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J Neurol Neurosurg Psychiatry. 2015 Feb;86(2):168-73. doi: 10.1136/jnnp-2013-307223. Epub 2014 Apr 25.

Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy.

Author information

1
'Rita Levi Montalcini' Department of Neuroscience, the ALS Center, University of Torino, Italy.
2
'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.
3
Department of Neurology, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
4
Laboratory of Molecular Genetics, Azienda Ospedaliera Città della Salute e della Scienza, Torino, Italy.
5
'Cognetti De Martiis' Department of Economical and Statistical Science, University of Torino, Italy.

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:

ALS; DEMENTIA; EPIDEMIOLOGY

PMID:
24769471
DOI:
10.1136/jnnp-2013-307223
[Indexed for MEDLINE]

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