Local Dynamic Stability of Trunk During Gait is Responsive to Rehabilitation in Subjects with Primary Degenerative Cerebellar Ataxia
This study aimed to assess the responsiveness to the rehabilitation of three trunk acceleration-derived gait indexes, namely the harmonic ratio (HR), the short-term longest Lyapunov's exponent (sLLE), and the step-to-step coefficient of variation (CV), in a sample of subjects with primary degenerative cerebellar ataxia (swCA), and investigate the correlations between their improvements (∆), clinical characteristics, and spatio-temporal and kinematic gait features. The trunk acceleration patterns in the antero-posterior (AP), medio-lateral (ML), and vertical (V) directions during gait of 21 swCA were recorded using a magn...
Source: Cerebellum - January 26, 2024 Category: Neuroscience Authors: Stefano Filippo Castiglia Dante Trabassi Carmela Conte Valeria Gioiosa Gabriele Sebastianelli Chiara Abagnale Alberto Ranavolo Cherubino Di Lorenzo Gianluca Coppola Carlo Casali Mariano Serrao Source Type: research

Factors Influencing Health-Related Quality of Life of Patients with Spinocerebellar Ataxia
This study is to assess HRQoL progression and identify factors affecting SCA patients' HRQoL.METHODS: Longitudinal data (three-year follow-up) of 310 SCA patients of the European SCA3/Machado-Joseph-Disease Initiative (ESMI) (2016-2022) and 525 SCA patients (SCA1, SCA2, SCA3 or SCA6) of the EUROSCA natural history study cohort (2006-2015) were assessed. Both large cohort studies share standardized assessments of clinical measures, SARA, INAS, PHQ-9, and HRQoL (EQ-5D-3L). The association between HRQoL and clinical measures was assessed by Spearman Correlation (rs). Multivariable panel regression models were performed to eva...
Source: Cerebellum - January 26, 2024 Category: Neuroscience Authors: Niklas Weber Maresa Buchholz Anika R ädke Jennifer Faber Tanja Schmitz-H übsch Heike Jacobi Thomas Klockgether Wolfgang Hoffmann Bernhard Michalowsky EUROSCA study group ESMI study group Source Type: research

Cerebellar Neurostimulation for Boosting Social and Affective Functions: Implications for the Rehabilitation of Hereditary Ataxia Patients
Cerebellum. 2024 Jan 25. doi: 10.1007/s12311-023-01652-z. Online ahead of print.ABSTRACTBeyond motor deficits, spinocerebellar ataxia (SCA) patients also suffer cognitive decline and show socio-affective difficulties, negatively impacting on their social functioning. The possibility to modulate cerebello-cerebral networks involved in social cognition through cerebellar neurostimulation has opened up potential therapeutic applications for ameliorating social and affective difficulties. The present review offers an overview of the research on cerebellar neurostimulation for the modulation of socio-affective functions in both...
Source: Cerebellum - January 25, 2024 Category: Neuroscience Authors: Andrea Ciricugno Viola Oldrati Zaira Cattaneo Maria Leggio Cosimo Urgesi Giusy Olivito Source Type: research

Cerebellar-Induced Aphasia After Stroke: Evidence for the "Linguistic Cerebellum"
Cerebellum. 2024 Jan 20. doi: 10.1007/s12311-024-01658-1. Online ahead of print.ABSTRACTThe cerebellum is traditionally known to subserve motor functions. However, for several decades, the concept of the "cerebellar cognitive affective syndrome" has evolved. Studies in healthy participants and patients have confirmed the cerebellar role in language. The exact involvement of the cerebellum regarding cerebellar aphasia remains uncertain. We included 43 cerebellar stroke patients who were tested at 3 months post-onset with the Boston Naming Test (BNT), the Token Test (TT), and the Diagnostic Instrument for Mild Aphasia (DIMA)...
Source: Cerebellum - January 20, 2024 Category: Neuroscience Authors: Djaina Satoer Peter J Koudstaal Evy Visch-Brink Ruben S van der Giessen Source Type: research

