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Background: The Alzheimer's Disease Functional Assessment and Change Scale (ADFACS) is a functional assessment instrument widely used in clinical research. Aims: To test the diagnostic and concurrent validity of the Spanish version of this scale and to describe the functional deficit pattern for mild cognitive impairment (MCI) and Alzheimer's disease (AD) dementia. Methods: The ADFACS, the Interview for Deterioration in Daily Living Activities in Dementia (IDDD), and the Mini Mental State Examination (MMSE) were administered to 146 control subjects (CS) and 165 patients (67 MCI and 98 AD). Nonparametric tests were used to compare the diagnostic groups. Cronbach's α and correlations with the MMSE and the IDDD were calculated. Sensitivity, specificity and predictive values were studied. Results: The ADFACS had a high internal consistency (α = 0.95). Three cutoff points of 1, 4, and 17 were provided to separate CS and MCI patients, MCI and mild AD patients, and mild AD and moderate AD patients, respectively. The ADFACS strongly correlated with functional (IDDD, 0.927) and cognitive (MMSE, 0.747) measures. A similar pattern of dysfunction, but in different grades, was found for the MCI and AD groups. Conclusion: The ADFACS is a reliable, valid, and sensitive instrument to assess functional abilities; it is useful in dementia assessment for elderly populations. © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Acute appendicitis (AA) is the most common surgical emergency in children. Assessment scales improve the timely detection of cases., Objective: To evaluate the association of the Pediatric Appendicitis Scale (PAS) with hospital stay (HS) and postoperative complications of appendectomy., Material and Methods: Observational, analytical, longitudinal and retrolective study. Patients from 2 months to 15 years of age, without comorbidities, who underwent appendectomy for complicated (CAA) and uncomplicated (UCAA) acute appendicitis were included. PAS score prior to an appendectomy was obtained, and the time of HS and postoperative complications were evaluated., Results: 64 patients were evaluated, age 8 (IQR 8-12) years, PAS score 6 (IQR 4-8). The time from the onset of symptoms to the request for care in the emergency department was shorter (p < 0.0001) and the total neutrophil count was higher (p = 0.001) in the CAA group. No difference was shown in the PAS score between patients with CAA and UCAA (6 [4-7] vs. 7 [4-8], p = 0.087]. A PAS score ≥ 7 was associated with an increased risk of prolonged HE (p = 0.007), but was not associated with postoperative complications. The PAS score alongside the time elapsed from the onset of symptoms until the assistance in the Pediatric Emergency Department, were associated with an increased risk of prolonged HE (R2 = 0.2246, p = 0.003)., Conclusions: A PAS score ≥ 7 is associated with prolonged HS, likewise, the PAS score alongside the time elapsed between the onset of symptoms and assistance in emergency care is associated with prolonged HS., (Licencia CC 4.0 (BY-NC-ND) © 2023 Revista Médica del Instituto Mexicano del Seguro Social.)

The application of the Boston Naming Test (BNT) is time-consuming and shortened versions need to be developed for screening purposes. The aims of this study were to develop four equivalent 15-item forms of a Spanish adaptation of the BNT, to test the equivalence of the new versions in a clinical sample, and to provide normative data. The normative sample consisted of 340 subjects. The clinical sample included 172 patients (76 Mild Cognitive Impairment and 96 Alzheimer's disease). An empirical procedure was used to develop the shortened versions. All new versions demonstrated satisfactory internal consistency. Pearson's coefficient analysis showed strong relationships among the four short-form versions as well as between each of them and the 60-item test. The inferential confidence interval method demonstrated the equivalence between the four shortened versions. Age and education affected the score of all short-form versions, but sex was found to be unrelated to the performance. Normative data were calculated for midpoint age groups. This paper proposes four 15-item equivalent versions that could be useful and time-saving tools for screening purposes.

