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Introduction : Amyotrophic lateral sclerosis (ALS) has heterogeneous manifestations ranging from motor neuron degeneration to cognitive and behavioral impairment. This study aims to clarify the interactions between cognition and behavioral symptoms with relevant disease predictors and with cognitive reserve (CR), quantified through education, physical activity, and occupation proxies. Methods : A prospective sample of 162 ALS patients and 61 controls were evaluated with the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) (dependent variable), a Cognitive Reserve Index questionnaire (CRIq) and demographic data (age and sex), and, for patients, clinical variables: disease duration, site of onset, the ALS Functional Rating Scale (ALSFRS), forced vital capacity (FVC), and gene mutation chromosome 9 open reading frame 72 ( C9orf72 ) (independent variables). Multiple regression and mediation analyses were performed to predict cognitive and behavioral symptoms. Results : For the ALS group, the statistical model explained 38.8% of variance in ECAS total ( p  < 0.001), 59.4% of executive functions ( p  < 0.001), and 55% of behavioral symptoms ( p  < 0.001). For controls, it accounted for 52.8% of variance in ECAS total ( p  < 0.001). Interaction effects and mediation analysis showed CR is an ECAS total modulator, with a differential effect within groups ( p  < 0.001). Verbal fluency was the single best cognitive score to differentiate patients from controls ( p  = 0.004), and the gene mutation C9orf72 was found to be a behavioral symptom' predictor in patients ( p  = 0.009). Conclusion : This study supports the proposed concept that CR acts as a cognitive modulator in ALS patients and healthy individuals. Moreover, CR also modulates behavioral manifestations in ALS.

Objective: For patients with Parkinson's disease (PD), cognitive impairment is one of the most disabling non-motor symptoms, particularly in the late disease stages (LSPD). Without a common cognitive assessment battery, it is difficult to estimate its prevalence and limits comparisons across studies. In addition, some instruments traditionally used in PD may not be adequate for use in LSPD. We sought to identify instruments used to assess cognition in LSPD and to investigate their global characteristics and psychometric properties to recommend a cognitive battery for the LSPD population., Method: We conducted a literature search of EMBASE and MEDLINE for articles reporting the use of cognitive tests in LSPD. The global characteristics and psychometric properties of the four most used cognitive tests in each cognitive domain were verified to recommend a cognitive assessment battery., Results: Of 60 included studies, 71.7% used screening scales to assess cognition. Of the 53 reported instruments, the Montreal Cognitive Assessment, the Digit Span, the Trail Making Test, the Semantic Fluency test, the Rey Auditory Verbal Learning Test, the Brief Visuospatial Memory Test-Revised, the Boston Naming Test, the Judgment of Line Orientation, and the Clock Drawing Test corresponded best overall to the requirements considered important for selecting instruments in LSPD., Conclusion: Screening scales are frequently used to assess cognition in LSPD. We recommend a cognitive assessment battery that considers the special characteristics of the LSPD population, including being quick and easy to use, with minimized motor demands, and covering all relevant cognitive domains.

Objective: One of the most disabling aspects of living with chronic epilepsy is the unpredictability of seizures. Cumulative research in the past decades has advanced our understanding of the dynamics of seizure risk. Technological advances have recently made it possible to record pertinent biological signals, including electroencephalogram (EEG), continuously. We aimed to assess whether patient-specific seizure forecasting is possible using remote, minimally invasive ultra-long-term subcutaneous EEG., Methods: We analyzed a two-center cohort of ultra-long-term subcutaneous EEG recordings, including six patients with drug-resistant focal epilepsy monitored for 46-230 days with median 18 h/day of recorded data, totaling >11 000 h of EEG. Total electrographic seizures identified by visual review ranged from 12 to 36 per patient. Three candidate subject-specific long short-term memory network deep learning classifiers were trained offline and pseudoprospectively on preictal (1 h before) and interictal (>1 day from seizures) EEG segments. Performance was assessed relative to a random predictor. Periodicity of the final forecasts was also investigated with autocorrelation., Results: Depending on each architecture, significant forecasting performance was achieved in three to five of six patients, with overall mean area under the receiver operating characteristic curve of .65-.74. Significant forecasts showed sensitivity ranging from 64% to 80% and time in warning from 10.9% to 44.4%. Overall, the output of the forecasts closely followed patient-specific circadian patterns of seizure occurrence., Significance: This study demonstrates proof-of-principle for the possibility of subject-specific seizure forecasting using a minimally invasive subcutaneous EEG device capable of ultra-long-term at-home recordings. These results are encouraging for the development of a prospective seizure forecasting trial with minimally invasive EEG., (© 2022 International League Against Epilepsy.)

