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Amyotrophic lateral sclerosis (ALS) primarily affects motor and speech abilities. In addition, cognitive functions are impaired in a subset of patients. There is a need to establish an eye movement-based method of neuropsychological assessment suitable for severely physically impaired patients with ALS. Forty-eight ALS patients and thirty-two healthy controls matched for age, sex and education performed a hand and speech motor-free version of the Raven's coloured progressive matrices (CPM) and the D2-test which had been especially adapted for eye-tracking control. Data were compared to a classical motor-dependent paper-pencil version. The association of parameters of the eye-tracking and the paper-pencil version of the tests and the differences between and within groups were studied. Subjects presented similar results in the eye-tracking and the corresponding paper-pencil versions of the CPM and D2-test: a correlation between performance accuracy for the CPM was observed for ALS patients ( p < 0.001) and controls ( p < 0.001) and in the D2-test for controls ( p = 0.048), whereas this correlation did not reach statistical significance for ALS patients ( p = 0.096). ALS patients performed worse in the CPM than controls in the eye-tracking ( p = 0.053) and the paper-pencil version ( p = 0.042). Most importantly, eye-tracking versions of the CPM ( p < 0.001) and the D2-test ( p = 0.024) reliably distinguished between more and less cognitively impaired patients. Eye-tracking-based neuropsychological testing is a promising approach for assessing cognitive deficits in patients who are unable to speak or write such as patients with severe ALS. [ABSTRACT FROM AUTHOR]

The article discusses three research papers on seizures in patients with dementia. The first study used electroencephalography (EEG) to examine suspected seizure activity in patients with Alzheimer's disease. The second paper investigated the impact of subclinical epileptiform activity and its defects on cognition in patients with dementia. The last research explored the pathological mechanism of cognitive impairment in temporal lobe epilepsy in patients with Alzheimer's disease.

Objective: Smartphone-based cognitive assessment measures allow efficient, rapid, and convenient collection of cognitive datasets. Establishment of feasibility and validity is essential for the widespread use of this approach. We describe a novel smartphone application (HD-Mobile) that includes three performance-based cognitive tasks with four key outcome measures, for use with Huntington's disease (HD) samples. We describe known groups and concurrent validity, test–retest reliability, sensitivity, and feasibility properties of the tasks. Methods: Forty-two HD CAG-expanded participants (20 manifest, 22 premanifest) and 28 healthy controls completed HD-Mobile cognitive tasks three times across an 8-day period, on days 1, 4, and 8. A subsample of participants had pen-and-paper cognitive task data available from their most recent assessment from their participation in a separate observational longitudinal study, Enroll-HD. Results: Manifest-HD participants performed worse than healthy controls for three of four HD-Mobile cognitive measures, and worse than premanifest-HD participants for two of four measures. We found robust test–retest reliability for manifest-HD participants (ICC = 0.71–0.96) and with some exceptions, in premanifest-HD (ICC = 0.52–0.96) and healthy controls (0.54–0.96). Correlations between HD-Mobile and selected Enroll-HD cognitive tasks were mostly medium to strong (r = 0.36–0.68) as were correlations between HD-Mobile cognitive tasks and measures of expected disease progression and motor symptoms for the HD CAG-expanded participants (r = − 0.34 to − 0.54). Conclusions: Results indicated robust known-groups, test–retest, concurrent validity, and sensitivity of HD-Mobile cognitive tasks. The study demonstrates the feasibility and utility of HD-Mobile for conducting convenient, frequent, and potentially ongoing assessment of HD samples without the need for in-person assessment. [ABSTRACT FROM AUTHOR]

Background and purpose: Cognitive and emotional problems occur frequently after stroke. Patients with minor stroke are more likely to be discharged home. This paper compares early cognitive and emotional outcomes in patients discharged home after stroke versus patients discharged to inpatient rehabilitation, and examines the effect of cognitive and emotional outcomes on long-term participation. Methods: In this multicenter prospective cohort study, patients with stroke were assessed at two months with the Hospital Anxiety and Depression Scale (HADS), the Checklist for Cognitive and Emotional Consequences following Stroke (CLCE-24) and the Montreal Cognitive Assessment (MoCA). One year post stroke, participation was assessed with the Restriction subscale of the Utrecht Scale for Evaluation of Rehabilitation—Participation (USER-P Restriction). Results: The study included 332 patients. Two months post stroke, anxiety and cognitive problems were equally prevalent among patients discharged home (n = 243; 73%) and patients discharged to inpatient rehabilitation (n = 89; 27%) (HADS-A = 4.8 ± 3.9 versus 4.6 ± 4.0, p = 0.747; MoCA < 26: 66.7% versus 70.8%, p = 0.477; CLCE-cognition = 3.0 ± 2.9 versus 3.3 ± 2.8, p = 0.499). Depressive symptoms were less severe in patients discharged home (HADS-D = 4.3 ± 3.9 versus 5.5 ± 3.8, p = 0.010). In patients discharged home, cognitive complaints were predictive of long-term participation (B = − 2.03; 95% CI − 3.15, − 0.90), while cognitive or emotional outcomes were not predictive in patients discharged to inpatient rehabilitation. Conclusions: Cognitive and emotional problems at two months post stroke were comparable between patients discharged home and those discharged to inpatient rehabilitation. For patients discharged home, cognitive complaints were predictive of long-term participation. [ABSTRACT FROM AUTHOR]