The aim of this study was to characterize the neuropsychological and neuroimaging profiles of mild cognitive impairment (MCI) and Alzheimer's disease (AD) patients, and to study the magnitude of the differences by comparing both outcomes with healthy subjects in a cross-sectional manner. Five hundred and thirty-five subjects (356 cognitively normal adults (CONT), 79 MCI, and 100 AD) were assessed with the NEURONORMA neuropsychological battery. Thirty CONT, 23 MCI, and 23 AD subjects from this sample were included in the neuroimaging substudy. Patients' raw cognitive scores were converted to age and education-adjusted scaled ones (range 2-18) using co-normed reference values. Medians were plotted to examine the cognitive profile. MRIs were processed by means of FreeSurfer. Effect size indices (Cohen's d) were calculated in order to compare the standardized differences between patients and healthy subjects. Graphically, the observed cognitive profiles for MCI and AD groups produced near to parallel lines. Verbal and visual memories were the most impaired domains in both groups, followed by executive functions and linguistic/semantic ones. The largest effect size between AD and cognitively normal subjects was found for the FCSRT (d = 4.05, AD versus CONT), which doubled the value obtained by the best MRI measure, the right hippocampus (d = 1.65, AD versus CONT). Our results support the notion of a continuum in cognitive profile between MCI and AD. Neuropsychological outcomes, in particular the FCSRT, are better than neuroimaging ones at detecting differences among subjects.

Mild cognitive impairment (MCI) is a transitional state between normal aging and Alzheimer disease (AD). Artificial neural networks (ANNs) are computational tools that can provide valuable support to clinical decision making, classification, and prediction of cognitive functioning. The aims of this study were to develop, train, and explore and develop the ability of ANNs to differentiate MCI and AD, and to study the relevant variables in MCI and AD diagnosis. The sample consisted of 346 controls and 79 MCI and 97 AD patients. A linear discriminant analysis (LDA) and ANNs with 12 input neurons (10 subtests of a neuropsychological test, the abbreviated Barcelona Test; age; and education), 4 hidden neurons, and output neuron (diagnosis) were used to classify the patients. The ANNs were superior to LDA in its ability to classify correctly patients (100-98.33% vs. 96.4-80%, respectively) and showed better predictive performance. Semantic fluency, working and episodic memory and education showed up as the most significant and sensitive variables for classification. Our results indicate that ANNs have an excellent capacity to discriminate MCI and AD patients from healthy controls. These findings provide evidence that ANNs can be a useful tool for the analysis of neuropsychological profiles related to clinical syndromes.

The abbreviated Barcelona Test (a-BT) is an instrument widely used in Spain and Latin American countries for general neuropsychological assessment. The purpose of the present study was to provide new norms for the a-BT as part of the Neuronorma project. The sample consisted of 346 healthy controls. Overlapping cell procedure and midpoint techniques were applied to develop the normative data. Age, education, and sex influences were studied. Results indicated that although age and education affected the score on this test, sex did not. Raw scores were transformed to age-adjusted scaled scores (SS(A)) based on percentile ranks. These SS(A) were also converted into age-education scaled scores using a linear regression model. Norms were presented on age-education scaled scores. Also, the a-BT cognitive profile was presented and should prove to be clinically useful for interpretation. These co-normed data will allow clinicians to compare scores from a-BT with all the tests included in the Neuronorma project.

This study forms part of the Spanish Multicenter Normative Studies (NEURONORMA project). Normative data for people aged over 49 years are presented for selected tasks of the visual object and space perception battery (VOSP) and for the judgment of line orientation (JLO) test. Age-adjusted norms were derived from a sample of 341 participants who are cognitively normal and community-dwelling. Age- and education-adjusted norms are also provided. Years of education were modeled on age-scaled scores to derive regression equations that were applied for further demographic adjustments. The normative information provided here should prove useful for characterizing and interpreting individual test performances as well as comparing the scores from these tests with any other test using NEURONORMA norms.

