Validez discriminante y concordancia interobservador de 2 métodos de puntuación del test del reloj / Discriminant validity and inter-rater concordance of two scoring systems for the clock test

Objetivos Comparar la validez discriminante y la fiabilidad interobservador de los 2 métodos de corrección del test del reloj más usados en España. Metodología Se han evaluado 2 colecciones de dibujos del reloj obtenidos en un contexto clínico (116 casos; 56,8% mujeres, edad media 73,1±7,7 años) y en una cohorte de voluntarios (2.039 dibujos de 579 sujetos; 59,5% mujeres, edad media 78,3±3,8 años). Todos los sujetos fueron clasificados como sin deterioro cognitivo (DC−) o con deterioro cognitivo (DC+) tras una extensa evaluación clínica y neuropsicológica. Evaluadores expertos han valorado estos dibujos de forma independiente y sin conocimiento del diagnóstico con los métodos de Sunderland y Solomon estandarizados en español por Cacho (rango: 0 a 10) y del Ser (rango: 0 a 7), respectivamente. Se ha calculado la validez discriminante de cada método mediante el área bajo la curva ROC (aROC) en las 2 muestras, y la fiabilidad interobservador mediante el coeficiente de correlación intraclase (CCI) y el coeficiente kappa en la muestra clínica que fue valorada por los 2 evaluadores. Resultados No hay diferencias significativas en la validez discriminante de los métodos de Sunderland y Solomon en ninguna de las muestras (clínica: aROC: 0,73 [IC 95%: 0,64-0,81] y 0,77 [IC 95%: 0,69-0,85], respectivamente, p=0,19; voluntarios: aROC: 0,69 [IC 95%: 0,67-0,71] y 0,72 [IC 95%: 0,69-0,73], respectivamente, p=0,08). Los puntos de corte ≤8 y ≤5 clasifican correctamente al 71 y 73% de la muestra clínica y al 82 y 84% de la muestra de voluntarios, respectivamente. Los 2 métodos tienen una buena concordancia en la muestra clínica (AU)

Objective To compare the discriminant validity and inter-rater reliability of the two scoring systems for the Clock test that are most used in Spain. Methodology Two collections of clock drawings obtained in a clinical context (116 cases; 56.8% women, mean age 73.1±7.7 years) and in a cohort of volunteers (2039 drawings of 579 subjects; 59.5% women, mean age 78.3±3.8 years) have been assessed. All subjects were classified as cognitively normal (CN) or cognitively impaired (CI) after extensive clinical and neuropsychological evaluation. Expert raters have evaluated these drawings independently and without knowledge of the diagnosis using the Sunderland and Solomon systems standardized in Spanish by Cacho (range 0 to 10) and del Ser (range 0 to 7) respectively. The discriminant validity of each method was calculated in the two samples using the area under the ROC curve (aROC), and the inter-rater reliability was calculated in the clinical sample, that was assessed by the two evaluators, using the intraclass correlation coefficient (ICC) and the kappa coefficient. Results There are no significant differences in the discriminant validity of the Sunderland and Solomon systems in any of the samples (clinical: aROC 0.73 [CI95%: 0.64-0.81] and 0.77 [CI95%: 0.69-0.85] respectively, P=.19; volunteers: aROC 0.69 [CI95%: 0.67-0.71] and 0.72 [CI95%: 0.69-0.73] respectively, P=.08). The cut-off points ≤8 and ≤5 correctly classify 71% and 73% of the clinical sample and 82% and 84% of the volunteer sample, respectively. Both systems have good agreement in the clinical sample (Sunderland: ICC 0.90 [CI95%: 0.81-0.93], kappa 0.76 [CI95%: 0.70-0.83]; Solomon: 0.92 [CI95%: 0.88-0.95] and 0.77 [CI95%: 0.71-0.83] respectively), somewhat higher in the second, although the differences are not significant (AU)

[Discriminant validity and inter-rater concordance of two scoring systems for the clock test]. / Validez discriminante y concordancia interobservador de 2 métodos de puntuación del test del reloj.