Our companion paper documents pervasive inhibitory deficits in multiple sclerosis (MS) using ocular motor (OM) measures. Here we investigated the utility of an OM working memory (WMem) task in characterising WMem deficits in these patients as a function of disease status and disease duration. 22 patients with CIS, 22 early clinically definite MS patients (CDMS: <7 years of diagnosis), 22 late CDMS patients (>7 years from diagnosis), and 22 healthy controls participated. All participants completed the ocular motor WMem task, the paced auditory serial addition test (PASAT), and the symbol digit modalities test (SDMT). Clinical disability was characterised in CDMS patients using the Expanded Disability Severity Scale (EDSS). WMem performance was measured as proportion of errors (WMem errors), saccade latency, and relative sensitivity to WMem loading (WMem effect), an indicator of WMem capacity. All patient groups performed more WMem errors than controls with proportion of WMem errors, and degree of WMem effect increasing with increasing disease duration. A larger WMem effect, reflecting poorer WMem capacity, corresponded to poorer performance on neuropsychological measures, and a higher disability score for CDMS patients with the longest disease duration; an observation that suggests wider implication of WMem executive processes with advancing disease. Conspicuously, performance decrements on standard neuropsychological testing did not similarly increase commensurate with disease duration. The ocular motor WMem task appears to meaningfully dissociate WMem deficit from healthy individuals as well as a function of increasing disease duration. Potentially, this task represents a highly informative and objective method by which to ascertain progressive WMem changes from the earliest inception of MS. [ABSTRACT FROM AUTHOR]

Objective: This systematic review aimed to assess the intellectual outcome of children who underwent surgery for epilepsy. Methods: A systematic review of electronic databases was conducted on December 3, 2021, for PubMed and January 11, 2022, for Web of Science. The review was conducted according to the PRISMA guidelines. The included studies reported on intelligence quotient (IQ) or developmental quotient (DQ) before and after epilepsy surgery in children. Studies were included, if the patients had medically intractable epilepsy and if the study reported mainly on curative surgical procedures. We conducted a random-effects meta-analysis to determine the mean change of IQ/DQ. Results: Fifty-seven studies reporting on a total of 2593 patients met the inclusion criteria. The mean age at surgery was 9.2 years (± 3.44; range 2.4 months–19.81 years). Thirty-eight studies showed IQ/DQ improvement on a group level, 8 yielded stable IQ/DQ, and 19 showed deterioration. Pooled analysis revealed a significant mean gain in FSIQ of + 2.52 FSIQ points (95% CI 1.12–3.91). The pooled mean difference in DQ was + 1.47 (95% CI − 6.5 to 9.5). The pooled mean difference in IQ/DQ was 0.73 (95% CI − 4.8 to 6.2). Mean FSIQ gain was significantly higher in patients who reached seizure freedom (+ 5.58 ± 8.27) than in patients who did not (+ 0.23 ± 5.65). It was also significantly higher in patients who stopped ASM after surgery (+ 6.37 ± 3.80) than in patients who did not (+ 2.01 ± 2.41). Controlled studies showed a better outcome in the surgery group compared to the non-surgery group. There was no correlation between FSIQ change and age at surgery, epilepsy duration to surgery, and preoperative FSIQ. Significance: The present review indicates that there is a mean gain in FSIQ and DQ in children with medically intractable epilepsy after surgery. The mean gain of 2.52 FSIQ points reflects more likely sustainability of intellectual function rather than improvement after surgery. Seizure-free and ASM-free patients reach higher FSIQ gains. More research is needed to evaluate individual changes after specific surgery types and their effect on long-term follow-up. [ABSTRACT FROM AUTHOR]

