Selective Patterns of Cognitive Impairment in Spinocerebellar Ataxia Type 6 and Idiopathic Late-Onset Cerebellar Ataxia.

PURPOSE: To determine cognitive impairment patterns in patients with spinocerebellar ataxia type 6 (SCA6) compared to patients with idiopathic late-onset cerebellar ataxia (ILOCA). METHODS: Neurocognitive testing was conducted on 21 SCA6, nine ILOCA, and 27 controls subjects. Intergroup differences were assessed using the Wilcoxon signed-ranked test or Student's t-test. Principal component analysis (PCA) was performed on nine cognitive variables, and Hotelling's T-squared test assessed group-specific differences. Pearson's correlations assessed changes in cognitive performance and disease progression. Intra-group differences among SCA6 were examined in a post-hoc analysis. RESULTS: SCA6 and ILOCA patients showed impairment in visuo-spatial executive function, phonemic verbal fluency, and semantic-verb word generation. ILOCA showed impairment in mental flexibility/response inhibition, verbal learning, semantic-noun verbal fluency, and forward numerical working memory. Within the first three principal components, SCA6 and ILOCA differed from controls and from each other. Verbal working and immediate visuo-spatial memory correlated with disease duration for SCA6. For ILOCA, Mini-Mental Status Exam and RCF copy correlated with disease duration. CONCLUSION: Differing patterns of cognitive dysfunction were seen in SCA6 and ILOCA. PCA suggested that distinct SCA6 subgroups may exist, SCA61 with significant ILOCA overlap in several cognitive deficits, and SCA62 showing deficits in visuo-spatial performance only.

A potential biomarker in sports-related concussion: brain functional connectivity alteration of the default-mode network measured with longitudinal resting-state fMRI over thirty days.

Current diagnosis and monitoring of sports-related concussion rely on clinical signs and symptoms, and balance, vestibular, and neuropsychological examinations. Conventional brain imaging often does not reveal abnormalities. We sought to assess if the longitudinal change of functional and structural connectivity of the default-mode network (DMN) can serve as a potential biomarker. Eight concussed Division I collegiate football student-athletes in season (one participated twice) and 11 control subjects participated in this study. ImPACT (Immediate Post-Concussion Assessment and Cognitive Testing) was administered over the course of recovery. High-resolution three dimensional T1-weighted, T2*-weighted diffusion-tensor images and resting-state functional magnetic resonance imaging (rs-fMRI) scans were collected from each subject within 24 h, 7±1 d and 30±1 d after concussion. Both network based and whole-brain based functional correlation analyses on DMN were performed. ImPACT findings demonstrated significant cognitive impairment across multiple categories and a significant increase of symptom severity on Day 1 following a concussion but full recovery by 6.0±2.4 d. While the structural connectivity within DMN and gross anatomy appeared unchanged, a significantly reduced functional connectivity within DMN from Day 1 to Day 7 was found in the concussed group in this small pilot study. This reduction was seen in eight of our nine concussion cases. Compared with the control group, there appears a general trend of increased DMN functional connectivity on Day 1, a significant drop on Day 7, and partial recovery on Day 30. The results of this pilot study suggest that the functional connectivity of DMN measured with longitudinal rs-fMRI can serve as a potential biomarker to monitor the dynamically changing brain function after sports-related concussion, even in patients who have shown clinical improvement.

Cognitive impairment and psychiatric symptoms in 133 patients with diseases associated with cerebellar degeneration.

The authors performed a chart review to determine the frequency with which neurologists detect cognitive and psychiatric symptoms in patients with cerebellar degeneration. Psychopathology, including depression, personality change, cognitive impairment, anxiety, and psychosis was noted in 51% of 133 patients.