Prevalence of dementia subtypes in El Prat de Llobregat, Catalonia, Spain: the PRATICON study.

BACKGROUND: Studies on dementia subtypes show a wide variation in the prevalence of Alzheimer's disease (AD) and vascular dementia (VD) worldwide. However, studies reporting on Lewy body dementia (LBD) and frontotemporal dementia (FTD) are sparse. AIMS: To describe the prevalence of dementia and subtypes. METHOD: A 34% sample of 5,150 subjects aged 70 years and over in El Prat de Llobregat (Barcelona) were screened by the Mini-Mental State Examination. When scoring <24, participants were assessed to establish a diagnosis. RESULTS: There were 165 subjects diagnosed with dementia (prevalence of 9.4%). Subtypes of dementia were: AD 69.1%, VD 12.7%, LBD 9.1%, FTD 3% and secondary dementia 1.8%. Prevalences were: AD 6.5%, VD 1.2%, LBD 0.9% and FTD 0.3%. CONCLUSIONS: AD and VD were the most common type of dementia. Prevalence of dementia, AD and FTD were similar to those reported, while prevalence of VD and LBD were lower.

[Values of GERMCIDE neuropsychological protocol in a sample of normal subjects]. / Valores del protocolo neuropsicológico GERMCIDE en una muestra de sujetos normales.

INTRODUCTION: The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function). METHODS: In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia. RESULTS: A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented. CONCLUSIONS: The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia.

Valores del protocolo neuropsicológico GERMCIDE en una muestra de sujetos normales / Values of GERMCIDE neuropsychological protocol in a sample of normal subjects

Introducción. El diagnóstico de los pacientes con deterioro cognitivo o demencia exige un enfoque global en el que la exploración neuropsicológica es una pieza clave. Como parte del estudio GERMCIDE (Grupo para el Estudio y Registro Multicéntrico de Casos Incidentes de Demencia en España) se diseñó un protocolo que incluye una valoración de las diferentes funciones cognitivas que con mayor frecuencia se alteran en las demencias (memoria, orientación, lenguaje, praxias, capacidad de abstracción y función ejecutiva). Métodos. Con el objetivo de obtener datos en sujetos normales este protocolo neuropsicológico se aplicó a un grupo de personas mayores de 50 años sin deterioro cognitivo ni demencia. Resultados. Se estudiaron 103 sujetos con edades comprendidas entre 50 y 95 años (media: 73,5; desviación estándar [DE]: '9,3 años); 39 (37,90J0) hombres y 64 (62,1 %) mujeres. La puntuación media en el Mini-Mental State Examination (MMSE) fue de 27/30 (DE: 2,0). En las pruebas de lenguaje y praxias el 900J0 de los sujetos obtuvieron el valor máximo, mientras que en memoria, razonamiento y programación los rendimientos fueron más dispares. Se presenta la puntuación media, DE y distribución en percentiles para cada subtest. Conclusiones. Los valores obtenidos en esta muestra de sujetos normales y su distribución en percentiles pueden ser de gran ayuda para facilitar la interpretación de los hallazgos de la exploración neuropsicológica con el protocolo GERMCIDE en las consultas de neurología general y también en las consultas especializadas en demencia

Introduction. The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function). Methods. In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia. Results. A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented. Conclusions. The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia

Banco de tejidos neurológicos destinados a la investigación en neurociencias. Una realidad en Navarra / Neurological tissue bank for research in neuroscience. A reality in Navarre

No disponible

Deterioro cognitivo y demencia de origen vascular / Cognitive deterioration and dementia of vascular origin

No disponible

[Multiple sclerosis and epileptic seizures]. / Esclerosis múltiple y crisis epilépticas.

INTRODUCTION: Although epileptic seizures are uncommon in multiple sclerosis they are more prevalent than in the general population, which supports an aetiological relationship. Similarly in a considerable proportion of patients with multiple sclerosis and epileptic seizures, alterations in magnetic resonance and electroencephalogram studies which could be correlated with the clinical features of epilepsy were observed. Nevertheless, it is difficult to establish definite clinical characteristics in these patients since the underlying pathogenic mechanisms are poorly understood and there is great variability with regard to the type of seizure, point at which this occurs during the course of the disease, degree of recurrence and other aspects. CLINICAL CASE: We report the clinical, electroencephalographical and neuroimaging findings of seven patients with multiple sclerosis who had epileptic seizures and those in whom there was no evidence of other potentially epileptogenic pathology. In two patients the epileptic seizures formed part of the first episode of their illness. One patient presented more than one type of epileptic seizure. These seizures were generalized in two cases, partial sensory and/or motor with secondary generalization in three, simple partial motor in one and partial complex in two. The epileptic seizures coincided with other clinical features of episodes in three cases and the electroencephalogram showed anomalies in five cases. CONCLUSIONS: The findings observed were of a wide variety, as was found in other reported series. We point out certain correlations between the clinical data, magnetic resonance and electroencephalogram which may help to orientate the management of these patients.

[Variation of Mini-Mental-State examination scores due to age and educational level. Normalized data in the population over 70 years of age in Pamplona]. / Variación de las puntuaciones en el Mini-Mental-State con la edad el nivel educativo. Datos normalizados en la población mayor de 70 años de Pamplona.