Objective: This study uses the Wechsler intelligence and memory scales to characterize the cognitive function of patients with autoimmune encephalitis (AE) in the chronic stage of the disease. AE is a group of neuroinflammatory disorders, and cognitive impairment is a significant source of chronic morbidity in these patients. Methods: Fifty patients with an average disease duration of 3.2 years after diagnosis were prospectively recruited from four hospitals. They underwent a comprehensive cognitive examination using the Wechsler Abbreviated Scale of Intelligence (WASI-II), Wechsler Adult Intelligence Scale (WAIS-IV) and Wechsler Memory Scale (WMS-IV). Summary statistics were computed, and single-sample and independent-samples t tests were used to compare the cohort to normative data. Results: The results revealed significantly reduced performances in perceptual reasoning, processing speed, and working memory among AE patients. Seropositive AE patients exhibited below-norm processing speed, while the seronegative group showed reduced working memory and processing speed. Delayed memory performance was significantly below expectations only in seronegative patients. Pattern analysis indicated that intact cognition was the most observed outcome after AE, but significant heterogeneity was observed among the impaired patients. Conclusions: The study identified deficits in perceptual reasoning, processing speed, and working memory among chronic AE patients. Pattern analysis highlighted positive long-term cognitive outcomes for many but varied outcomes for those with ongoing difficulties. Although severely cognitively impaired patients were not included, the findings apply to AE cohorts who attend outpatient clinical neuropsychology consultations emphasizing the need for thorough cognitive assessment. The results suggest a need for further research targeting other cognitive domains, including executive functions. [ABSTRACT FROM AUTHOR]

Pre-existing or enhanced cognitive abilities influence symptom onset and severity in neurodegenerative diseases, which improve an individual's ability to deal with neurodegeneration. This process is named cognitive reserve (CR), and it has acquired high visibility in the field of neurodegeneration. However, the investigation of CR has been neglected in the context of cerebellar neurodegenerative disorders. The present study assessed CR and its impact on cognitive abilities in spinocerebellar ataxia type 2 (SCA2), which is a rare cerebellar neurodegenerative disease. We investigated the existence of CR networks in terms of compensatory mechanisms and neural reserve driven by increased cerebello-cerebral functional connectivity. The CR of 12 SCA2 patients was assessed using the Cognitive Reserve Index Questionnaire (CRIq), which was developed for appraising life-span CR. Patients underwent several neuropsychological tests to evaluate cognitive functioning and a functional MRI examination. Network based statistics analysis was used to assess functional brain networks. The results revealed significant correlations of CRIq measures with cognitive domains and patterns of increased connectivity in specific cerebellar and cerebral regions, which likely indicated CR networks. This study showed that CR may influence disease-related cognitive deficits, and it was related to the effective use of specific cerebello-cerebral networks that reflect a CR biomarker. [ABSTRACT FROM AUTHOR]

This study aimed to assess if children with a vestibular impairment (VI) are more prone to have neurocognitive deficits compared to typically developing (TD) peers, taking into account important confounding factors with hearing loss being the most important. The neurocognitive performance of fifteen VI children (6–13 years old) was compared to that of an age-, handedness- and sex-weighted group of TD peers (n = 60). Secondly, their performance was also compared to matched groups of TD and hearing impaired (HI) children to evaluate the involvement of HI. The protocol comprises cognitive tests assessing response inhibition, emotion recognition, visuospatial memory, selective and sustained attention, visual memory and visual-motor integration. Based on the results, the VI group had significantly reduced scores on 'social cognition' (p = 0.018), 'executive functions' (p < 0.01), and 'perceptual-motor functioning' (p = 0.020) compared to their TD and HI peers. For the categories 'complex attention' and 'learning and memory' no differences could be observed. Analogous to the findings of previous literature, the symptoms of a VI are often not limited to the primary functions of the system, but also comprise an impact on emotional and cognitive performance. Therefore, more holistic rehabilitation approaches should be encouraged, with a screening and attention for cognitive, emotional and behavioral dysfunctions in the vestibular population. Since this is one of the first studies to investigate the involvement of a VI in a child's cognitive development, these findings support the need for studies further characterizing the impact of a VI, the underlying pathophysiology and the effect of different rehabilitation procedures. [ABSTRACT FROM AUTHOR]

