Tratamiento inicial de la enfermedad de Parkinson clínicamente establecida / Initial treatment of clinically established Parkinson's disease

Introducción. La enfermedad de Parkinson es una enfermedad cuyo diagnóstico continúa siendo clínico y aunque en la actualidad no existe una solución definitiva para la misma, se dispone de diversos fármacos para su tratamiento. Desarrollo. Se repasan los distintos fármacos que pueden tener su utilidad en la fase inicial de la enfermedad: L-dopa, inhibidores de la catecol-o-metil-transferasa, agonistas dopaminérgicos, inhibidores de la monoamino-oxidasa tipo B, amantadina, zonisamida y anticolinérgicos; así como terapias no farmacológicas. Asimismo se dan unas pautas sobre cómo emplear estos fármacos de una forma racional. Conclusiones. Actualmente sólo rasagilina ha cumplido los requisitos para poder ser considerada como tratamiento modificador de la enfermedad. L-dopa sigue siendo el mejor tratamiento sintomático, pero su utilización se asocia a aparición de complicaciones motoras. Los agonistas dopaminérgicos o los inhibidores de la monoamino-oxidasa tipo B pueden ser alternativas para retrasar el uso de la L-dopa. Zonisamida puede ser útil de inicio en pacientes jóvenes con cuadros de predominio tremórico. Los anticolinérgicos pueden ser empleados de forma excepcional para control de temblor parkinsoniano intenso no controlable con otros fármacos en pacientes jóvenes. El ejercicio físico, la hidroterapia y la terapia de la voz también pueden aportar beneficios (AU)

ntroduction. The diagnosis of Parkinson's disease continues to be clinically-based and although there is currently no definitive solution for it, a number of drugs are available to treat it. Development. Here we review the different drugs that can be useful in the early phases of the disease. These include: L-dopa, catechol-O-methyl transferase inhibitors, dopamine agonists, monoamine oxidase type B inhibitors, amantadine, zonisamide and anticholinergic agents, as well as non-pharmacological therapies. Likewise, guidelines are also provided on how to use these drugs in a rational way. Conclusions. At present, only rasagiline complies with the requirements to be considered a disease-modifying treatment. L-dopa continues to be the best symptomatic treatment, but its use is associated with the appearance of motor complications. Dopamine agonists or monoamine oxidase type B inhibitors can be employed as alternatives to delay the use of L-dopa. Zonisamide can be useful in the early phases in young patients with clinical pictures in which tremor predominates. The anticholinergics can be employed exceptionally to control intense parkinsonian tremor in young patients that cannot be controlled with other drugs. Physical exercise, hydrotherapy and voice therapy can also be beneficial (AU)

Demencia por cuerpos de Lewy / Dementia with Lewy bodies

La demencia por cuerpos de Lewy (DCL) es una enfermedad neurodegenerativa, que supone en torno al 10-25% de todas las demencias de la población general y es la segunda causa de demencia degenerativa, en el anciano, tras la enfermedad de Alzheimer. Se caracteriza clínicamente por deterioro cognitivo, con rasgos de demencia frontal, acompañado de: fluctuaciones, parkinsonismo y alucinaciones visuales. Su confirmación diagnóstica se realiza por anatomía patológica posmortem: presencia de abundantes cuerpos de Lewy (CL) en las neuronas de la corteza y en otras zonas cerebrales. Los CL son inclusiones intracitoplasmáticas eosinófilas, esféricas y están constituidos por más de 20 componentes proteicos. Se han definido, en el año 2005, unos criterios diagnósticos para facilitar su reconocimiento y poder etiquetar a los pacientes de posible o probable DCL. La causa de la DCL es desconocida; se supone que, al igual que en la enfermedad de Parkinson, influyen factores ambientales y genéticos. No hay ninguna prueba complementaria ni marcador biológico específico de esta enfermedad, pero la combinación de biomarcadores, los tests neuropsicológicos y las pruebas de neuroimagen son de especial ayuda en el diagnóstico. El tratamiento de la enfermedad es complejo; porque los fármacos que mejoran unos síntomas pueden empeorar otros. En cualquier caso solo hay posibilidad de tratamiento sintomático (AU)

Dementia with Lewy bodies (DLB) is a neurodegenerative disease, accounting for about 10-25% of all dementias in the general population and is the second most common type of degenerative dementia in elderly people after Alzheimer's disease. Clinically, it is characterized by cognitive impairment (with features of frontal dementia) accompanied by fluctuating cognition, parkinsonism and visual hallucinations. Diagnostic confirmation is made by pathologic autopsy: abundant presence of Lewy bodies (LB) in the neurons of the cortex and other areas in the brain. The LB are eosinophilic intracytoplasmic inclusions, spherical and consist of more than 20 protein components. Diagnostic criteria to facilitate recognition of patients with possible or probable DLB were defined in 2005. The etiology of DLB is unknown, although is assumed that, environmental factors and genetic influence, like in Parkinson's disease. There are not additional evidence or specific biological markers of this disease, but neuropsychological and neuroimaging tests are helpful in diagnosis. The treatment of the disease is difficult, there is no cure, only symptomatic. Some drugs may improve some symptoms but also can worse others (AU)