The Mini-Mental-State Examination (MMSE) is widely used as a screening tool for dementia in epidemiological studies. Its applicability in population-based studies is nevertheless limited by its low specificity. The effect of age and educational level have been usually ignored when cut-off scores have been selected. The aim of this study was to evaluate the effect of age and educational level on the MMSE scores in a representative sample of subjects older than 70 and provide adjusted normalised data according to these two variables, after excluding subjects with dementia or cognitive decline. Population-based, cross-sectional and longitudinal study of a representative cohort of 1367 subjects older than 70. All subjects with suspected dementia or cognitive decline received a neurological evaluation where clinical and etiological diagnosis were established. Normal MMSE scores, as defined by the 10th percentile, varied significantly across age and educational level groups. Exclusion of demented or cognitively declined patients from the reference population reduced the variability and "range of normality", but this remained excessively high in the older and less educated groups. The use of different cut-off points for each age and educational level groups may improve the specificity and applicability of the MMSE in population-based epidemiological studies. However, the wide amplitude of the range of normality suggests that different approaches, other than this vibariate analysis, may prove more adequate in the selection of cut-off scores for the MMSE.

[Phenotype variability in adrenoleukodystrophy. Presentation of three new cases and a review of literature]. / Variabilidad fenotípica de la adrenoleucodistrofia. Presentación de tres casos y revisión de literatura.

INTRODUCTION: Adrenoleukodystrophy is a hereditary recessive sex-linked disorder with very variable phenotype expression, including classical infantil ALD, adrenomyeloneuropathy (AMN) in adults and sex-linked Addison's disease. Clinical observations. Three affected patients are presented. The first showed signs of myeloneuropathy from the age of 38 and diagnosis was made by showing raised serum and fibroblasts levels of very long chain fatty acids (C26:0). In the second case symptoms started at the age of 13 and cerebral and peripheral nervous system changes developed progressively. This patient's brother was the third case, showing symptoms when he was 21 and developing cerebral, medullary and peripheral nervous system involvement. In the latter two cases, diagnosis was made by showing intracytoplasmatic trilaminary inclusions in the nervous system. CONCLUSIONS: It is important to recognize the different varieties of this disease in view of the possibilities of genetic counselling and of the therapeutic implications which are currently being evaluated.

Prevalence of Alzheimer's disease and other dementing disorders in Pamplona, Spain.

We investigated the prevalence of dementing disorders in the city of Pamplona, Spain, using a door-to-door two-phase approach. We first administered the Cambridge Examination of Mental Disorders of the Elderly (CAMDEX) to all survivors, as of March 1, 1991, of a probability sample of the total population identified in 1989 (n = 1,127). Using specified diagnostic criteria, the study neurologists extensively investigated those subjects who screened positive on CAMDEX. We found 194 subjects affected by dementia, 119 had Alzheimer's disease; 51 vascular or mixed dementia, and 16 secondary dementia. The prevalence of both dementia and Alzheimer's disease increased steeply with advancing age and was consistently higher in women. The prevalence of combined vascular and mixed dementia increased less rapidly with age, and was generally higher in men. Alzheimer's disease was the most common type of dementia. Our prevalence figures for dementia and Alzheimer's disease are similar to those previously reported in Europe.

[Multicenter register of index cases of dementia. A study by the Spanish Neurological Society's dementia group]. / Registro multicéntrico de casos incidentes de demencia. Un estudio del grupo de demencias de la Sociedad Española de Neurología.

We report the objectives and case registration methods used by neurologists belonging to the Spanish Neurological Society's dementia group (GERMICIDE). Forerunners of the present project are also described. The group's principal aim, which is to determine the distribution of dementia in the "neurological" environment is pursued by enrolling consecutive index cases (new presentations) arriving at 18 Spanish neurological units over a period of 2 years. A standard protocol was used to study the patients and final diagnoses were agreed upon in keeping with international criteria for dementia and the main neurological diseases (DSM-III-R, CDR and others). A second objective is to study risk factors for the main etiological groups of dementia (Alzheimer's disease and vascular dementia) and to follow each patient in the cohort until death in order to assess the course of disease and its biological markers. The series will be large, as there are already over 800 cases listed in the coordinating centre even though the period of enrollment has not ended.

[Brain photon emission tomography. Value of the corticocerebellar index and gammagraphic patterns in Alzheimer's disease and other diseases]. / Tomografía de emisión de fotones cerebral. Valor del índice corticocerebeloso y patrones gammagráficos en la enfermedad de Alzheimer y otras afecciones.

Brain single photon emission computed tomography (SPECT) with 99mTc-HMPAO is a diagnostic tool for evaluating regional cerebral blood flow. Recently, the diagnostic possibilities of the method are being investigated in some neurologic disorders, such as cerebrovascular accidents, seizures and dementia. This work has been carried out with 54 subjects, 9 healthy volunteers and 45 patients (31 dementia and 14 epileptics), in order to evaluate gammagraphic patterns and the utility of cortico/cerebellar activity indexes. An interesting diagnostic finding is a significant decrease (p less than 0.001) in perfusion of temporoparietal regions in the patients with Alzheimer's disease in relation with the healthy volunteers' group. We have not found significant changes in perfusion in the group of epileptic patients during the interictal phase. We conclude emphasizing the interest of the SPECT in the differential diagnosis of dementia.