Background: Cognitive impairment in people with MS (PwMS) has primarily been investigated using conventional imaging markers or fluid biomarkers of neurodegeneration separately. However, the single use of these markers do only partially explain the large heterogeneity found in PwMS. Objective: To investigate the use of multimodal (bio)markers: i.e., serum and cerebrospinal fluid (CSF) levels of neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) and conventional imaging markers in predicting cognitive functioning in PwMS. Methods: Eighty-two PwMS (56 females, disease duration = 14 ± 9 years) underwent neuropsychological and neurological examination, structural magnetic resonance imaging, blood sampling and lumbar puncture. PwMS were classified as cognitively impaired (CI) if scoring ≥ 1.5SD below normative scores on ≥ 20% of test scores. Otherwise, PwMS were defined as cognitively preserved (CP). Association between fluid and imaging (bio)markers were investigated, as well as binary logistics regression to predict cognitive status. Finally, a multimodal marker was calculated using statistically important predictors of cognitive status. Results: Only higher NfL levels (in serum and CSF) correlated with worse processing speed (r = − 0.286, p = 0.012 and r = − 0.364, p = 0.007, respectively). sNfL added unique variance in the prediction of cognitive status on top of grey matter volume (NGMV), p = 0.002). A multimodal marker of NGMV and sNfL yielded most promising results in predicting cognitive status (sensitivity = 85%, specificity = 58%). Conclusion: Fluid and imaging (bio)markers reflect different aspects of neurodegeneration and cannot be used interchangeably as markers for cognitive functioning in PwMS. The use of a multimodal marker, i.e., the combination of grey matter volume and sNfL, seems most promising for detecting cognitive deficits in MS. [ABSTRACT FROM AUTHOR]

Background: Individuals with Friedreich's ataxia (FRDA) display significantly lower performances in many cognitive domains with a pattern of impairment that falls within the cerebellar cognitive affective syndrome (CCAS). Objective: To assess in a large cohort of individuals with FRDA, the main determinant of the CCAS using multiple variable regression models. Methods: This is a monocentric observational study that included 39 individuals with FRDA. Ataxic motor symptoms were evaluated with the SARA and cognitive functions with the CCAS-Scale (CCAS-S). Age, SARA, GAA1, Age of symptoms onset (ASO), Age and disease duration (DD) were chosen as covariates in a linear regression model to predict CCAS-S failed items and covariates in a logistic regression model to predict definite CCAS. Results: Patients mean age, SARA score, ASO, DD and GAA1 were respectively of 29 ± 14, 22 ± 10, 14 ± 11, 15 ± 9 and 712 ± 238 (4 point-mutations). Mean CCAS-S raw score was of 86 ± 16, mean number of failed items was 2.9 ± 1.6. Twenty-three individuals had definite CCAS. The multiple linear regression model with age, SARA, ASO, DD & GAA1 as covariates was statistically significant to predict CCAS-S failed items. The SARA was the only significant coefficient in regression models for predicting CCAS-S failed items number and the definite CCAS occurrence. Conclusions: CCAS is highly prevalent in adult individuals with FRDA. CCAS is predicted by ataxic motor symptoms severity. This finding supports common core cerebellar pathophysiology in both cognitive and motor symptoms in FRDA and warrants screening for CCAS, especially in patients with SARA > 20. [ABSTRACT FROM AUTHOR]

Background: Cognitive impairment is a disabling non-motor symptom of Parkinson's disease (PD). It remains uncertain whether declines in specific cognitive domains relate to imaging or plasma biomarkers across the disease continuum. Objective: We investigated whether neuroimaging and plasma biomarkers correlate with individual task-specific cognitive domain declines evidenced by computerized neuropsychological tests in PD patients. Methods: A total of 107 participants, including 87 PD patients (30 with normal cognition [PD-NC], 30 with mild cognitive impairment [PD-MCI], 27 with dementia [PDD]), and 20 healthy controls, were recruited. All received the Cambridge Neuropsychological Test Automatic Battery (CANTAB) test, brain MRI, and assays of plasma biomarkers, including α-synuclein, tau, Aβ42, and Aβ40. Results: PD patients had generally poorer cognitive performance than controls. Patients with PD-MCI and PDD had worse performance in visual, verbal, and working memory and executive function than those with PD-NC. After adjusting for covariates, PDD patients had global cortical thinning, especially in the temporal and parietal lobes, and higher plasma α-synuclein levels and tau:Aβ42 ratios than PD-NC group. Plasma α-synuclein level was associated with frontal lobe-mediated tasks, while the tau:Aβ42 ratio was associated with posterior cortical-mediated tasks. Facial emotion recognition tasks and visual pattern recognition associated with frontotemporal cortical thinning. The accuracy of predicting PDD using age alone (area under the curve [AUC] 0.756) increased by incorporating plasma biomarkers (AUC = 0.851, p = 0.025). Conclusions: Cognitive decline in PD patients has a task-specific correlation with neuroimaging and plasma biomarkers, which may implicate the underlying neuropathological process of PDD. [ABSTRACT FROM AUTHOR]

