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Background: The "Triana Test" is a novel story recall test based on emotional material with demonstrated accuracy in diagnosing mild cognitive impairment patients., Objective: This study aims to obtain normative data for the "Triana Test"., Methods: A normative study was conducted at a university hospital in Spain. Partners of patients were systematically recruited if eligible (age ≥50, no memory complaints, and a total TMA-93 score at or above the 10th percentile). The "Triana Test" was administered and scored. For developing the normative data, a regression-based method was followed., Results: The final sample included 362 participants (median age = 66, range = 50-88; 64.9% females). A model including age and educational level better predicted the total scores. Combinations of these variables resulted in different 10th percentile scores., Conclusions: Norms for using the "Triana Test" are now available. The provided cutoffs for the 10th percentile will aid in the diagnosis of prodromal Alzheimer's disease., Competing Interests: Silvia Rodrigo-Herrero and Emilio Franco-Macías are the authors of the “Triana Test”., (© 2023 – The authors. Published by IOS Press.)

Background: TMA-93 examines relational binding using images. Biomarker validation has demonstrated that it is discriminative for diagnosing early AD. The effect of cognitive reserve on TMA-93 performance remains unexplored and could improve the interpretative framework for using the test., Objective: To study the effect of cognitive reserve on TMA-93 performance and to provide new norms for the test that include its measurement., Methods: Cognitively unimpaired people aged 55 and over were systematically recruited for this cross-sectional normative study in southern Spain. Age, sex, and scores on the Cognitive Reserve Questionnaire (CRQ; maximum score: 25 points) were collected, and the TMA-93 was administered (maximum score: 30 points). Percentile-based reference data that captured combinations of socio-demographics variables with significant effect on TMA-93 performance were calculated., Results: 902 participants (62.5% female; age: median = 68, IQR = 61-75, range = 55-90) were included. CRQ total scores were globally low (median = 8, IQR = 5-13, range = 0-24). Cognitive reserve, including modifiable items as reading activity and intellectual gaming activity, and age mainly supported the TMA-93 total score variance. Sex seemed to have some influence in the elderly. TMA-93 total scores medians began to drop from 70-75 years old. Higher total score on the CRQ and, possibly, female sex determined a gentler slope. New norms based on these variables showed wide variations in scores for the 5th and 10th percentiles., Conclusion: Visual relational binding ability depends on cognitive reserve, including modifiable items. The age-related binding deficit is buffered by higher cognitive reserve and, at older ages, by female sex.

Characterization of the genetic landscape of Alzheimer's disease (AD) and related dementias (ADD) provides a unique opportunity for a better understanding of the associated pathophysiological processes. We performed a two-stage genome-wide association study totaling 111,326 clinically diagnosed/'proxy' AD cases and 677,663 controls. We found 75 risk loci, of which 42 were new at the time of analysis. Pathway enrichment analyses confirmed the involvement of amyloid/tau pathways and highlighted microglia implication. Gene prioritization in the new loci identified 31 genes that were suggestive of new genetically associated processes, including the tumor necrosis factor alpha pathway through the linear ubiquitin chain assembly complex. We also built a new genetic risk score associated with the risk of future AD/dementia or progression from mild cognitive impairment to AD/dementia. The improvement in prediction led to a 1.6- to 1.9-fold increase in AD risk from the lowest to the highest decile, in addition to effects of age and the APOE ε4 allele., (© 2022. The Author(s).)

Background: TMA-93 examines relational binding using images. The test has been proven to be discriminative for diagnosing early Alzheimer's disease by biomarkers. Norms for this test are available, but the elderly, at high risk for Alzheimer's disease, have not yet been widely represented., Objective: To extend normative data on the TMA-93 for people aged 75 and over., Methods: An extension of the Spanish TMA-93 normative study was undertaken. Only cognitively unimpaired people aged 75 and over were included. Age, gender, and educational attainment were registered as socio-demographic variables. Using histograms analysis, median comparisons, and linear regression analysis, we selected variables that demonstrated influence on TMA-93 total scores and provided percentile-base reference data according to combinations of those variables., Results: We included 431 new participants, resulting in a total sample of 657 individuals (median age = 78, interquartile range = 76-81, range = 75-93). Percentile-base reference data stratified by a combination of age ranges (75-79, n = 428; and ≥80 years, n = 229), and educational attainment (< first grade, n = 253; first grade, n = 209; > first grade, n = 195) revealed that participants achieved a minimum TMA-93 total score of 26/30 at the 50th-percentile regardless of stratum. At the 10th-percentile, a maximum of 24/30 was achieved in the more educated stratum contrasting with a minimum of 19/30 in the less educated stratum., Conclusion: Although mitigated by lower levels of education, performance on the TMA-93 is widely preserved in cognitively unimpaired people aged 75 and over. The test could facilitate the screening of elderly patients with memory complaints.