OBJECTIVE: To compare the discriminant validity and inter-rater reliability of the two scoring systems for the Clock test that are most used in Spain. METHODOLOGY: Two collections of clock drawings obtained in a clinical context (116 cases; 56.8% women, mean age 73.1±7.7 years) and in a cohort of volunteers (2039 drawings of 579 subjects; 59.5% women, mean age 78.3±3.8 years) have been assessed. All subjects were classified as cognitively normal (CN) or cognitively impaired (CI) after extensive clinical and neuropsychological evaluation. Expert raters have evaluated these drawings independently and without knowledge of the diagnosis using the Sunderland and Solomon systems standardized in Spanish by Cacho (range 0 to 10) and del Ser (range 0 to 7) respectively. The discriminant validity of each method was calculated in the two samples using the area under the ROC curve (aROC), and the inter-rater reliability was calculated in the clinical sample, that was assessed by the two evaluators, using the intraclass correlation coefficient (ICC) and the kappa coefficient. RESULTS: There are no significant differences in the discriminant validity of the Sunderland and Solomon systems in any of the samples (clinical: aROC 0.73 [CI95%: 0.64-0.81] and 0.77 [CI95%: 0.69-0.85] respectively, P=.19; volunteers: aROC 0.69 [CI95%: 0.67-0.71] and 0.72 [CI95%: 0.69-0.73] respectively, P=.08). The cut-off points ≤8 and ≤5 correctly classify 71% and 73% of the clinical sample and 82% and 84% of the volunteer sample, respectively. Both systems have good agreement in the clinical sample (Sunderland: ICC 0.90 [CI95%: 0.81-0.93], kappa 0.76 [CI95%: 0.70-0.83]; Solomon: 0.92 [CI95%: 0.88-0.95] and 0.77 [CI95%: 0.71-0.83] respectively), somewhat higher in the second, although the differences are not significant. CONCLUSIONS: The discriminant validity and inter-observer reliability of these two Clock Test correction systems are similar. Solomon's method, shorter and simpler, may be more advisable in pragmatic terms.

Prevalence of treated patients with Alzheimer's disease: current trends and COVID-19 impact.

BACKGROUND: There are few updated studies on the prevalence and management of Alzheimer's disease (AD), which could be underdiagnosed or undertreated. The COVID-19 pandemic may have worsened the deficiencies in the diagnosis and treatment of these patients. Electronic medical records (EMR) offer an opportunity to assess the impact and management of medical processes and contingencies in the population. OBJECTIVE: To estimate AD prevalence in Spain over a 6-year period, based on treated patients, according to usual clinical practice. Additionally, to describe the management of AD-treated patients and the evolution of that treatment during the 2020 COVID-19 pandemic. METHODS: Retrospective study using the Spanish IQVIA EMR database. Patients treated with donepezil, galantamine, rivastigmine, and/or memantine were included in the study. Annual AD prevalence (2015-2020) was estimated and extrapolated to the national population level. Most frequent treatments and involved specialties were described. To assess the effect of COVID-19, the incidence of new AD cases in 2020 was calculated and compared with newly diagnosed cases in 2019. RESULTS: Crude AD prevalence (2015-2020) was estimated at 760.5 per 100,000 inhabitants, and age-standardized prevalence (2020) was 664.6 (male 595.7, female 711.0). Monotherapy was the most frequent way to treat AD (86.2%), in comparison with dual therapy (13.8%); rivastigmine was the most prescribed treatment (37.3%), followed by memantine (36.4%) and donepezil (33.0%). Rivastigmine was also the most utilized medication in newly treated patients (46.7%), followed by donepezil (29.8%), although donepezil persistence was longer (22.5 vs. 20.6 months). Overall, donepezil 10 mg, rivastigmine 9.5 mg, and memantine 20 mg were the most prescribed presentations. The incidence rate of AD decreased from 148.1/100,000 (95% confidence interval [CI] 147.0-149.2) in 2019 to 118.4/100,000 (95% CI 117.5-119.4) in 2020. CONCLUSIONS: The obtained prevalence of AD-treated patients was consistent with previous face-to-face studies. In contrast with previous studies, rivastigmine, rather than donepezil, was the most frequent treatment. A decrease in the incidence of AD-treated patients was observed during 2020 in comparison with 2019, presumably due to the significant impact of the COVID-19 pandemic on both diagnosis and treatment. EMR databases emerge as valuable tools to monitor in real time the incidence and management of medical conditions in the population, as well as to assess the health impact of global contingencies and interventions.

Using Explainable Artificial Intelligence in the Clock Drawing Test to Reveal the Cognitive Impairment Pattern.

The prevalence of dementia is currently increasing worldwide. This syndrome produces a deterioration in cognitive function that cannot be reverted. However, an early diagnosis can be crucial for slowing its progress. The Clock Drawing Test (CDT) is a widely used paper-and-pencil test for cognitive assessment in which an individual has to manually draw a clock on a paper. There are a lot of scoring systems for this test and most of them depend on the subjective assessment of the expert. This study proposes a computer-aided diagnosis (CAD) system based on artificial intelligence (AI) methods to analyze the CDT and obtain an automatic diagnosis of cognitive impairment (CI). This system employs a preprocessing pipeline in which the clock is detected, centered and binarized to decrease the computational burden. Then, the resulting image is fed into a Convolutional Neural Network (CNN) to identify the informative patterns within the CDT drawings that are relevant for the assessment of the patient's cognitive status. Performance is evaluated in a real context where patients with CI and controls have been classified by clinical experts in a balanced sample size of [Formula: see text] drawings. The proposed method provides an accuracy of [Formula: see text] in the binary case-control classification task, with an AUC of [Formula: see text]. These results are indeed relevant considering the use of the classic version of the CDT. The large size of the sample suggests that the method proposed has a high reliability to be used in clinical contexts and demonstrates the suitability of CAD systems in the CDT assessment process. Explainable artificial intelligence (XAI) methods are applied to identify the most relevant regions during classification. Finding these patterns is extremely helpful to understand the brain damage caused by CI. A validation method using resubstitution with upper bound correction in a machine learning approach is also discussed.

