[Rivastigmine in a case of autopsy proved frontotemporal dementia (Pick's disease)]. / Rivastigmina en un caso de demencia frontotemporal, enfermedad de Pick verificada por autopsia.

INTRODUCTION: Cholinesterase inhibitors are useful in the treatment of behavioural and psychological symptoms in Alzheimer's disease. Their effectiveness in frontotemporal dementia has not been proved, since such a claim has only been backed by the publication of one open-label trial in which the behavioural and psychological symptoms of the patients treated with rivastigmine over a 12-month period improved significantly with respect to those belonging to a group that were given a placebo. We report a case of frontotemporal dementia, Pick's disease, which improved with rivastigmine treatment. CASE REPORT: A 61-year-old male who presented a progressive clinical picture of behavioural disorders and executive-cognitive impairment that had begun two years earlier. Magnetic resonance imaging of the head revealed severe frontotemporal atrophy. Neuropsychological Inventory (NPI). Overall score 36/144 (6/12: anxiety, disinhibition and aberrant motor behaviour, 4/12: agitation, irritability and apathy; 3/12: sleep and eating disorders. After three months' treatment with rivastigmine, the overall score on the NPI was 10/144. This improvement remained stable over the months that followed. The patient died eight months later after developing liver cancer with metastasis. The microscopic study of the brain showed tau-positive neuronal inclusions, gliosis and neuronal loss. The inclusions were well-circumscribed Pick bodies, which were present in the frontal and temporal cortices and in the dentate gyrus of the hippocampus. CONCLUSIONS: This case confirms the idea that treatment with cholinesterase inhibitors can be effective in the behavioural and psychological symptoms of frontotemporal dementia.

Rivastigmina en un caso de demencia frontotemporal, enfermedad de Pick verificada por autopsia / Rivastigmine in a case of autopsy proved frontotemporal dementia (pick´s disease)

Introducción. Los inhibidores de la colinesterasa son útiles en el tratamiento de los síntomas conductuales y psicológicos en la enfermedad de Alzheimer. Su eficacia en la demencia frontotemporal no está demostrada, pues sólo la avala la publicación de un estudio abierto en el que los síntomas conductuales y psicológicos de los pacientes tratados con rivastigmina durante 12 meses mejoraron significativamente respecto de los del grupo placebo. Describimos un caso de demencia frontotemporal, enfermedad de Pick, que mejoró con el tratamiento con rivastigmina. Caso clínico. Varón de 61 años de edad que presentaba un cuadro clínico progresivo de alteraciones de conducta y afectación ejecutivocognitiva de dos años de evolución. En la resonancia magnética craneal se observó una atrofia frontotemporal grave. En el inventario neuropsicológico (NPI) alcanzó una puntuación global de 36/144 (6/12: ansiedad, desinhibición y conducta motora aberrante, 4/12: agitación, irritabilidad y apatía; 3/12: alteraciones del sueño y de la conducta alimentaria). Tras tres meses de tratamiento con rivastigmina, la puntuación global en el NPI fue de 10/144. Esta mejoría se mantuvo estable en los siguientes meses. Falleció ocho meses después tras desarrollar un hepatocarcinoma con metástasis. En el estudio microscópico del cerebro se observaron inclusiones neuronales tau positivas, pérdida neuronal y gliosis. Las inclusiones eran cuerpos de Pick, bien circunscritos, presentes en la corteza frontal, temporal y en el giro dentado del hipocampo. Conclusión. Este caso confirma que el tratamiento con inhibidores de la colinesterasa puede ser eficaz en los síntomas conductuales y psicológicos de demencia frontotemporal (AU)

