Social Cognition in Patients With Cerebellar Neurodegenerative Disorders.

Olivera Tamaš,Milutin Kostić,Nataša Dragašević-Mišković,Aleksandra Kačar,Elka Stefanova,Mirjana Ðorđević,Dejana Stanisavljević,Andona Milovanović,Biljana Salak Ðokić,Nenad Glumbić

doi:10.3389/fnsys.2021.664223

Abstract

ObjectiveCerebellar neurodegenerative disorders (CDs) are a heterogeneous group of disorders. It is known that the cerebellum plays a role not only in motor, but also in cognitive and social cognitive functions. The aim of this study was to investigate social cognition in patients with different CDs.Materials and MethodsSocial cognition was examined in 34 patients, 12 with spinocerebellar ataxia type 1 (SCA1), 6 with spinocerebellar ataxia type 2 (SCA2), and 16 with idiopathic late onset cerebellar ataxia (ILOCA). All patients were clinically evaluated using the Scale for the Rating and Assessment of Ataxia. In addition, 34 age, sex, and education-matched healthy control (HC) subjects were similarly analyzed. Social cognition was studied using two tests: the Faux Pas Recognition Test and the Reading the Mind in the Eyes Test (RMET). An appropriate array of neuropsychological tests was used to assess the global cognitive status as well as the frontal functions and mood.ResultsCD patients achieved significantly worse results on both tests of social cognition compared to the HCs. The SCA1 + 2 group achieved the poorest results on the Faux Pas Recognition Test and exhibited poor performance on all cognitive tests, but was only significantly worse compared to the ILOCA group on the Free and Cued Selective Reminding Test (FCSRT) – recognition. The patients in the SCA1 + 2 and ILOCA groups obtained similar scores on RMET. In the SCA1 + 2 group the findings significantly correlated with clinical parameters of disease severity and duration and executive functions (EFs), and with mood and executive functions in the ILOCA group. In the SCA group EFs appeared as the only significant predictor of RMET achievement. The Boston Naming Test (BTN) was a significant predictor of the CD patients’ achievement on RMET, while the BTN, the Trail Making Test Part A and FCSRT – Delayed free recall predicted their performance on the Faux Pas Recognition Test.ConclusionPatients with CD have social cognitive impairments as demonstrated by the Faux Pas Test and the RMET test results. The SCA1 and 2 patients exhibited a more pronounced impairment compared with the ILOCA patients. The independent cognitive predictors of social cognition impairment were EFs and language.

Highlights

Cerebellar neurodegenerative disorders (CD) encompass a group of diverse disorders affecting the cerebellum and its pathways
Social cognition was studied in 34 patients [mean age/SD: 48.9/11.8; mean education/SD: 12.7/2.1; M/F: 20/14]: 18 with spinocerebellar ataxias (12 SCA1 and 6 SCA2 patients) and 16 idiopathic late-onset cerebellar ataxias (ILOCA) patients
All patients presented with diffuse cerebellar atrophy on MRI, while SCA1 and SCA2 patients further exhibited brainstem atrophy and generalized cortical atrophy, and genetic disorders were confirmed by genetic analyses

Summary

Introduction

Cerebellar neurodegenerative disorders (CD) encompass a group of diverse disorders affecting the cerebellum and its pathways. SCA1 and SCA2, the two types of cerebellar ataxias which are caused by trinucleotide expansion, represent the most common types in the Serbian population (Dragasevicet al., 2006) Another group of ataxias is idiopathic late-onset cerebellar ataxias (ILOCA) or sporadic adult-onset ataxias, whose etiology still remains unknown. These disorders are non-hereditary and degenerative ataxias characterized by a slowly progressive cerebellar syndrome (Barbosa et al, 2016; Klockgether, 2018). It is a well-known and widely reported fact that the cerebellum plays a vital role in motor functions (Glickstein, 1992; Manto et al, 2012). Patients with degenerative ataxias exhibit changes in emotions (Adamaszek et al, 2017) and social cognition (SC) (Sokolovsky et al, 2010)

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