Objective: Extensive animal research has shown that vestibular damage can be associated with cognitive deficits. More recently, new evidence has emerged linking vestibular disorders to cognitive impairment in humans. Herein, we review contemporary research on the pathophysiology of cognitive-vestibular interactions and discuss its emerging clinical relevance. Data sources: PubMed, Embase, and Cochrane databases. Review methods: A systematic literature search was performed with combinations of search terms: "cognition," "cognitive impairment," "chronic fatigue," "brain fog," "spatial navigation," "attention," "memory," "executive function," "processing speed," and "vestibular hypofunction." Relevant articles were considered for inclusion, including basic and clinical studies, case series, and major reviews. Conclusions: Patients with vestibular disorders can demonstrate long-term deficits in both spatial and nonspatial cognitive domains. The underlying mechanism(s) linking the vestibular system to cognitive function is not well characterized, but several neuro-biologic correlates have been identified. Additional screening tools are required to identify individuals at risk for cognitive impairment, and further research is needed to determine whether treatment of vestibular dysfunction has the capacity to improve cognitive function. Implications for practice: Physicians should be aware of emerging data supporting the presence of cognitive deficits in patients with vestibular disorders. Prevention and treatment of long-term cognitive deficits may be possible through screening and rehabilitation. [ABSTRACT FROM AUTHOR]

Background and objectives: Thalamic atrophy (TA) represents a biomarker of neurodegeneration and associated dysfunction/decline in physical and cognitive functioning among persons with multiple sclerosis (MS). Aerobic fitness, as an end point of exercise training, represents a promising target for restoring function in MS, but it is unknown if such effects differ by TA. This cross-sectional study examined whether aerobic fitness was differentially associated with cognitive processing speed and walking endurance in persons with MS who present with and without TA. Methods: 44 fully ambulatory persons with MS completed a graded exercise test for measuring aerobic fitness (VO2peak) and underwent 3T MRI for measuring TA, the Symbol Digit Modalities Test (SDMT), and the 6-min walk (6MW). We performed Spearman correlations (rs) among VO2peak, SDMT, and 6MW scores overall, and in persons with and without TA. We applied Fisher's z-test for comparing correlations based on TA status. Results: When controlling for age, EDSS score, and global MRI measures of atrophy, VO2peak was strongly associated with SDMT scores (prs = 0.74, p < 0.01) and 6MW performance (prs = 0.77, p < 0.01) in persons with TA, whereas VO2peak was not associated with SDMT scores (prs = − 0.01, p = 0.99) or 6MW performance (prs = 0.25, p = 0.38) in those without TA. The correlations between VO2peak and SDMT (z = 2.86, p < 0.01) and VO2peak and 6MW (z = 2.33, p = 0.02) were significantly stronger in the TA group. Discussion: This study provides initial evidence of strong, selective associations among aerobic fitness, cognitive processing speed, and walking endurance in persons with TA as a biomarker for MS-related neurodegeneration. Such data support TA as a moderator of the association among aerobic fitness, cognitive processing speed, and walking endurance in persons with MS. Future research should carefully consider the role of TA when designing trials of aerobic exercise, cognition, and mobility in MS. [ABSTRACT FROM AUTHOR]

Objective: To investigate the association of chronic obstructive pulmonary disease (COPD) and Preserved Ratio Impaired Spirometry (PRISm) with cognitive performance and presence of vascular brain lesions (VBL). Methods: We determined both cross-sectional and longitudinal association of lung function impairment with cognition, as well as cross-sectional association of lung function impairment with VBL, in the general population. Between 2009 and 2014 we included 3,941 participants from the Rotterdam Study with spirometry tests, brain MRI scans and cognition tests, of whom 1815 had follow-up data on cognition. Results: Our finding indicated that cross-sectionally, participants with PRISm or COPD GOLD2-4 had a worse global cognitive performance. We did not find differences in cognition over time between those with normal spirometry versus those with lung function impairment. In addition, PRISm and COPD GOLD2-4 were associated with a higher prevalence of lacunar infarcts compared to normal spirometry. Conclusions: This study suggests that persons with COPD GOLD2-4 or restrictive lung function, defined as PRISm, are characterized by poorer global cognitive function and a higher prevalence of lacunar infarcts. [ABSTRACT FROM AUTHOR]