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Research paper
Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy
  1. Anna Montuschi1,
  2. Barbara Iazzolino1,
  3. Andrea Calvo1,2,3,
  4. Cristina Moglia1,
  5. Leonardo Lopiano3,
  6. Gabriella Restagno4,
  7. Maura Brunetti4,
  8. Irene Ossola4,
  9. Anna Lo Presti5,
  10. Stefania Cammarosano1,
  11. Antonio Canosa1,
  12. Adriano Chiò1,2,3
  1. 1‘Rita Levi Montalcini’ Department of Neuroscience, the ALS Center, University of Torino, Italy
  2. 2The Neuroscience Institute of Torino (NIT)
  3. 3Department of Neurology, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy
  4. 4Laboratory of Molecular Genetics, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy
  5. 5‘Cognetti De Martiis’ Department of Economical and Statistical Science, University of Torino, Italy
  1. Correspondence to Professor Adriano Chiò, ALS Center, ‘Rita Levi Montalcini’ Department of Neuroscience, University of Torino, via Cherasco 15, 10126 Torino, Italy; achio{at}


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.

  • ALS

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