title: Microstructural white matter correlates of emotion recognition impairment in Amyotrophic Lateral Sclerosis.
abstract: Amyotrophic Lateral Sclerosis (ALS) is associated in about half of the cases with behavioral and cognitive disorders, including impairments in socio-emotional processing, considered as key-features for the diagnosis of the behavioral variant of frontotemporal dementia (bv-FTD). The neurostructural bases of emotional deficits in ALS, however, still remain largely unexplored. Here we aim to assess emotion recognition in non-demented sporadic ALS patients compared with healthy controls, and to explore for the first time its microstructural white-matter correlates. Twenty-two subjects with either probable or definite diagnosis of ALS and 55 age-, gender-, and education-matched healthy controls were recruited in the study. All participants performed the Ekman 60-Faces Test, assessing the recognition of six basic emotions (i.e., anger, disgust, fear, sadness, surprise and happiness). A subgroup of subjects, comprising 19 patients and 20 healthy controls, also underwent a Diffusion Tensor Imaging scanning. Behavioral analysis highlighted a significant decline of emotion recognition skills in patients compared to controls, particularly affecting the identification of negative emotions. Moreover, the Diffusion Tensor Imaging analyses revealed a correlation between this impairment and the alteration of white-matter integrity along the right inferior longitudinal fasciculus and inferior fronto-occipital fasciculus. Our findings indicate the presence of an early emotion recognition deficit in non-demented sporadic ALS patients, associated with microstructural changes in ventral associative bundles connecting occipital, temporo-limbic and orbitofrontal regions in the right hemisphere. These changes may represent a frontotemporal-limbic microstructural marker of socio-emotional impairment in ALS.
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: crespi.chiara@hsr.it.
authors.forename: Chiara
authors.initials: C
authors.lastname: Crespi
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: cerami.chiara@hsr.it.
authors.forename: Chiara
authors.initials: C
authors.lastname: Cerami
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: dodich.alessandra@hsr.it.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Dodich
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: canessa.nicola@hsr.it.
authors.forename: Nicola
authors.initials: N
authors.lastname: Canessa
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy. Electronic address: m.arpone@studenti.unisr.it.
authors.forename: Marta
authors.initials: M
authors.lastname: Arpone
authors.affiliation: San Raffaele Scientific Institute, Milan, Italy. Electronic address: iannaccone.sandro@hsr.it.
authors.forename: Sandro
authors.initials: S
authors.lastname: Iannaccone
authors.affiliation: NEuroMuscolar Omnicentre (NEMO), Niguarda Cà Granda Hospital - Piazza Ospedale Maggiore 3, Milan, Italy. Electronic address: massimo.corbo@centrocliniconemo.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Corbo
authors.affiliation: NEuroMuscolar Omnicentre (NEMO), Niguarda Cà Granda Hospital - Piazza Ospedale Maggiore 3, Milan, Italy. Electronic address: christian.lunetta@centrocliniconemo.it.
authors.forename: Christian
authors.initials: C
authors.lastname: Lunetta
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: scola.elisa@hsr.it.
authors.forename: Elisa
authors.initials: E
authors.lastname: Scola
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: falini.andrea@hsr.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Falini
authors.affiliation: Università Vita Salute San Raffaele, Milan, Italy; San Raffaele Scientific Institute, Milan, Italy. Electronic address: cappa.stefano@hsr.it.
authors.forename: Stefano F
authors.initials: SF
authors.lastname: Cappa
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology* / Q000523:psychology*; D001921:Brain / Q000473:pathology* / Q000648:ultrastructure; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology; D056324:Diffusion Tensor Imaging; D004644:Emotions / Q000502:physiology*; D005149:Facial Expression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011597:Psychomotor Performance / Q000502:physiology; D021641:Recognition, Psychology / Q000502:physiology*; D012938:Social Perception
keywords: ALS-FTD continuum hypothesis; Amyotrophic Lateral Sclerosis; Diffusion Tensor Imaging; Emotion recognition impairment; Inferior Longitudinal Fasciculus and Inferior Fronto-occipital Fasciculus