A Systematic Review of the Spectrum and Prevalence of Non-motor Symptoms in Multiple System Atrophy
CONCLUSION: In routine clinical practice, NMS in MSA are under-recognised by clinicians. These impact hugely on patient quality of life and contribute to their overall morbidity. A methodical ascertainment of these complaints will address an unmet need, and lead to a more holistic approach of care for individuals with MSA.PMID:38227270 | DOI:10.1007/s12311-023-01642-1 (Source: Cerebellum)
Source: Cerebellum - January 16, 2024 Category: Neuroscience Authors: Chulika Makawita Piriyankan Ananthavarathan Rajith de Silva Naveed Malek Source Type: research

Early-Life Social Determinants of SCA6 Age at Onset, Severity, and Progression
Cerebellum. 2024 Jan 13. doi: 10.1007/s12311-023-01655-w. Online ahead of print.ABSTRACTSCA6 patients with the same size CAG repeat allele can vary significantly in age at onset (AAO) and clinical progression. The specific external factors affecting SCA6 have yet to be investigated. We assessed the effect of early life events on AAO, severity, and progression in SCA6 patients using a social determinant of health approach. We performed a survey of biological and social factors in SCA6 patients enrolled in the SCA6 Network at the University of Chicago. AAO of ataxia symptoms and patient-reported outcome measure (PROM) of ata...
Source: Cerebellum - January 13, 2024 Category: Neuroscience Authors: Tiffany X Chen Hannah L Casey Chi-Ying R Lin Theresa A Boyle Jeremy D Schmahmann Gilbert J L'Italien Sheng-Han Kuo Christopher M Gomez Source Type: research

Patient-Related Outcome Measures for Oculomotor Symptoms in the Cerebellar Ataxias: Insights from Non-Cerebellar Disorders
This study aimed to analyze the literature on patient-related outcome measures (PROMs) assessing QoL impacts of vestibular and cerebellar oculomotor abnormalities in patients with CA to identify the strengths and limitations of existing scales and highlight any areas of unmet need. A systematic review was conducted (Medline, Embase) of English-language original articles reporting on QoL measures in patients with vertigo, dizziness or CA. Pre-specified parameters were retrieved, including diseases studied, scales applied and conclusions drawn. Our search yielded 3671 articles of which 467 studies (n = 111,606 participants) ...
Source: Cerebellum - January 12, 2024 Category: Neuroscience Authors: David J Szmulewicz Rocco Galli Alexander A Tarnutzer Source Type: research

The Phenotypic Spectrum of Spinocerebellar Ataxia Type 19 in a Series of Latin American Patients
This study contributes to a better understanding of the clinical spectrum of SCA19, laying the groundwork for further genotype-phenotype correlations and functional studies to elucidate the underlying pathophysiology.PMID:38180701 | DOI:10.1007/s12311-023-01654-x (Source: Cerebellum)
Source: Cerebellum - January 5, 2024 Category: Neuroscience Authors: Diana Avila-Jaque Fernanda Martin M Leonor Bustamante Mariana Luna Álvarez Jos é Manuel Fernández David Jos é Dávila Ortiz de Montellano Rosa Pardo Diego Varela Marcelo Miranda Source Type: research

The Phenotypic Spectrum of Spinocerebellar Ataxia Type 19 in a Series of Latin American Patients
This study contributes to a better understanding of the clinical spectrum of SCA19, laying the groundwork for further genotype-phenotype correlations and functional studies to elucidate the underlying pathophysiology.PMID:38180701 | DOI:10.1007/s12311-023-01654-x (Source: Cerebellum)
Source: Cerebellum - January 5, 2024 Category: Neuroscience Authors: Diana Avila-Jaque Fernanda Martin M Leonor Bustamante Mariana Luna Álvarez Jos é Manuel Fernández David Jos é Dávila Ortiz de Montellano Rosa Pardo Diego Varela Marcelo Miranda Source Type: research

The Phenotypic Spectrum of Spinocerebellar Ataxia Type 19 in a Series of Latin American Patients
This study contributes to a better understanding of the clinical spectrum of SCA19, laying the groundwork for further genotype-phenotype correlations and functional studies to elucidate the underlying pathophysiology.PMID:38180701 | DOI:10.1007/s12311-023-01654-x (Source: Cerebellum)
Source: Cerebellum - January 5, 2024 Category: Neuroscience Authors: Diana Avila-Jaque Fernanda Martin M Leonor Bustamante Mariana Luna Álvarez Jos é Manuel Fernández David Jos é Dávila Ortiz de Montellano Rosa Pardo Diego Varela Marcelo Miranda Source Type: research