The cognitive and psychiatric subacute impairment in severe Covid-19.

Neurologic impairment persisting months after acute severe SARS-CoV-2 infection has been described because of several pathogenic mechanisms, including persistent systemic inflammation. The objective of this study is to analyze the selective involvement of the different cognitive domains and the existence of related biomarkers. Cross-sectional multicentric study of patients who survived severe infection with SARS-CoV-2 consecutively recruited between 90 and 120 days after hospital discharge. All patients underwent an exhaustive study of cognitive functions as well as plasma determination of pro-inflammatory, neurotrophic factors and light-chain neurofilaments. A principal component analysis extracted the main independent characteristics of the syndrome. 152 patients were recruited. The results of our study preferential involvement of episodic and working memory, executive functions, and attention and relatively less affectation of other cortical functions. In addition, anxiety and depression pictures are constant in our cohort. Several plasma chemokines concentrations were elevated compared with both, a non-SARS-Cov2 infected cohort of neurological outpatients or a control healthy general population. Severe Covid-19 patients can develop an amnesic and dysexecutive syndrome with neuropsychiatric manifestations. We do not know if the deficits detected can persist in the long term and if this can trigger or accelerate the onset of neurodegenerative diseases.

"Don't know" sign: description and evaluation of its diagnostic accuracy for cognitive impairment.

OBJECTIVES: Patients in neurology clinics are sometimes not aware of the reason for the consultation, and we have called this circumstance the "Don't know" sign (DKS). Our objective was to define this new sign and its modalities and to evaluate its prevalence and its diagnostic accuracy for cognitive impairment (CI) in comparison to other observation-based signs. DESIGN, SETTING, AND PARTICIPANTS: A cross-sectional prospective study included all new outpatients evaluated by the authors at neurology consultation. MEASUREMENTS: We recorded observation-based signs. The Global Deterioration Scale (GDS) was used to assess the cognitive status of patients, based on clinical history, caregiver interview, and cognitive test results. We analyzed the prevalence and the diagnostic accuracy for CI of DKS, "head turning sign," "attending with," verbal repetition, and combinations, calculating sensitivity (Se), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV). RESULTS: We enrolled 673 consecutive patients (62% female) with a mean ± SD age of 59.3 ± 20.2 years. DKS was positive in 94 patients (14%) and was strongly associated with GDS score. DKS had a Se of 0.41, Sp of 0.98, PPV of 0.89, and NPV of 0.79 for CI diagnosis. The presence of at least two positive observation signs yielded a Se of 0.50, Sp of 0.97, PPV of 0.86, and NPV of 0.81. CONCLUSIONS: DKS is frequently observed in neurology outpatients. It has low sensitivity but high specificity and PPV for CI diagnosis. It does not require additional consultation time, and its use can be recommended in combination with other observation-based signs.

PET-Amyloid After Inconclusive Cerebrospinal Fluid Biomarkers in Clinical Practice. Is it Necessary to Duplicate Procedures?

INTRODUCTION: In the absence of a gold standard for in vivo Alzheimer disease (AD) diagnosis, AD biomarkers such as cerebrospinal fluid biomarkers (CSF-B) and PET-Amyloid are considered diagnostically useful in clinical practice guidelines and have consensual appropriate use criteria (AUC). However, little evidence has been published on their utilization in the clinical setting or on approaches to mismatched results. The objective of this work was to evaluate the use of AD biomarkers in clinical practice, focusing on the implementation of PET-Amyloid in cases of inconclusive CSF-B. METHODS: This naturalistic, ambispective case series included patients fulfilling AUC for CSF-B and PET-Amyloid whose CSF-B results were non-diagnostic (target population), analyzing the diagnostic certainty, the treatment approach, and the relationship between CSF-B and PET-Amyloid results. RESULTS: Out of 2373 eligible patients, AD biomarkers were studied in 417 (17.6%), most frequently due to cognitive impairment in under 65-year-olds, using CSF-B in 311 patients and PET-Amyloid in 150. CSF-B results were non-diagnostic for 44 patients (52.3% male; aged 60.9±6.6 years), who then underwent PET-Amyloid study, which was positive in 31. A 'k' coefficient of 0.108 was obtained between CSF-B and PET-amyloid (54.5% concordance). In multivariate regression analysis, Aß42 was the only significant predictor (p= 0.018) of a positive PET-Amyloid result. In the target population, PETAmyloid increased diagnostic confidence by 53.7% (p <0.001) and modified the therapeutic approach in 36.4% of cases. CONCLUSION: These findings support the duplication of AD biomarkers and demonstrate that the implementation of PET-Amyloid provides an early and certain diagnosis to guide appropriate treatment.

