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Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder
  1. Patricia Lillo1,2,3,
  2. Paulo Caramelli4,5,
  3. Gada Musa6,
  4. Teresa Parrao7,
  5. Ricardo Hughes3,8,
  6. Andres Aragon1,9,
  7. Daniel Valenzuela1,9,
  8. Gabriel Cea10,
  9. Rafael Aranguiz11,
  10. Henrique Cerqueira Guimarães4,
  11. Laura Rousseff4,
  12. Leandro Boson Gambogi4,
  13. Luciano Inácio Mariano4,
  14. Antônio Lúcio Teixeira12,13,
  15. Andrea Slachevsky2,6,10,14,15,16,17,
  16. Leonardo Cruz de Souza4,5
  1. 1 Departamento de Neurologia Sur, Facultad de Medicina, Universidad de Chile, Santiago, Región Metropolitana, Chile
  2. 2 Geroscience Center for Brain Health and Metabolism GERO, Agencia Nacional de Investigación y Desarrollo ANID, Santiago, Región Metropolitana, Chile
  3. 3 Unidad de Neurología, Hospital San José, Servicio de Salud Metropolitano Sur, Santiago, Región Metropolitana, Chile
  4. 4 Programa de Pós-Graduação em Neurociências, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
  5. 5 Behavioral and Cognitive Neurology Research Group, Departmento de Clínica Médica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
  6. 6 Neuropsychology and Clinical Neuroscience Laboratory (LANNEC), Physiopathology Department, ICBM, Neuroscience Department, Faculty of Medicine, Universidad de Chile, Santiago, Chile
  7. 7 Facultad de Psicología, Universidad Alberto Hurtado, Santiago, Chile
  8. 8 Unidad Neuromuscular, Dpto. Neurología y Neurocirugía, Hospital Clínico Universidad de Chile, Santiago, Chile
  9. 9 Servicio de Neurología, Complejo Asistencial Barros Luco, Santiago, Región Metropolitana, Chile
  10. 10 Ciencias Neurologicas Oriente, Universidad de Chile Facultad de Medicina, Santiago, Chile
  11. 11 Instituto Nacional de Geriatria, Santiago, Chile
  12. 12 Santa Casa BH, Ensino e Pesquisa, Belo Horizonte, MG, Brazil
  13. 13 Neuropsychiatry Program, Department of Psychiatry and Behavioral Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, Texas, USA
  14. 14 Memory and Neuropsychiatric Clinic (CMYN) East Neurology Department, Hospital del Salvador & University of Chile, Santiago, Chile
  15. 15 Center for Advanced Research in Education (CIAE), Universidad de Chile, Santiago, Chile
  16. 16 Servicio de Neurología, Departamento de Medicina, Clínica Alemana-Universidad del Desarrollo, Santiago, Chile
  17. 17 Departamento de Neurociencia, Facultad de Medicina, Universidad de Chile, Santiago, Chile
  1. Correspondence to Dr Patricia Lillo, Facultad de Medicina. Departamento de Neurologia Sur, University of Chile School of Medicine, Santiago, Region Metropolitana, Chile; pclz{at}


Objective To compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).

Methods We included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the faux pas test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8).

Results No significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the faux pas (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls.

Discussion Our findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

  • frontotemporal dementia
  • ALS
  • cognition
  • motor neuron disease

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  • PC and AS contributed equally.

  • Collaborators Carla Ferreira; Luiz Sérgio Mageste; Rodrigo Santiago Gomez.

  • Contributors PL, GM, AS and LCdS contributed to conception or design of the work.

    PL, AS, LCdS, GM and TP collected the data. All authors analysed and interpreted data. PL and LCdS drafted the article. All authors critically revised the article and gave final approval of the version to be published.

  • Funding This study was sponsored by ANID/FONDECYT REGULAR/1160940 and ANID/FONDAP/15150012 (PL, AS, RH, AA, DV, GM, TP). It was partially funded by Conselho Nacional de Pesquisa Científica (CNPq), Brazil: Grant 402853/2012. ALT, LCdS and PC are supported by the Brazilian National Council for Scientific and Technological Development (CNPq–Bolsa de Produtividade em Pesquisa).

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval Comité de Etica en Investigación en Seres Humanos, Faculty of Medicine, University of Chile. Nº109-2016. Informed consent was obtained from all ALS patients, their relatives and controls. Ethical approval was granted by: South and East Metropolitan Services of Health in Chile, University of Chile (Faculty of Medicine) and Universidade Federal de Minas Gerais (Brazil).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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