Objective: Retaining the identity or location of decontextualized objects in visual short-term working memory (VWM) is impaired by healthy and pathological ageing, but research remains inconclusive on whether these two features are equally impacted by it. Moreover, it is unclear whether similar impairments would manifest in naturalistic visual contexts., Method: 30 people with mild cognitive impairment (MCI) and 32 age-matched control participants (CPs) were eye-tracked within a change detection paradigm. They viewed 120 naturalistic scenes, and after a retention interval (1 s) asked whether a critical object in the scene had (or not) changed on either: identity (became a different object), location (same object but changed location), or both (changed in location and identity)., Results: MCIs performed worse than CP but there was no interaction with the type of change. Changes in both were easiest while changes in identity alone were hardest. The latency to first fixation and first-pass duration to the critical object during successful recognition was not different between MCIs and CPs. Objects that changed in both features took longer to be fixated for the first time but required a shorter first pass compared to changes in identity alone which displayed the opposite pattern., Conclusions: Locations of objects are better remembered than their identities; memory for changes is best when involving both features. These mechanisms are spared by pathological ageing as indicated by the similarity between groups besides trivial differences in overall performance. These findings demonstrate that VWM mechanisms in the context of naturalistic scene information are preserved in people with MCI. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Background: Stroke rehabilitation interventions are routinely personalized to address individuals' needs, goals, and challenges based on evidence from aggregated randomized controlled trials (RCT) data and meta-syntheses. Individual participant data (IPD) meta-analyses may better inform the development of precision rehabilitation approaches, quantifying treatment responses while adjusting for confounders and reducing ecological bias., Aim: We explored associations between speech and language therapy (SLT) interventions frequency (days/week), intensity (h/week), and dosage (total SLT-hours) and language outcomes for different age, sex, aphasia severity, and chronicity subgroups by undertaking prespecified subgroup network meta-analyses of the RELEASE database., Methods: MEDLINE, EMBASE, and trial registrations were systematically searched (inception-Sept2015) for RCTs, including ⩾ 10 IPD on stroke-related aphasia. We extracted demographic, stroke, aphasia, SLT, and risk of bias data. Overall-language ability, auditory comprehension, and functional communication outcomes were standardized. A one-stage, random effects, network meta-analysis approach filtered IPD into a single optimal model, examining SLT regimen and language recovery from baseline to first post-intervention follow-up, adjusting for covariates identified a-priori . Data were dichotomized by age (⩽/> 65 years), aphasia severity (mild-moderate/ moderate-severe based on language outcomes' median value), chronicity (⩽/> 3 months), and sex subgroups. We reported estimates of means and 95% confidence intervals. Where relative variance was high (> 50%), results were reported for completeness., Results: 959 IPD (25 RCTs) were analyzed. For working-age participants, greatest language gains from baseline occurred alongside moderate to high-intensity SLT (functional communication 3-to-4 h/week; overall-language and comprehension > 9 h/week); older participants' greatest gains occurred alongside low-intensity SLT (⩽ 2 h/week) except for auditory comprehension (> 9 h/week). For both age-groups, SLT-frequency and dosage associated with best language gains were similar. Participants ⩽ 3 months post-onset demonstrated greatest overall-language gains for SLT at low intensity/moderate dosage (⩽ 2 SLT-h/week; 20-to-50 h); for those > 3 months, post-stroke greatest gains were associated with moderate-intensity/high-dosage SLT (3-4 SLT-h/week; ⩾ 50 hours). For moderate-severe participants, 4 SLT-days/week conferred the greatest language gains across outcomes, with auditory comprehension gains only observed for ⩾ 4 SLT-days/week; mild-moderate participants' greatest functional communication gains were associated with similar frequency (⩾ 4 SLT-days/week) and greatest overall-language gains with higher frequency SLT (⩾ 6 days/weekly). Males' greatest gains were associated with SLT of moderate (functional communication; 3-to-4 h/weekly) or high intensity (overall-language and auditory comprehension; (> 9 h/weekly) compared to females for whom the greatest gains were associated with lower-intensity SLT (< 2 SLT-h/weekly). Consistencies across subgroups were also evident; greatest overall-language gains were associated with 20-to-50 SLT-h in total; auditory comprehension gains were generally observed when SLT > 9 h over ⩾ 4 days/week., Conclusions: We observed a treatment response in most subgroups' overall-language, auditory comprehension, and functional communication language gains. For some, the maximum treatment response varied in association with different SLT-frequency, intensity, and dosage. Where differences were observed, working-aged, chronic, mild-moderate, and male subgroups experienced their greatest language gains alongside high-frequency/intensity SLT. In contrast, older, moderate-severely impaired, and female subgroups within 3 months of aphasia onset made their greatest gains for lower-intensity SLT. The acceptability, clinical, and cost effectiveness of precision aphasia rehabilitation approaches based on age, sex, aphasia severity, and chronicity should be evaluated in future clinical RCTs.