Objective: To first validate the diagnostic accuracy of the "Triana Test," a new story recall test based on emotional material., Method: A phase I study of validation. We included 55 patients with amnestic Mild Cognitive Impairment and 69 healthy controls, diagnosed according to the "Memory Associative Test of the district of Seine-Saint-Denis" (TMA-93), and matched by age, gender, and educational level. The Triana Test's diagnostic accuracy was calculated by ROC curve analysis and Spearman correlations estimated its convergent validity with a hippocampal memory test, the Free and Cued Selective Reminding Test with Immediate Recall (FCSRT+IR)., Results: The "Triana Test" immediate and delayed recalls showed adequate diagnostic accuracy (AUC ≥ 0,74). The delayed free recall showed the highest diagnostic accuracy (AUC = 0.86). Correlations with the FCSRT+IR were moderate to strong., Conclusions: The "Triana Test" demonstrated accuracy for discriminating amnestic Mild Cognitive Impairment patients from healthy controls and convergent validity with the FCSRT+IR.

Background: Memory tests focused on binding may be more sensitive to diagnose Alzheimer's disease (AD) at an early phase. TMA-93 examines relational binding by images., Objective: Evaluate the reliability (internal consistency and inter-rater and test-retest reliability) and feasibility of the TMA-93 in a clinic setting with low-educated individuals and limited face-to-face time per patient., Methods: The study was undertaken in a neurology outpatient clinic of a hospital in Southern Spain. The internal consistency of the TMA-93 was estimated in 35 patients with amnestic mild cognitive impairment (aMCI) and 40 healthy controls (HCs). The inter-rater reliability (by two raters) and feasibility (by recording the percentage of participants who completed the test, and by timing the administration time) were evaluated in HCs ( n  = 16), aMCI patients ( n  = 18), and mild dementia patients ( n  = 15). The test-retest reliability for the TMA-93 total score was studied in 51 HCs tested by the same examiner 2-4 months apart. The internal consistency was estimated by Cronbach's alpha. The inter-rater and test-retest reliability was quantified by the intraclass correlation coefficient (ICC). The administration time was compared by diagnosis., Results: The internal consistency was "optimal" (Cronbach's alpha = 0.936). The test-retest reliability was "good" [ICC = 0.802 (CI 95%  = 0.653-0.887)]. The inter-rater reliability was "optimal" [ICC = 0.999, (CI 95%  = 0.999-1)]. All participants completed the test. The administration time ranged from less than 3 min in HCs to 6 min in aMCI patients, and 7 min in mild dementia patients., Conclusion: Good feasibility and reliability support using the TMA-93 for examining visual relational binding, particularly in the context of low-educational attainment and limited time per patient., Competing Interests: The authors have no conflict of interest to report., (© 2020 – IOS Press and the authors. All rights reserved.)

Background: TMA-93 examines binding by images, a potential advantage for less-educated individuals., Objective: To obtain norms from older Spanish adults for TMA-93., Methods: A cross-sectional normative study was undertaken in a general neurology outpatient clinic of a university hospital in the Southern Spanish region of Andalusia. Partners of patients who attended the clinic were systematically recruited when eligible: aged 50 and over, no memory complaints, and a total score equal or above percentile 10 on Phototest. Age, gender, and educational attainment were considered as sociodemographic variables. TMA-93 was administered and the total score was registered., Results: The final sample contained 1,131 participants (mean age = 65.7, SD = 9.2), including 305 individuals (27%) who did not completed primary studies. The total score on TMA-93 showed a non-normal, left asymmetric, and leptokurtic distribution (median = 29, interquartile range = 27-30, range = 16-30) mitigated by lower education and older age. Stratified analysis by age and education showed wide variations of the scores for the 5-percentile., Conclusion: TMA-93 runs with a ceiling effect in non-cognitively impaired older Spanish adults. The score for the 5-percentile depends on age and education. The test is feasible for low-educated individuals.