Introduction. Cholinesterase inhibitors are useful in the treatment of behavioural and psychological symptoms in Alzheimer's disease. Their effectiveness in frontotemporal dementia has not been proved, since such a claim has only been backed by the publication of one open-label trial in which the behavioural and psychological symptoms of the patients treated with rivastigmine over a 12-month period improved significantly with respect to those belonging to a group that were given a placebo. We report a case of frontotemporal dementia, Pick’s disease, which improved with rivastigmine treatment. Case report. A 61-year-old male who presented a progressive clinical picture of behavioural disorders and executive-cognitive impairment that had begun two years earlier. Magnetic resonance imaging of the head revealed severe frontotemporal atrophy. Neuropsychological Inventory (NPI). Overall score 36/144 (6/12: anxiety, disinhibition and aberrant motor behaviour, 4/12: agitation, irritability and apathy; 3/12: sleep and eating disorders. After three months’ treatment with rivastigmine, the overall score on the NPI was 10/144. This improvement remained stable over the months that followed. The patient died eight months later after developing liver cancer with metastasis. The microscopic study of the brain showed tau-positive neuronal inclusions, gliosis and neuronal loss. The inclusions were well-circumscribed Pick bodies, which were present in the frontal and temporal cortices and in the dentate gyrus of the hippocampus. Conclusions. This case confirms the idea that treatment with cholinesterase inhibitors can be effective in the behavioural and psychological symptoms of frontotemporal dementia (AU)

[Description of a novel test for the early detection of Alzheimer's disease]. / Descripción de un nuevo test para la detección precoz de la enfermedad de Alzheimer.

INTRODUCTION: Diagnosing Alzheimer's disease (AD) as soon as possible is critical to achieve an effective treatment of patients. AIM: To make up a test capable of differentiating those patients suffering from the early stages of AD compared to healthy people or patients with mild cognitive impairment (MCI). SUBJECTS AND METHODS: A test with 10 memory and language tasks was applied to 85 people: 34 in the early stage of probable AD, 26 with MCI, and 25 healthy people. The groups did not differ with regard to age, sex or studies. RESULTS: The analysis of variance showed significative differences between groups in all tasks as well as in the global score. Sensibility and specificity were calculated to MCI and AD. CONCLUSIONS: We designed a new test to detect AD patients on an early stage that is quick and easy to apply. The preliminary scale results in controls, MCI and early AD are shown.

Descripción de un nuevo test para la detección precoz de la enfermedad de Alzheimer / Description of a novel test for the early detection of Alzheimer´s disease

Introducción. El diagnóstico temprano de los pacientes con enfermedad de Alzheimer (EA) es fundamental para poder iniciar los tratamientos que puedan ralentizar la progresión de esta enfermedad. Objetivo. Elaborar una prueba que sea capaz de diferenciar a los pacientes con EA en la etapa inicial de los pacientes con deterioro cognitivo leve (DCL) y de las personas sanas. Sujetos y métodos. Se aplicó una prueba formada por 10 tareas de memoria y lenguaje a un grupo de 85 personas: 34 enfermos con probable EA en estadio leve, 26 con DCL y 25 individuos sanos. No había diferencias entre los grupos en cuanto a edad, sexo o años de estudio. Resultados. El análisis de la varianza reflejó diferencias significativas entre los tres grupos en todas las tareas, así como en la puntuación global de la prueba. Se calcularon la sensibilidad y la especificidad del test para diferenciar el DCL y la presencia o no de EA. Conclusiones. Se presenta una nueva prueba diseñada para detectar los pacientes con EA incipiente que es fácil y rápida de aplicar, y se muestran los resultados preliminares de baremación en controles, pacientes con DCL y pacientes con EA leve

Introduction. Diagnosing Alzheimer’s disease (AD) as soon as possible is critical to achieve an effective treatment of patients. Aim. To make up a test capable of differentiating those patients suffering from the early stages of AD compared to healthy people or patients with mild cognitive impairment (MCI). Subjects and methods. A test with 10 memory and language tasks was applied to 85 people: 34 in the early stage of probable AD, 26 with MCI, and 25 healthy people. The groups did not differ with regard to age, sex or studies. Results. The analysis of variance showed significative differences between groups in all tasks as well as in the global score. Sensibility and specificity were calculated to MCI and AD. Conclusions. We designed a new test to detect AD patients on an early stage that is quick and easy to apply. The preliminary scale results in controls, MCI and early AD are shown

[Muscular pseudohypertrophy and intraspinal neurinoma]. / Seudohipertrofia muscular y neurinoma intrarraquídeo.

We present a patient with protracted hypertrophy of the left calf, light triceps suralis weakness, and mild inconstant left foot pain. The clinical picture evolved slowly throughout more than eight years, and lately slight weakness in anterior right leg musculature appeared. The magnetic resonance examination revealed the existence of an intrarachideal extraspinal tumor adjacent to the conus medullaris that histologically was a neurinoma. Although the neurogenic pseudohypertrophy has diverse etiology, the present association is unpublished.