This study aims to investigate if education (as a cognitive reserve proxy) modifies the profile of cognitive performance. We hypothesize that participants with higher education can remain functional (due to a better executive performance), despite a more severe memory impairment, compared with lower education individuals. One hundred and sixty-six mild cognitive impairment (MCI) individuals with at least one comprehensive neuropsychological evaluation were included in a retrospective, cross-sectional study and divided into two groups (Low Education-LE [1-4 years] and Medium-to-High Education-MHE [> 4 years]). A total of 22 neuropsychological measures were analyzed. Age-adjusted results were subject to simple regression analyses to determine the variance explained by education. Average scores and proportions of low performances were subject to group comparison. The results showed similar cognitive decline patterns between individuals with LE and MHE, with no significant difference in each cognitive domain. However, MHE revealed a steeper decline in certain cognitive domains, such as sustained attention and episodic memory, compared with the LE. Moreover, MHE showed a trend to higher proportion of tests affected when compared to LE. These suggest that individuals with higher education may remain in a MCI stage despite a more widespread cognitive impairment, reflecting a higher cognitive reserve.

Introduction: The profile of cognitive impairment associated with the late stages of Parkinson's disease (LSPD) is rarely reported. Its characterization is necessary to better understand the cognitive changes that occur as the disease progresses and to better contribute to its management., Methods: In this cross-sectional study, we characterized the cognitive profile of LSPD patients using the comprehensive assessment methodology proposed by the International Parkinson and Movement Disorders Society Task Force. The association of clinical and demographic variables with dementia diagnosis was also investigated using binary logistic regression analysis., Results: Eighty-four LSPD patients were included (age 75.4 ± 6.9; disease duration 16.9 ± 7.5). Fifty-four (64.3%) were classified as demented and presented a global impairment cognitive profile. In the nondemented group (N = 30), 25 (83.3%) LSPD patients met the diagnostic criteria for mild cognitive impairment, mostly with multiple domain impairment (96.0%) and a heterogeneous profile. Memory was the most frequent and severely impaired cognitive domain in both groups. Disease disability, orientation, complex order comprehension, verbal learning, and visuoconstructive abilities were significantly associated with dementia diagnosis (p < .05)., Conclusions: Cognitive impairment in multiple domains was common in LSPD patients. The most frequent and prominent deficits were in the memory domain, with a strong interference from attention impairment. Disease disability, orientation, complex order comprehension, verbal learning, and visuoconstructive abilities proved to be important determinants for dementia diagnosis., (© 2022 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Background: There is a considerable variation in the reported frequency of dementia in Parkinson's disease (PDD). The aim of this study was to evaluate the frequency of PDD reported in published studies and to examine the different methodological, clinical, and demographic factors that may contribute to this variation., Methods: We conducted a systematic review, searching EMBASE and MEDLINE databases for relevant articles on PDD frequency published before May 2019. A global estimation of PDD was calculated. Different subgroup analyses were performed for methodological, clinical, and demographic characteristics. Meta-regression was also conducted to identify any significant differences within the subgroups., Results: We included 295 studies. The global pooled dementia frequency was 26.3%. These estimations varied according to methodological (14%-55%), clinical (18%-46%), and demographic (21%-43%) variables. The declared primary objective of the studies (to study PDD), the follow-up length (≥7 years), the age of the participants (≥75 years), Parkinson's disease (PD) duration (>10 years), and the Hoehn & Yahr (H&Y) stage (>3) were important factors affecting reported dementia frequency., Conclusions: This systematic review found that approximately one-quarter of the PD patients were diagnosed with PDD. Dementia frequency varied according to methodological, clinical and