Background: TMA-93 examines binding by images, an advantage for the less educated individuals., Aim: To compare the discriminative validity of TMA-93 against the picture version of Free and Cued Selective Reminding Test (FCSRT) to distinguish patients with amnestic mild cognitive impairment (aMCI) from normal controls (NCs) without excluding less educated individuals., Design: Phase I diagnostic evaluation study., Participants: A total of 30 patients with aMCI and 30 NCs matched for sociodemographics variables., Statistical Analysis: The diagnostic accuracy for each test was calculated by conducting receiver operating characteristic curve analysis. Hanley and McNeil method was used to compare diagnostic accuracy of different tests on the same sample., Results: Up to 41.7% of the sample had less than a first grade of education. Both tests showed excellent diagnostic accuracy. The comparisons did not show significant differences., Conclusions: TMA-93 is so accurate as FCSRT to differentiate aMCI from controls including less educated individuals. The test could be considered as a choice in this sociodemographic context.

Background and Purpose: Carotid angioplasty stent placement (CAS) is an accepted alternative to endarterectomy. However, little is known about its effect on cerebral hemodynamics. We prospectively studied the early and delayed hemodynamic changes after CAS., Materials and Methods: Sixty patients underwent CAS for >or=70% internal carotid artery stenosis. Transcranial Doppler (TCD) was done before, 6 hours, and 30 days after CAS. Cerebrovascular reserve to apnea (CVR) was measured., Results: On the ipsilateral side, basal middle cerebral artery (MCA) middle flow velocity (MFV) and pulsatility index (PI) increased from 49.7 cm/s and 0.85, respectively, to 62.5 cm/s and 1.09 immediately after CAS (P < .05); anterior cerebral artery (ACA) MFV and PI increased from 50.7 cm/s and 0.90, to 58.9 cm/s and 1.08 (P < .05); and posterior cerebral artery (PCA) MFV decreased from 47.5 to 36.5 cm/s, with no change in PI (P < .05). On the contralateral side, nonsignificant changes were seen on MCA, immediately after CAS; ACA and PCA MFV decreased from 63.7 and 45.3 cm/s to 50.3 and 38.6 cm/s, respectively (P < .05); ACA and PCA PI increased from 0.96 and 1.00, respectively, to 1.04 and 1.04 (P < .05). At 30 days, ipsilateral MCA MFV and PI were 52.8 cm/s and 1.12, respectively (P < .05), and contralateral values were 49.6 cm/s (P < .05) and 1.02 (nonsignificant), respectively. Basal ipsilateral and contralateral CVR improved from 26.0% to 37.0% (P < .05), and from 30.1% to 33.5% (nonsignificant), respectively, at 30 days., Conclusions: CAS produces an early significant increase of MFV and PI in the ipsilateral anterior circulation. This effect is maintained, though minor, 30 days later. In addition, CAS results in an improvement of CVR at 30 days.

A Spanish family is reported with dystrophinopathy of myalgia and cramps syndrome type. There were five affected males and three females, and also six asymptomatic carriers. Muscle biopsy showed a dystrophic pattern, but immunohistochemistry carried out with three anti-dystrophin antibodies was normal. Dystrophin analysis by western blot revealed a dystrophin of reduced quantity and molecular weight. DNA analysis showed a deletion of the dystrophin gene involving exons 45-52. The natural history of this disorder and the large intrafamilial clinical variability are discussed.

Background and Objective: Miller-Fisher syndrome (MFS) is considered the most common variant of Guillain-Barré syndrome (GBS) and is characterized by the clinical triad of ophthalmoplegia, ataxia and areflexia. Respiratory involvement and relapses are unusual. Patients with MFS usually have a good recovery and no residual deficits. We describe the clinical features, associated infections and evolution in eight patients with MFS., Patients and Method: Eight cases of MFS and sixty-one of GBS were studied between 1994 and 2003. All cases showed the clinical triad of MFS without major limb weakness or other signs suggestive of CNS involvement., Results: The proportion of MFS with respect to GBS during the same period was 13.1%. Four had a positive serology for Epstein-Barr virus, Salmonella enteritidis, Chlamydia pneumoniae and Mycoplasma pneumoniae. Our cases showed facial palsy (75%), dysphagia (75%), pupillary abnormalities (37.5%) and ventilation support (37.5%). Antiganglioside antibodies, determined in three cases (4 episodes), were positive [GQ1b (50%) and GD1b (50%)]. In all cases, there was a markedly reduced amplitude of the distal sensory as well as frequent axonal degeneration signs. The oldest three patients relapsed and required ventilation support., Conclusions: We report for the first time an association between S. enteritidis and C. pneumoniae and MFS. Older patients in our series suffered a faster disease progression with need of ventilation support. We conclude that an older age correlates with poor prognosis and relapses.