Discriminative validity of an abbreviated Semantic Verbal Fluency Test.

Semantic verbal fluency (SVF) is one of the most widely used tests for cognitive assessment due to its diagnostic utility (DU). OBJECTIVE: our objective is to evaluate the DU to detect cognitive impairment (CI) of a short version of the SVF applied in 30 seconds (SVF1-30). METHODS: a prospective sample of consecutive patients evaluated in a Neurology Unit between December 2016 and December 2017 were assessed with the Global Deterioration Scale (GDS), 30-second and 60-second SVF tests (animals), and the Fototest, which includes a fluency task of people's names. The DU for CI was evaluated by the area under the ROC curve and effect size ("d" Cohen). RESULTS: the study included 1012 patients (256 with CI, 395 with dementia). SVF1-30 shows a good correlation with GDS stage. The DU of SVF1-30 is identical to that of the classical version, applied in 60 seconds, (SVFtotal) for CI (0.89 ± 0.01; p > 0.50), and shows no significant difference for dementia (0.85 ± 0.01 vs. 0.86 ± 0.01, p > 0.15). DISCUSSION: the DU of SVF1-30 is similar to that of the SVFtotal, allowing a reduction in examination time with no loss of discriminative capacity.

A fluência verbal semântica (SVF) é um dos testes mais utilizados na avaliação cognitiva devido à sua utilidade diagnóstica (UD). OBJETIVO: Nosso objetivo foi o de avaliar o DU de uma versão abreviada do SVF aplicado em 30 segundos (SVF1-30) para a detecção do comprometimento cognitivo (CC). MÉTODOS: Amostra prospectiva de pacientes avaliados em uma Unidade de Neurologia entre dezembro de 2016 e dezembro de 2017. Global Deterioration Scale (GDS), um teste de SVF (animais), registrando os resultados em 30 e 60 segundos e Fototest, que inclui uma tarefa de fluência de nomes de pessoas foram aplicadas. A UD para CC foi avaliada pela área sob a curva ROC e o tamanho do efeito ("d" Cohen). RESULTADOS: foram incluídos 1012 sujeitos (256 CC e 395 demência). O SVF1-30 mostrou uma boa correlação com o estágio GDS. A UD de SVF1-30 é idêntico ao da versão clássica (SVFtotal) para CC (0,89 ± 0,01; p > 0,50) e sem diferença significativa para demência (0,85 ± 0,01 vs. 0,86 ± 0,01; p > 0,15). DISCUSSÃO: a UD do SVF1-30 é similar ao SVFtotal, o que permite diminuir o tempo de exploração sem perder a capacidade discriminativa.

Impact of amyloid-PET in daily clinical management of patients with cognitive impairment fulfilling appropriate use criteria.

To evaluate the use of amyloid-positron emission tomography (PET) in routine clinical practice, in a selected population with cognitive impairment that meets appropriate use criteria (AUC).A multicenter, observational, prospective case-series study of 211patients from 2 level-3 hospitals who fulfilled clinical AUC for amyloid-PET scan in a naturalistic setting. Certainty degree was evaluated using a 5-point Likert scale: 0 (very low probability); 1 (low probability); 2 (intermediate probability); 3 (high probability); and 4 (practically sure), before and after amyloid PET. The treatment plan was considered as cognition-specific or noncognition-specific.Amyloid-PET was positive in 118 patients (55.9%) and negative in 93 patients (44.1%). Diagnostic prescan confidence according amyloid-PET results showed that in both, negative and positive-PET subgroup, the most frequent category was intermediate probability (45.7% and 55.1%, respectively). After the amyloid-PET, the diagnostic confidence showed a very different distribution, that was, in the negative-PET group the most frequent categories are very unlikely (70.7%) and unlikely (29.3%), while in the positive-PET group were very probable (57.6%) and practically sure (39%). Only in 14/211 patients (6.6%) the result of the amyloid-PET did not influence the diagnostic confidence, while in 194 patients (93.4%), the diagnostic confidence improved significantly after amyloid-PET results. The therapeutic intention was modified in 93 patients (44.1%). Specific treatment for Alzheimer disease was started, before amyloid-PET, in 80 patients (37.9%).This naturalistic study provides evidence that the implementation of amyloid-PET is associated with a significant improvement in diagnostic confidence and has a high impact on the therapeutic management of patients with mild cognitive impairment fulfilled clinical AUC.