demographic variables. We cannot examine PDD frequency without considering all these variables that have an impact on it., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Deciding how to manipulate an object to fulfill a goal requires accessing different types of object-related information. How these different types of information are integrated and represented in the brain is still an open question. Here, we focus on examining two types of object-related information-tool-gesture knowledge (i.e., how to manipulate an object), and tool-gesture production (i.e., the actual manipulation of an object). We show a double dissociation between tool-gesture knowledge and tool-gesture production: Patient FP presents problems in pantomiming tool use in the context of a spared ability to perform judgments about an object's manipulation, whereas Patient LS can pantomime tool use, but is impaired at performing manipulation judgments. Moreover, we compared the location of the lesions in FP and LS with those sustained by two classic ideomotor apraxic patients (IMA), using a cortical thickness approach. Patient FP presented lesions in common with our classic IMA that included the left inferior parietal lobule (IPL), and specifically the supramarginal gyrus, the left parietal operculum, the left premotor cortex and the left inferior frontal gyrus, whereas Patient LS and our classic IMA patients presented common lesions in regions of the superior parietal lobule (SPL), motor areas (as primary somatosensory cortex, premotor cortex and primary motor cortex), and frontal areas. Our results show that tool-gesture production and tool-gesture knowledge can be behaviorally and neurally doubly dissociated and put strong constraints on extant theories of action and object recognition and use., Competing Interests: Declaration of competing interest The authors declare no competing interests., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

We aimed to identify the early predictors of cognitive decline, and primary care physicians' (PCP) ability to diagnose cognitively impaired subjects, in a cohort of individuals recruited in primary care centers. Independent adults, aged ≥50 years at inception, with an overall low level of education, undertook a prospective clinical and cognitive evaluation targeting memory, attention and executive functions. At follow-up subjects were classified as cognitively normal (CN) or impaired (CI). Of 275 subjects (70.4 ± 8.3 years old, 176 females, 7.5 ± 4.4 education, 162 with MRI), 31 (11.2%) presented CI 4.9 years later, the majority (64.5%) presenting subjective cognitive complaints. PCP could correctly identify 40% of CI individuals, particularly if they presented current cognitive complaints. Male sex (OR = 3.117; CI95%: 1.007-9.645), age (OR = 1.063; CI95%: 1.004-1.126) and baseline scores on TMT-B (OR = 0.225; CI95%: 0.073-0.688) and Vocabulary (OR =  0.940; 95% CI: 0.894-0.986) predicted CI. This study shows that measures indicating poor cognitive reserve and low executive performance (as shown by low vocabulary and executive test scores, respectively) can be early indicators of the risk of decline, stressing the role of cognitive assessment as part of prevention/early intervention programs. The results also underline the need to help PCP to improve the detection of subjects with cognitive decline.

Objective: To describe two patients with recurring unilateral brief headaches that fulfilled criteria for both primary cough headache (CH) and chronic paroxysmal hemicrania (CPH)., Background: CH is typically a bilateral headache, specifically triggered by cough, straining, or other Valsalva maneuvers. The report of cases sharing features with other primary headache disorders, such as CPH, suggest common pathogenic mechanisms., Methods: Case reports., Results: Two patients (one man), aged 55 and 64, had a two-year history of daily, unilateral, side-locked headache attacks, lasting about 15 minutes, and associated with ipsilateral tearing. Headaches were triggered by cough, sneezing, laughing, or bending forward. Both patients experienced a marked and sustained improvement with indomethacin, and both relapsed when it was interrupted., Conclusions: These cases suggest the existence of transitional phenotypes, or shared pathogenic mechanisms, between CH and CPH, two indomethacin-responsive headaches. A more comprehensive analysis of different CH subtypes is necessary to understand their relation with other primary headaches., (© 2017 American Headache Society.)