The Phenotypic Spectrum of Spinocerebellar Ataxia Type 19 in a Series of Latin American Patients
This study contributes to a better understanding of the clinical spectrum of SCA19, laying the groundwork for further genotype-phenotype correlations and functional studies to elucidate the underlying pathophysiology.PMID:38180701 | DOI:10.1007/s12311-023-01654-x (Source: Cerebellum)
Source: Cerebellum - January 5, 2024 Category: Neuroscience Authors: Diana Avila-Jaque Fernanda Martin M Leonor Bustamante Mariana Luna Álvarez Jos é Manuel Fernández David Jos é Dávila Ortiz de Montellano Rosa Pardo Diego Varela Marcelo Miranda Source Type: research

The Phenotypic Spectrum of Spinocerebellar Ataxia Type 19 in a Series of Latin American Patients
This study contributes to a better understanding of the clinical spectrum of SCA19, laying the groundwork for further genotype-phenotype correlations and functional studies to elucidate the underlying pathophysiology.PMID:38180701 | DOI:10.1007/s12311-023-01654-x (Source: Cerebellum)
Source: Cerebellum - January 5, 2024 Category: Neuroscience Authors: Diana Avila-Jaque Fernanda Martin M Leonor Bustamante Mariana Luna Álvarez Jos é Manuel Fernández David Jos é Dávila Ortiz de Montellano Rosa Pardo Diego Varela Marcelo Miranda Source Type: research

Cerebellar Asymmetry of Motivational Direction: Anger-Dependent Effects of Cerebellar Transcranial Direct Current Stimulation on Aggression in Healthy Volunteers
Cerebellum. 2024 Jan 4. doi: 10.1007/s12311-023-01644-z. Online ahead of print.ABSTRACTIt has recently been theorized that the frontal asymmetry of approach- and avoidance-related motivation is mirrored in the posterolateral cerebellum. Accordingly, left-to-right dominant cerebellar activity is associated with avoidance-related motivation, whereas right-to-left dominant cerebellar activity is associated with approach-related motivation. The aim of this study was to examine the cerebellar asymmetry of motivational direction in approach-related behavior in the context of aggression. In this randomized double-blind sham-contr...
Source: Cerebellum - January 3, 2024 Category: Neuroscience Authors: Eline S Kruithof Jana Klaus Dennis J L G Schutter Source Type: research

Cerebellar Asymmetry of Motivational Direction: Anger-Dependent Effects of Cerebellar Transcranial Direct Current Stimulation on Aggression in Healthy Volunteers
Cerebellum. 2024 Jan 4. doi: 10.1007/s12311-023-01644-z. Online ahead of print.ABSTRACTIt has recently been theorized that the frontal asymmetry of approach- and avoidance-related motivation is mirrored in the posterolateral cerebellum. Accordingly, left-to-right dominant cerebellar activity is associated with avoidance-related motivation, whereas right-to-left dominant cerebellar activity is associated with approach-related motivation. The aim of this study was to examine the cerebellar asymmetry of motivational direction in approach-related behavior in the context of aggression. In this randomized double-blind sham-contr...
Source: Cerebellum - January 3, 2024 Category: Neuroscience Authors: Eline S Kruithof Jana Klaus Dennis J L G Schutter Source Type: research

Knockdown of the Non-canonical Wnt Gene Prickle2 Leads to Cerebellar Purkinje Cell Abnormalities While Cerebellar-Mediated Behaviors Remain Intact
Cerebellum. 2024 Jan 2. doi: 10.1007/s12311-023-01648-9. Online ahead of print.ABSTRACTAutism spectrum disorders (ASD) involve brain wide abnormalities that contribute to a constellation of symptoms including behavioral inflexibility, cognitive dysfunction, learning impairments, altered social interactions, and perceptive time difficulties. Although a single genetic variation does not cause ASD, genetic variations such as one involving a non-canonical Wnt signaling gene, Prickle2, has been found in individuals with ASD. Previous work looking into phenotypes of Prickle2 knock-out (Prickle2-/-) and heterozygous mice (Prickle...
Source: Cerebellum - January 2, 2024 Category: Neuroscience Authors: Parker W Abbott Jason B Hardie Kyle P Walsh Aaron J Nessler Sean J Farley John H Freeman John A Wemmie Linder Wendt Young-Cho Kim Levi P Sowers Krystal L Parker Source Type: research