Signo de la banda en la esclerosis lateral primaria / Band sign in primary lateral sclerosis

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Discriminative validity of an abbreviated Semantic Verbal Fluency Test / Validade discriminativa de um teste abreviado de Fluência Verbal Semântica

ABSTRACT. Semantic verbal fluency (SVF) is one of the most widely used tests for cognitive assessment due to its diagnostic utility (DU). Objective: our objective is to evaluate the DU to detect cognitive impairment (CI) of a short version of the SVF applied in 30 seconds (SVF1-30). Methods: a prospective sample of consecutive patients evaluated in a Neurology Unit between December 2016 and December 2017 were assessed with the Global Deterioration Scale (GDS), 30-second and 60-second SVF tests (animals), and the Fototest, which includes a fluency task of people's names. The DU for CI was evaluated by the area under the ROC curve and effect size ("d" Cohen). Results: the study included 1012 patients (256 with CI, 395 with dementia). SVF1-30 shows a good correlation with GDS stage. The DU of SVF1-30 is identical to that of the classical version, applied in 60 seconds, (SVFtotal) for CI (0.89 ± 0.01; p > 0.50), and shows no significant difference for dementia (0.85 ± 0.01 vs. 0.86 ± 0.01, p > 0.15). Discussion: the DU of SVF1-30 is similar to that of the SVFtotal, allowing a reduction in examination time with no loss of discriminative capacity.

RESUMO. A fluência verbal semântica (SVF) é um dos testes mais utilizados na avaliação cognitiva devido à sua utilidade diagnóstica (UD). Objetivo: Nosso objetivo foi o de avaliar o DU de uma versão abreviada do SVF aplicado em 30 segundos (SVF1-30) para a detecção do comprometimento cognitivo (CC). Métodos: Amostra prospectiva de pacientes avaliados em uma Unidade de Neurologia entre dezembro de 2016 e dezembro de 2017. Global Deterioration Scale (GDS), um teste de SVF (animais), registrando os resultados em 30 e 60 segundos e Fototest, que inclui uma tarefa de fluência de nomes de pessoas foram aplicadas. A UD para CC foi avaliada pela área sob a curva ROC e o tamanho do efeito ("d" Cohen). Resultados: foram incluídos 1012 sujeitos (256 CC e 395 demência). O SVF1-30 mostrou uma boa correlação com o estágio GDS. A UD de SVF1-30 é idêntico ao da versão clássica (SVFtotal) para CC (0,89 ± 0,01; p > 0,50) e sem diferença significativa para demência (0,85 ± 0,01 vs. 0,86 ± 0,01; p > 0,15). Discussão: a UD do SVF1-30 é similar ao SVFtotal, o que permite diminuir o tempo de exploração sem perder a capacidade discriminativa.

TMA-93 for Diagnosing Amnestic Mild Cognitive Impairment: A Comparison With the Free and Cued Selective Reminding Test.

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.

'Apuntes en Neurología': una síntesis de la evidencia en trastornos neurológicos comunes paroxísticos y en trastornos neurodegenerativos / 'Apuntes en Neurología' (Notes in Neurology): a synthesis of the evidence on common paroxysmal neurological disorders and on neurodegenerative disorders

Apuntes en Neurología' es una iniciativa en la cual líderes de primera línea nacional e internacional, con amplio reconocimiento académico, se reunieron para sintetizar los aspectos clínicos más destacables dentro de su área de interés y acercar las novedades en una lengua más próxima. Entender los factores que afectan al inicio y progresión de cualquier enfermedad neurológica a través de una revisión es importante para el desarrollo de estrategias en pro de reducir la carga de estas enfermedades, y conocer los aspectos clínicos es esencial para poder resolver los problemas de la practica clínica diaria. Los datos aquí recogidos reflejan el peso de la evidencia y algunos de ellos anticipan un futuro prometedor en el tratamiento de estas enfermedades. Esta primera edición se centra en trastornos neurológicos comunes paroxísticos como la migraña, la epilepsia y las alteraciones del sueño, y en trastornos neurodegenerativos como la enfermedad de Parkinson y el deterioro cognitivo. Se trata de patologías claramente diferentes, si bien algunas de ellas, como la migraña y la epilepsia, pueden compartir sintomatología clínica. Los trastornos del sueño, por su parte, son manifestaciones importantes de enfermedades neurodegenerativas que, en ocasiones, son clínicamente evidentes mucho antes del inicio de otros síntomas neurológicos. Tras recordar la fisiopatología y el diagnostico, la revisión actual se centra en acercar los principales avances en cinco de las principales enfermedades neurológicas

Introduction. 'Apuntes en Neurologia' is an initiative in which prominent national and international leaders, with broad academic recognition, came together to synthesise the most outstanding clinical aspects within their area of interest and to discuss the latest developments in a more accessible language. Understanding the factors that affect the onset and progression of any neurological disease through a review is important to be able to develop strategies to reduce the burden of these diseases. Moreover, knowledge of the clinical aspects is essential to solve the problems of daily clinical practice. The data collected here reflect the weight of evidence and some of them anticipate a promising future in the treatment of these diseases. This first edition focuses on common paroxysmal neurological disorders such as migraine, epilepsy and sleep disorders, as well as neurodegenerative disorders such as Parkinson' disease and cognitive impairment. These are clearly different pathologies, although some of them such as migraine and epilepsy, may share clinical symptoms. Sleep disorders, however, are important manifestations of neurodegenerative diseases that are sometimes clinically apparent long before the onset of other neurological symptoms. After recalling pathophysiology and diagnosis, the current review focuses on bringing together the main advances in five of the major neurological diseases

Usefulness of Dual-Point Amyloid PET Scans in Appropriate Use Criteria: A Multicenter Study.

