairment (ALS-NECI), behavioural impairment (ALS-Bi), non-classifiable cognitive impairment. We also assessed 127 age-matched and gender-matched controls identified through patients’ general practitioners. Results Out of the 281 incident patients, 207 (71.9%) underwent the neuropsychological testing; of these, 19 were excluded from the analysis due previous conditions affecting cognition. Ninety-one (49.7%) patients were cognitively normal, 23 (12.6%) had ALS-FTD, 36 (19.7%) ALS-ECI, 10 (5.5%) ALS-NECI, 11 (6.0%) ALS-Bi and 11 (6.0%) non-classifiable cognitive impairment, 1 had comorbid Alzheimer's disease. Patients with ALS-FTD were older, had a lower education level, and had a shorter survival than any other cognitive group. Of the nine cases with C9ORF72 mutation, six had ALS-FTD, two ALS-ECI and one was cognitively normal; one of the five patients with SOD1 mutations and one of the five patients with TARBDP mutations had ALS-Bi. Conclusions About 50% of Italian patients with ALS had some degree of cognitive impairment, in keeping with a previous Irish study, despite the largely different genetic background of the two populations. The lower educational attainment in patients with ALS-FTD indicated a possible role of cognitive reserve in ALS-related cognitive impairment. ALS-ECI and ALS-NECI may represent discrete cognitive syndromes in the continuum of ALS and FTD.Keywords
This publication has 28 references indexed in Scilit:
- Delineating the genetic heterogeneity of ALS using targeted high-throughput sequencingJournal of Medical Genetics, 2013
- Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIVNeurology, 2012
- Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort studyThe Lancet Neurology, 2012
- A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTDNeuron, 2011
- Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementiaBrain, 2011
- Executive dysfunction is a negative prognostic indicator in patients with ALS without dementiaNeurology, 2011
- Survival Profiles of Patients With Frontotemporal Dementia and Motor Neuron DiseaseArchives of Neurology, 2009
- Revisiting Brain Reserve Hypothesis in Frontotemporal Dementia: Evidence from a Brain Perfusion StudyDementia and Geriatric Cognitive Disorders, 2009
- Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosisAmyotrophic Lateral Sclerosis, 2009
- Clinical diagnosis of Alzheimer's diseaseNeurology, 1984