Background: Mig-SCog is a 9-item questionnaire developed to quantify attack-related cognitive complaints in migraine (M). The items relate to executive function and language, and the total Mig-SCog score is the sum of those scales., Objective: To evaluate the Mig-SCog scores regarding cognitive symptoms during a variety of conditions., Methods: We conducted a prospective comparative study of the Mig-SCog scores (1) between migraine and tension-type headache (TTH) patients during a headache; (2) in migraine patients between migraine attacks, non-headache pain and pain-free status; (3) in migraine patients during and outside a migraine attack., Results: One hundred forty-nine patients (98 M and 51 TTHA). Total Mig-SCog score was higher in migraine patients than TTH (8.0 ± 4.1 vs 3.4 ± 3.2, P < .0001). Sixty-three patients took part in the next part of the study. Migraine patients rated the Mig-SCog higher for migraine (7.9 ± 4.6) than for non-headache pain (2.3 ± 2.9, P < .0006) or pain-free (1.6 ± 2.4, P < .0006). In the final phase of the study, 38 patients Mig-SCog scores were not significantly different whether obtained during or outside an M attack (P = .26)., Conclusions: Attack-related subjective cognitive symptoms, assessed by Mig-SCog scores, differed between migraine and TTH patients. The Mig-SCog scores from migraine patients were found to be higher during migraine than during non-headache pain or pain-free conditions. Patient scoring from memory for usual attacks was not significantly different to scoring within attacks, We believe this demonstrates negligible recall bias., (© 2016 American Headache Society.)

Migraine and vertigo are two very prevalent conditions in general population. The coexistence of both in the same subject is a significant clinical challenge, since it is not always possible to understand whether they are causally related or associated by chance, requiring different diagnostic and therapeutic approaches. In this review we analyze and summarize the actual knowledge about vestibular migraine (VM), focusing on the new concepts proposed by the International Classification of Headache Disorders 3-beta and by the Bárány Society and also addressing the former concepts, which are still present in clinical practice. We conclude that clinical studies using a multidisciplinary approach are crucial in this field, since different specialists observe the same pathology with different eyes. Clinical presentation of VM is variable in what concerns vestibular symptoms temporal relation with migraine headache, as well as in their accompanying manifestations. Biomarkers, either genomics or functional, and molecular imaging techniques will be helpful to clarify many aspects of the complexity of this entity, helping to define to what extent can VM be considered a separate and independent clinical entity., Competing Interests: The authors declare that there is no conflict of interests regarding the publication of this paper.

Pure alexia (PA) has been associated with visual deficits or a failure to activate the visual word form area (VWFA). We report a patient with pure alexia due to posterior cortical atrophy, in whom event-related potentials revealed a delay in the P100 component and an absent N170 compared with controls. Furthermore, there was a tendency for a larger delay in P100 latencies associated with incorrectly read words. This suggests that some cases of PA might result from deficits in visual perception, signaled by the P100 early potential which could lead to an inability to consistently activate the VWFA, marked by the absent N170.

Introduction: In Portugal, new medical graduates undertake every year the Exame Nacional de Seriação, to be ranked for the available postgraduate residency posts within the National Health Service. Exame Nacional de Seriação is a multiple choice questions test on internal medicine., Objectives: This study analyses the Exame Nacional de Seriação results between 2006 and 2011, to identify the variables that predict its score., Material and Methods: Academic (graduating University, final classification) and biographic data (gender, nationality) of 8956 candidates were analyzed and related to the Exame Nacional de Seriação' score. A linear regression analysis was performed to determine the predictors of that score., Results: There were significant differences in the proportion of candidates coming from each National Medical School attending the Exame Nacional de Seriação, and minor but significant discrepancies in their classification. The percentage of international medical graduates increased to 15% in the last two years. Candidates' gender, nationality (Portuguese or other), year of exam, graduating medical school and final graduating classification had a significant relation with ENS score, explaining 39% of Exame Nacional de Seriação' variance. Final graduating classification was the single most predictive variable, which correlation with Exame Nacional de Seriação' score varied between .679 and .586 across Portuguese Medical Schools, but was weak among candidates graduated abroad., Conclusion: Pregraduate training (Final Graduating Classification and Medical School) is related to Exame Nacional de Seriação' score, particularly among national graduates. This data might be relevant to understand the impact of the changes on Exame Nacional de Seriação about to be implemented.