BACKGROUND: Biomarkers of neurodegeneration play a major role in the diagnosis of Alzheimer's disease (AD). Information on both amyloid-ß accumulation, e.g., from amyloid positron emission tomography (PET), and downstream neuronal injury, e.g., from 18F-fluorodeoxyglucose (FDG) PET, would ideally be obtained in a single procedure. OBJECTIVE: On the basis that the parallelism between brain perfusion and glucose metabolism is well documented, the objective of this work is to evaluate whether brain perfusion estimated in a dual-point protocol of 18F-florbetaben (FBB) PET can be a surrogate of FDG PET in appropriate use criteria (AUC) for amyloid PET. METHODS: This study included 47 patients fulfilling international AUC for amyloid PET. FDG PET, early FBB (pFBB) PET (0-10 min post injection), and standard FBB (sFBB) PET (90-110 min post injection) scans were acquired. Results of clinical subjective reports and of quantitative region of interest (ROI)-based analyses were compared between procedures using statistical techniques such as Pearson's correlation coefficients and t-tests. RESULTS: pFBB and FDG visual reports on the 47 patients showed good agreement (k  >  0.74); ROI quantitative analysis indicated that both data modalities are highly correlated; and the t-test analysis does not reject the null hypothesis that data from pFBB and FDG examinations comes from independent random samples from normal distributions with equal means and variances. CONCLUSIONS: A good agreement was found between pFBB and FDG data as obtained by subjective visual and quantitative analyses. Dual-point FBB PET scans could offer complementary information (similar to that from FDG PET and FBB PET) in a single procedure, considering pFBB as a surrogate of FDG.

Epilepsy and ovarian failure: Two cases of adolescent-onset ovarioleukodystrophy.

Vanishing white matter disease (VWM) was described by Van der Knaap in 1996. This association with premature ovarian failure is known as ovarioleukodystrophy. This is a rare entity caused by a mutation in one of the subunits of eukaryotic initiation factor 2B (EIF2B). The onset in adulthood or late in adolescence is very infrequent. A 41-years-old woman and her 37-years-old sister developed epilepsy in association with premature ovarian failure at the age of 13 and 18 respectively. The oldest-one started 17 years later progressive subcortical cognitive decline with predominant behavioural disorders and a progressive spastic paraparesis in association with symmetric cystic changes in the with matter of both hemispheres. In both patients we found the c.1117C>T (p.Arg373Cys) mutation in homozygosis in the EIF2B4 gen.

Oral Hygiene in the Elderly with Different Degrees of Cognitive Impairment and Dementia.

The control of bacterial dental plaque through daily oral hygiene is essential to prevent oral diseases such as caries or periodontal disease, especially in at-risk populations, including the elderly with mild cognitive impairment and dementia. The aim of this study was to determine the association between different levels of cognitive impairment and dementia in an elderly population and their capacity to maintain adequate oral hygiene. A case-control study (elderly with versus without mild cognitive impairment or dementia) was performed in Granada, Spain. Outcome variables were tooth/prosthesis-brushing frequency/day, bacterial plaque index, and gingival bleeding index. Statistical models were adjusted by age, sex, educational level, and tobacco and alcohol habits. The study included 240 cases and 324 controls. The final model, adjusted by age, sex, educational level, and tobacco and alcohol consumption, showed a significant association between degree of cognitive impairment and daily oral hygiene, accumulation of bacterial plaque, and gingival bleeding. In summary, deficient daily oral hygiene, evidenced by greater bacterial dental plaque accumulation and gingival inflammation, is independently associated with cognitive impairment, even at its earliest stage.

Prevalence of Drug-Induced Xerostomia in Older Adults with Cognitive Impairment or Dementia: An Observational Study.

BACKGROUND: Older adults, especially those with cognitive impairment or dementia, frequently consume drugs with potential xerostomic effects that impair their quality of life and oral health. OBJECTIVES: The objective of this study was to determine the prevalence and analyze the possible pharmacological etiology of xerostomia in older people with or without cognitive impairment. METHODS: Individuals with cognitive impairment were recruited from patients diagnosed using standardized criteria in two neurology departments in Southern Spain. A comparison group was recruited from healthcare centers in the same city after ruling out cognitive impairment. Data on oral health, xerostomia, and drug consumption were recorded in both groups. Dry mouth was evaluated using a 1-item questionnaire and recording clinical signs of oral dryness. All drugs consumed by the participants were recorded, including memantine, anticholinesterases, antipsychotics, antidepressants, and anxiolytics. RESULTS: The final sample comprised 200 individuals with mild cognitive impairment or dementia and 156 without. Xerostomia was present in 70.5 % of participants with cognitive impairment versus 36.5 % of those without, regardless of the drug consumed. Memantine consumption was the only variable significantly related to xerostomia in the multivariate model (OR 3.1; 95 % CI 1.1-8.7), and this relationship persisted after adjusting for possible confounders and forcing the inclusion of drugs with xerostomic potential. CONCLUSIONS: More than 70 % of participants diagnosed with cognitive impairment or dementia had xerostomia. Anticholinesterases and memantine were both associated with the presence of xerostomia. In the case of memantine, this association was independent of the consumption of the other drugs considered.