Lexical fluency tests are frequently used in clinical practice to assess language and executive function. As part of the Spanish multicenter normative studies (NEURONORMA project), we provide age- and education-adjusted norms for three semantic fluency tasks (animals, fruit and vegetables, and kitchen tools), three formal lexical tasks (words beginning with P, M, and R), and three excluded letter fluency tasks (excluded A, E, and S). The sample consists of 346 participants who are cognitively normal, community dwelling, and ranging in age from 50 to 94 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. The current norms should provide clinically useful data for evaluating elderly Spanish people. These data may also be of considerable use for comparisons with other international normative studies. Finally, these norms should help improve the interpretation of verbal fluency tasks and allow for greater diagnostic accuracy.

As part of the NEURONORMA project, we provide age- and education-adjusted norms for the Stroop color-word interference test (SCWT)-Golden version and the Tower of London-Drexel University version (TOL(DX)). The sample consists of 344 and 347 participants, respectively, who are cognitively normal, community dwelling, and ranging in age from 50 to 90 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. Demographic variables, age, and education significantly affect scores of the SWCT and TOL(DX), sex, however, was found to be unrelated to performance in this sample. The normative data presented here were obtained from the same study sample as all the other NEURONORMA tests. In addition, the same statistical procedures for data analyses were applied. These co-normed data allow clinicians to compare scores from one test with all tests.

As part of the Spanish Multicenter Normative Studies (NEURONORMA project), we provide age- and education-adjusted norms for the following instruments: verbal span (digits), visuospatial span (Corsi's test), letter-number sequencing (WAIS-III), trail making test, and symbol digit modalities test. The sample consists of 354 participants who are cognitively normal, community-dwelling, and age ranging from 50 to 90 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. The current norms should provide clinically useful data for evaluating elderly Spanish people. These data may be of considerable use for comparisons with other normative studies. Limitations of these normative data are mainly related to the techniques of recruitment and stratification employed.

The Rey-Osterrieth complex figure (ROCF) and the free and cued selective reminding test (FCSRT) are frequently used in clinical practice. The ROCF assesses visual perception, constructional praxis, and visuospatial memory, and the FCSRT assesses verbal learning and memory. As part of the Spanish Normative Studies (NEURONORMA), we provide age- and education-adjusted norms for the ROCF (copy and memory) and for the FCSRT. The sample consists of 332 and 340 participants, respectively, who are cognitively normal, community dwelling, and ranging in age from 50 to 94 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. Although age and education affected the score of the ROCF and FCSRT, sex was found to be unrelated in this normal sample. The normative data presented here were obtained from the same study sample as all other NEURONORMA norms and the same statistical procedures were applied. These co-normed data will allow clinicians to compare scores from one test with all the tests included in the project.

This paper describes the methods and sample characteristics of a series of Spanish normative studies (The NEURONORMA project). The primary objective of our research was to collect normative and psychometric information on a sample of people aged over 49 years. The normative information was based on a series of selected, but commonly used, neuropsychological tests covering attention, language, visuo-perceptual abilities, constructional tasks, memory, and executive functions. A sample of 356 community dwelling individuals was studied. Demographics, socio-cultural, and medical data were collected. Cognitive normality was validated via informants and a cognitive screening test. Norms were calculated for midpoint age groups. Effects of age, education, and sex were determined. The use of these norms should improve neuropsychological diagnostic accuracy in older Spanish subjects. These data may also be of considerable use for comparisons with other normative studies. Limitations of these normative data are also commented on.

As part of the Spanish Multicenter Normative Studies (NEURONORMA project), we provide age- and education-adjusted norms for the Boston naming test and Token test. The sample consists of 340 and 348 participants, respectively, who are cognitively normal, community-dwelling, and ranging in age from 50 to 94 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. Age and education affected the score of the both tests, but sex was found to be unrelated to naming and verbal comprehension efficiency. Our norms should provide clinically useful data for evaluating elderly Spaniards. The normative data presented here were obtained from the same study sample as all the other NEURONORMA norms and the same statistical procedures for data analyses were applied. These co-normed data allow clinicians to compare scores from one test with all tests.

Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE; MIM 600513) has been associated with mutations in the genes coding for the alfa-4 (CHRNA4), beta-2 (CHRNB2), and alpha-2 (CHRNA2) subunits of the neuronal nicotinic acetylcholine receptor (nAChR) and for the corticotropin-releasing hormone (CRH). A four-generation ADNFLE family with six affected members was identified. All affected members presented the clinical characteristics of ADNFLE. Interictal awake and sleep EEG recordings showed no epileptiform abnormalities. Ictal video-EEG recordings showed focal seizures with frontal lobe semiology. Mutation analysis of the CHRNB2 gene revealed a c.859G>A transition (Val287Met) within the second transmembrane domain, identical to that previously described in a Scottish ADNFLE family. To our knowledge, this is the third family reported presenting a mutation in CHRNB2. The clinical phenotype appears similar to that described with mutations in CHRNA4, suggesting that mutations in these two subunits lead to similar functional alterations of the nAChR.

During recent years a new definition for transient ischemic attack (TIA) has been proposed. This has been based on the advances in neuroimaging techniques and because it has been observed that most TIA last only a few minutes. Brain damage must be ruled out and TIA duration can be no longer than one hour. TIA increases the chance of stroke or vascular episodes, above all during the first days and of other vascular diseases such as ischemic heart disease. It is a prevalent condition which must be considered as an emergency even though the patient is usually asymptomatic. For the initial evaluation, routine blood test, electrocardiogram, chest X-ray, brain computed tomography and extra and intracranial ultrasonography study must be performed. Treatment is based on the control of risk factors and antithrombotic therapy.

Introduction: Anticonvulsant hypersensitivity syndrome (AHS) is characterised by fever, skin rashes and involvement of the internal organs. Owing to the low frequency with which it appears and its high clinical heterogeneity, it is not always suspected. Moreover, the symptoms often overlap with those of a vasculitis or of an infection. The most commonly associated antiepileptic drugs (AED) are the aromatic agents. We report the case of a female patient who developed AHS with several different AED and presented an especially severe kidney and skin disorder due to carbamazepine (CBZ)., Case Report: We describe the case of a 26-year-old woman who, after being diagnosed as suffering from secondarily generalised partial seizures, began treatment with 200 mg/12 hours CBZ. A few weeks later, she developed itchy skins lesions compatible with exanthematic pustulosis, together with acute kidney failure requiring haemodialysis. A biopsy study of the kidney revealed immunoallergic tubulointerstitial nephropathy, which is a lesion that has only very occasionally been reported in relation to CBZ therapy. The patient also presented a moderate rise in the level of transaminases and leukocytosis with eosinophilia. She was discharged from hospital without AED but suffered new seizures and was treated with phenytoin and, later, with valproic acid, both as monotherapy. With these drugs she developed AHS consisting in fever, rashes, eosinophilia and subclinical hepatitis. In epicutaneous tests with anticonvulsants, the three AED presented a positive reading, as well as others. The patient was treated with tiagabine, and there were no further hypersensitivity phenomena and a good control of seizures was achieved., Conclusions: AHS is an infrequent, but potentially serious, clinical entity and must therefore be suspected in patients taking AED who develop fever, rashes or disorders affecting the internal organs.

Background: Bradykinesia is the clinical feature of Huntington's disease most closely related to functional discapacity and stage, probably as a consequence of spreading of neuronal injury. The aim of the present article is to verify whether a choice reaction time could be considered an estimate measurement of clinical bradykinesia, and its possible relationships with other evolutive parameters such as functional discapacity, clinical stage and prefrontal executive dysfunction., Methods: Fifteen patients were studied (9 in advanced stage and 6 initials), equal number of controls and 3 asymptomatic gene carriers. We used clinical bradykinesia and functional capacity scales, an extensive prefrontal battery and a computerized paradigm of reaction time., Results: Clinical bradykinesia and reaction time are closely related. The associations between reaction time with those parameters indicatives of prefrontal dysfunction, functional discapacity and clinical stage are closer and more significatives than those that could be established with clinical bradykinesia., Conclusions: Reaction time is an objective measurement of global motor slowness that allow us assigning each subject to a specific stage, and avoid possible errors derived from interobserver bias in clinical scales.