Evaluation of executive functions is essential in clinical diagnosis, yet there are limited data regarding the performance of participants with low education. We present results on several measures of executive functions obtained in community-dwelling adults with an overall low education and study the effect of this variable in each test. A sample of 479 adults (64% female, mean age 66.4 years) was assessed by a battery comprising 13 measures of executive function (Trail Making Test; Symbol Search; Matrix reasoning; Semantic and phonemic verbal fluencies; Stroop test; and digit spans). Tests' psychometric properties and the effects of age, gender, and education were studied across education levels within each age group. Tests showed good psychometric properties. Education explained more variance than age in the majority of measures, with lower educational levels being significantly associated to worse scores. Tables are presented with mean scores, standard deviation, and the value of extreme percentiles for younger (50-65, N = 232) and older (>65 years, N = 247) × education (0-3, 4, 5-9, and >9 years) subgroups. Education-adjusted norms are necessary for an adequate interpretation of test results. The present data may be useful for clinicians caring for populations with low literacy.

Background: The occurrence of headaches during cerebral angiography or endovascular procedures is an ill-defined issue, as limited information is available to define its frequency, risk factors, pathogenesis and implications for future pain management., Purpose: To determine the frequency of headaches during endovascular procedures (HdEVP) and to define their characteristics and risk factors for their occurrence., Methods: Prospective cross-sectional observational study of adult patients undertaking cerebral angiography or endovascular procedures, recording HdEVP clinical features, previous headache history and procedural variables., Results: One hundred and twenty two procedures were evaluated. HdEVP occurred in 13 patients, both in diagnostic and therapeutic procedures. Pain was described as brief, stabbing or localized pressure of mild to moderate intensity, felt ipsilaterally to the manipulated vessel. Its occurrence was associated with therapeutic interventions (p = 0.007), female gender (p = 0.015) and previous history of more than 4 headache episodes per month (p = 0.018)., Conclusion: HdEVP is an uncommon brief headache that should be further evaluated in the future. Its pathogenesis is probably related to mechanical vessel distension, which activates the trigeminovascular nociceptive system in susceptible individuals.

The objective is to identify the pathogenesis of each autonomic manifestation in cluster headache (CH). Through a deductive statistics method (factor analysis) we analysed the type of autonomic symptoms reported by 157 CH patients. Three principal components were identified in the analysis: parasympathetic activation (lacrimation, conjunctival injection and rhinorrhoea), sympathetic defect (miosis and ptosis) and parasympathetic mediated effect (nasal congestion, eyelid oedema and forehead sweating). This work suggests that there are three different mechanisms underlying autonomic manifestations in CH.

Studies concerning time perception lack a validated assessment tool and a consensual "gold-standard" measure. Moreover, the present evidence suggests modification of timing with aging. This study aimed to develop and validate a neuropsychological tool to measure time perception and to study temporal perception with aging. Eighty-six healthy participants, aged 15-90 years old, were asked to verbally estimate and produce empty intervals signaled by auditory beeps, of 7-, 32-, and 58-s duration. Two tests were used as "gold-standards": estimation of the duration of time necessary to draw a clock ("clock time") and estimation of the duration of neuropsychological evaluation ("global time"). Results showed a correlation between estimation and production (p < .01) and a correlation between estimation or production and "global time" (p < .01). The correlation between either estimation or production and age (p < .01), suggested a faster "internal-clock" in the older participants. However, this finding lost significance when controlled for literacy. The results suggest that these tests are potentially a useful tool to measure subjective perception of time. They also corroborate the hypothesis of a change in subjective time perception with aging. It was not possible to conclude if this effect was a specific result of aging or biased by the interference of literacy.