Evaluación de la equivalencia de tres versiones paralelas del Fototest / Evaluation of the equivalence of three parallel versions of the Phototest

Introducción: el Fototest es un test cognitivo muy breve y aplicable a analfabetos que se ha mostrado válido y coste- efectivo en la detección del deterioro cognitivo y la demencia y en el seguimiento de estos pacientes. Las aplicaciones repetidas de un test cognitivo pueden inducir mejorías en el rendimiento debido al fenómeno «efecto de la práctica »; este efecto puede minimizarse con el uso de formas paralelas. Nuestro objetivo es evaluar la equivalencia de tres versiones paralelas del Fototest. Material y métodos: estudio transversal en una muestra de conveniencia; los sujetos se distribuyeron aleatoriamente en tres grupos a los que se aplicó respectivamente la versión original (Fototest- 1) y dos versiones paralelas (Fototest-2 y Fototest-3) del Fototest, que diferían en los objetos por denominar y recordar. Análisis estadístico: estadística descriptiva univariada y comparación entre grupos con ANOVA o χ2 según tipo de variables. Resultados: 223 sujetos (65,3 % mujeres) con una edad de 58,0 ± 16,8 (media ± DE) años, distribuidos aleatoriamente en tres grupos de 75 (Fototest-1), 76 (Fototest-2) y 72 (Fototest-3) sujetos; estos grupos no diferían entre sí en edad, sexo o nivel educativo. No hay diferencias significativas entre los grupos en las puntuaciones parciales (denominación, fluencia hombres, fluencia mujeres, recuerdo libre y recuerdo facilitado), subtotales (fluencia total, recuerdo total) y total del Fototest (Fototest-1: 37,8 ± 5,6, Fototest-2: 36,8 ± 7,5, Fototest-3: 37,4 ± 5,8; p = 0,66). Discusión: las tres versiones del Fototest son equivalentes e intercambiables, lo que puede facilitar la labor del explorador y contrarrestar el «efecto de la práctica » asociado al uso repetido (AU)

Introduction: The Phototest is a very short cognitive test that is applicable to illiterates and has proven to be valid and cost-effective for the detection of cognitive impairment and dementia and for the follow-up of these patients. Repeated applications of a cognitive test may induce improvements in performance due to «practice effects», which can be minimized by the use of parallel forms. Our objective was to evaluate the equivalence of three parallel versions of the Phototest. Material and Methods: Cross-sectional study of a convenience sample; participants were randomly distributed into three groups for the respective application of the original version of the Phototest (Phototest-1) and two parallel versions (Phototest-2 and Phototest-3), which differ in the objects to be named and recalled. Statistical analysis: univariate descriptive statistics and comparison among groups using ANOVA or the chi-square test according to the type of variable. Results: 223 participants (65.3% females) with a mean±SD of 58.0±16.8 yrs were randomly distributed among three groups of 75 (Phototest-1), 76 (Phototest-2) and 72 (Phototest-3) participants, with no significant inter-group differences in age, sex, or educational level. The groups did not significantly differ in partial Phototest scores (naming, fluency of males, fluency of females, free recall or cued recall), subtotal scores (total fluency, total recall) or total scores (Phototest-1: 37.8±5.6, Fototest-2: 36.8±7.5, Fototest-3: 37.4±5.8; p=0.66). Discussion: The three versions of Phototest are equivalent and interchangeable, which can assist the work of the examiner and counteract the 'practice effects' associated with repeated applications (AU)

Creation and design of a test for the Evaluation of Upper Limb Apraxia (EULA) based on a cognitive model: a pilot study.

INTRODUCTION: Apraxia is regarded as neurological disorder characterized by a loss of ability to execute and carry out skilled movements and gestures despite intact motor and sensory systems, coordination, and comprehension. As reflected in the specialized literature, there are currently few tests that provide a global evaluation of this syndrome. This research created and designed a test for the Evaluation of Upper Limb Apraxia (EULA), based on theoretical models of apraxia. SUBJECTS AND METHODS: A sample of 57 patients was selected with subjective cognitive manifestations (complaints of cognitive impairment) and 39 subjects without cognitive impairment. Both groups were given the EULA test as well as other tests. The structure of the EULA was verified with principal components factor analysis, and the reliability and validity of this instrument were also calculated. RESULTS: The factor analysis classified all of the items in the test in nine factors with an explained total variance of 69.91%. The high reliability of the test was reflected in a Cronbach's alpha of 0.929 and a Guttman split-half coefficient of 0.870. The construct validity was also satisfactory as shown in the significant correlation of six of the nine factors in the test with two other well-known apraxia subtests. CONCLUSIONS: The healthy subjects had a higher test score than the subjects with complaints of cognitive impairment, which confirmed the reliability and construct validity of the EULA.