To furnish greater specificity in the analysis of cerebrospinal fluid from patients suspected of having multiple sclerosis, we studied the sensitivity and efficiency of indices and formulas used to calculate intrathecal IgG synthesis in a group of 49 patients with clinically defined multiple sclerosis, using cutoff values based on preestablished levels of specificity (75 and 90%), and compared the findings to those for a control group of patients with other neurological diseases. The best results were obtained with the indices and formulas based on computer models of brain-blood barrier function set forth most recently, namely Reiber's formula and Ohman's index, which had the highest specificity with the least loss of sensitivity.

In the present study the lesions in MRI imaging were quantified and compared with the clinical and functional abnormalities in 56 patients (27 females and 29 males) with definite multiple sclerosis (MS). The evolution was relapsing in 30, there were relapses followed by progressive evolution in 10, and evolution was progressive from the onset in 16. A good correlation was found between the disability scale EDSS and total lesions in MRI (r = 0.45), and also with the disability scale EDSS and hemispheric (r = 0.45) and centrioval (r = 0.41) involvements and with lesions near the third ventricle (r = 0.32). The clinical parameters predicting a greater surface of involvement in MRI were late onset (beyond 45 years) and progressive evolution. The best correlation between disability scale and MRI lesions was found between EDSS and periventricular (lateral ventricles) lesions (r = 0.46). The linearly correlation between both disability scales was good (r = 0.86), but it improved when an exponential equation was used (r = 0.91). This could allow to use the more simple scale (ISS) with a mathematical transformation. When the patients with a greatest surface of involvement were compared with the remaining MS patients, significant differences were found for nearly all evaluated disability items. Patients with greater involvement surfaces in MRI had greater disability.

We have carried out a follow-up study in 70 patients that were included in a follow-up protocol when they met the criteria of MS, so as to evaluate the variations in the disability scale per unit of time of follow-up (index of progression) in relation to the several risk factors. There were 31 females and 39 men followed up for 19.6 +/- 12 months. One half of the patients (35) had their first symptoms between 25 and 45 years of age, in 27 the onset was after age 25 and in 8 after age 45. At the end of follow-up 42 patients were classified as having remitting forms, 12 as progressive forms after a remitting phase and 16 as progressive forms from the onset. Significant differences in the evolutive forms were only found in remitting-progressive forms, which had a quicker progression than the purely remitting and chronic progressive forms. The patients who initially had cerebellar symptoms had a quicker progression than those with any other type of presentation. Whereas patients with late onset had a quicker progression than the group with intermediate onset, the patients with early onset had a slower progression.

To assess the presence of antiviral antibody synthesis in the cerebrospinal fluid in patients with multiple sclerosis, concurrent plasma and spinal fluid determination of antibody titers against measles, varicella-zoster, rubella, mumps, cytomegalovirus, and herpes viruses were performed in 29 samples and were compared with a control group. The study revealed an increased titre of antiviral antibodies in the spinal fluid in patients with multiple sclerosis. This increased activity was markedly significant for the varicella-zoster and cytomegalovirus in patients with clinical symptoms of multiple sclerosis. There was also and increased antibody titre against cytomegalovirus and varicella-zoster in patients with well defined illness. No antibody reaction was observed in the control group. The study of the antiviral antibody activity in the spinal fluid in patients with multiple sclerosis is useful in the follow-up control and in the diagnosis of the disorder specially in our community, where the investigation of antibodies anti cytomegalovirus appears to be the most appropriate method due to its high sensitivity and absence of false positive tests.