Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy.
BACKGROUND
There is less data available regarding the characteristics of cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) in a population-based series.


METHODS
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.
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
Anna
A
Montuschi
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
Barbara
B
Iazzolino
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy The Neuroscience Institute of Torino (NIT) Department of Neurology, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Andrea
A
Calvo
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
Cristina
C
Moglia
Department of Neurology, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Leonardo
L
Lopiano
Laboratory of Molecular Genetics, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Gabriella
G
Restagno
Laboratory of Molecular Genetics, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Maura
M
Brunetti
Laboratory of Molecular Genetics, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Irene
I
Ossola
'Cognetti De Martiis' Department of Economical and Statistical Science, University of Torino, Italy.
Anna
A
Lo Presti
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
Stefania
S
Cammarosano
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
Antonio
A
Canosa
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy The Neuroscience Institute of Torino (NIT) Department of Neurology, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
Adriano
A
Chiò
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000453:epidemiology* / Q000235:genetics / Q000523:psychology*; D000073885:C9orf72 Protein; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000145:classification / Q000150:complications / Q000453:epidemiology* / Q000235:genetics / Q000523:psychology*; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D009154:Mutation; D009483:Neuropsychological Tests; D011506:Proteins / Q000235:genetics; D012307:Risk Factors; D013482:Superoxide Dismutase / Q000235:genetics; D000072105:Superoxide Dismutase-1; D016019:Survival Analysis
D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; D004268:DNA-Binding Proteins; D011506:Proteins; C000606290:SOD1 protein, human; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1
ALS; DEMENTIA; EPIDEMIOLOGY