TITLE: Creacion y diseño de un test para la evaluacion de la apraxia de los miembros superiores (EULA) basado en un modelo cognitivo: un estudio piloto.Introduccion. La apraxia es un trastorno neurologico caracterizado por la dificultad en la ejecucion de habilidades gestuales aprendidas a pesar de tener preservados los sistemas motores y sensoriales, la coordinacion y la comprension, asi como de una adecuada colaboracion. Actualmente, existen pocas herramientas validadas que evaluen este sindrome de manera global. En el presente estudio, se ha creado y diseñado un test para la evaluacion de la apraxia de los miembros superiores (EULA), basado en modelos teoricos. Sujetos y metodos. Se selecciono una poblacion de 57 pacientes con quejas subjetivas de deterioro cognitivo y 39 personas sin quejas ni deterioro cognitivo, a las cuales se les administro el test EULA, entre otros tests. Se realizo un analisis factorial de componentes principales y un calculo tanto de la fiabilidad como de la validez de dicho instrumento. Resultados. El analisis factorial agrupo en nueve factores todos los items de la prueba, con una varianza total explicada del 69,91%. El test ha mostrado una alta fiabilidad, con un alfa de Cronbach de 0,929 y un coeficiente de Guttman de 0,870 con el metodo de las dos mitades. El test tambien mostro tener una adecuada validez de constructo, al existir correlacion significativa entre seis factores del test y dos subtests de apraxia. Conclusiones. El test EULA, surgido de las propuestas de evaluacion a nivel teorico desarrolladas por diferentes autores, muestra una puntuacion superior en personas sanas respecto a personas con manifestaciones subjetivas de deterioro cognitivo, ademas de tener una alta fiabilidad y validez de constructo.

Creación y diseño de un test para la evaluación de la apraxia de los miembros superiores (EULA) basado en un modelo cognitivo: un estudio piloto / Creation and design of a test for the Evaluation of Upper Limb Apraxia (EULA) based on a cognitive model: a pilot study

Introducción. La apraxia es un trastorno neurológico caracterizado por la dificultad en la ejecución de habilidades gestuales aprendidas a pesar de tener preservados los sistemas motores y sensoriales, la coordinación y la comprensión, así como de una adecuada colaboración. Actualmente, existen pocas herramientas validadas que evalúen este síndrome de manera global. En el presente estudio, se ha creado y diseñado un test para la evaluación de la apraxia de los miembros superiores (EULA), basado en modelos teóricos. Sujetos y métodos. Se seleccionó una población de 57 pacientes con quejas subjetivas de deterioro cognitivo y 39 personas sin quejas ni deterioro cognitivo, a las cuales se les administró el test EULA, entre otros tests. Se realizó un análisis factorial de componentes principales y un cálculo tanto de la fiabilidad como de la validez de dicho instrumento. Resultados. El análisis factorial agrupó en nueve factores todos los ítems de la prueba, con una varianza total explicada del 69,91%. El test ha mostrado una alta fiabilidad, con un alfa de Cronbach de 0,929 y un coeficiente de Guttman de 0,870 con el método de las dos mitades. El test también mostró tener una adecuada validez de constructo, al existir correlación significativa entre seis factores del test y dos subtests de apraxia. Conclusiones. El test EULA, surgido de las propuestas de evaluación a nivel teórico desarrolladas por diferentes autores, muestra una puntuación superior en personas sanas respecto a personas con manifestaciones subjetivas de deterioro cognitivo, además de tener una alta fiabilidad y validez de constructo (AU)

Introduction. Apraxia is regarded as neurological disorder characterized by a loss of ability to execute and carry out skilled movements and gestures despite intact motor and sensory systems, coordination, and comprehension. As reflected in the specialized literature, there are currently few tests that provide a global evaluation of this syndrome. This research created and designed a test for the Evaluation of Upper Limb Apraxia (EULA), based on theoretical models of apraxia. Subjects and methods. A sample of 57 patients was selected with subjective cognitive manifestations (complaints of cognitive impairment) and 39 subjects without cognitive impairment. Both groups were given the EULA test as well as other tests. The structure of the EULA was verified with principal components factor analysis, and the reliability and validity of this instrument were also calculated. Results. The factor analysis classified all of the items in the test in nine factors with an explained total variance of 69.91%. The high reliability of the test was reflected in a Cronbach’s alpha of 0.929 and a Guttman split-half coefficient of 0.870. The construct validity was also satisfactory as shown in the significant correlation of six of the nine factors in the test with two other well-known apraxia subtests. Conclusions. The healthy subjects had a higher test score than the subjects with complaints of cognitive impairment, which confirmed the reliability and construct validity of the EULA (AU)