Análisis de costes directos, indirectos e intangibles de la epilepsia / Analysis of direct, indirect, and intangible costs of epilepsy

Introducción: Se ha realizado una estimación económica de los costes de epilepsia en adultos.Métodos: Estudio observacional prospectivo realizado durante 6 meses, en pacientes epilépticos mayores de 14 años. Se excluyeron los pacientes con enfermedades concomitantes que pudieran influir en la evolución de la epilepsia. Los costes directos incluyeron: tratamiento administrado, número de consultas en Neurología, Atención Primaria, y Urgencias, número de días de ingreso hospitalario, número y tipo de pruebas diagnósticas, uso de los medios de transporte al hospital y desde el hospital, y los apoyos psicopedagógicos y sociales por epilepsia. Los costes indirectos se analizaron en función de la pérdida de productividad laboral de los pacientes, con consideración de los familiares cuando los pacientes necesitaban supervisión a causa de la epilepsia. Los costes totales se derivaron de la suma de los costes directos e indirectos. Los costes intangibles se evaluaron según el cuestionario QOLIE-10. Resultados:La media de los costes directos por paciente fue de 1.055,2 €. El gasto económico medio en los costes indirectos ascendió a 1.528,8 € por paciente. El total de los costes asociados a la epilepsia supuso una media de 2.584 € por cada paciente, derivados sobre todo de la pérdida de productividad laboral (p<0,05). En los costes intangibles, según la escala QOLIE-10, la media obtenida fue de 77,8. Conclusiones: El mayor porcentaje de los costes asociados a la epilepsia corresponde a la pérdida de productividad laboral de los pacientes. Los costes del sufrimiento psicológico y social en epilepsia provocan deterioro de la calidad de vida (AU)

Introduction: A financial estimate has been made of the costs of epilepsy in adults. Methods: A prospective, observational study, over a period of 6 months, on epileptic patients over 14 years-old. Patients with concomitant diseases that could influence the outcome of the epilepsy were excluded. The direct costs included: treatment received, number of visits to neurology, primary care, and emergencies, number of days admitted to hospital, number and type of diagnostic tests, use of transport to and from hospital, and psychopedagogic and social support due to the epilepsy. The indirect costs were analysed according to, loss of work productivity of the patients, taking into account families where the patient needed supervision due to epilepsy. The total costs were derived from the sum of the direct and indirect costs. The intangible costs were calculated according to QOLIE-10 questionnaire.Results: The mean direct cost per patient was 1,055.2 €. The mean indirect financial costs came to 1,528.8 € per patient. The total cost associated to epilepsy was a mean of 2,584 € for each patient, mainly arising from loss of work days (p<.05). For intangible costs according to the QOLIE-10 scale a mean of 77.8 was obtained.Conclusions: The greatest percentage of costs associated to epilepsy is due to the work productivity loss by the patients. The costs of psychological and social suffering in epilepsy lead to a deterioration in the quality of life (AU)

Analysis of direct, indirect, and intangible costs of epilepsy.

INTRODUCTION: A financial estimate has been made of the costs of epilepsy in adults. METHODS: A prospective, observational study, over a period of 6 months, on epileptic patients over 14 years-old. Patients with concomitant diseases that could influence the outcome of the epilepsy were excluded. The direct costs included: treatment received, number of visits to neurology, primary care, and emergencies, number of days admitted to hospital, number and type of diagnostic tests, use of transport to and from hospital, and psychopedagogic and social support due to the epilepsy. The indirect costs were analysed according to, loss of work productivity of the patients, taking into account families where the patient needed supervision due to epilepsy. The total costs were derived from the sum of the direct and indirect costs. The intangible costs were calculated according to QOLIE-10 questionnaire. RESULTS: The mean direct cost per patient was 1,055.2 €. The mean indirect financial costs came to 1,528.8 € per patient. The total cost associated to epilepsy was a mean of 2,584 € for each patient, mainly arising from loss of work days (p<.05). For intangible costs according to the QOLIE-10 scale a mean of 77.8 was obtained. CONCLUSIONS: The greatest percentage of costs associated to epilepsy is due to the work productivity loss by the patients. The costs of psychological and social suffering in epilepsy lead to a deterioration in the quality of life.

[Dystonia: their epidemiology, aetiology, diagnosis and treatment]. / Distonías: epidemiologia, etiología, diagnóstico y tratamiento.

INTRODUCTION: Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. AIMS: To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. DEVELOPMENT: Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can be classified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among the different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. CONCLUSIONS: Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias.

Distonías: epidemiología, etiología, diagnóstico y tratamiento / Destonia: Thir epidemiology, aetiology, diagnosis and treatment

Resumen. Introducción. La distonía es el segundo trastorno del movimiento más frecuente después de los parkinsonismos. No se conocen con exactitud las cifras de incidencia/prevalencia de las distintas formas de distonía, ya que las cifras varían considerablemente en función de la fuente, método de estudio y origen étnico de la población estudiada. Objetivo. Describir y resumir los conocimientos actuales sobre la epidemiología, etiología, diagnóstico y tratamiento de las distonías. Desarrollo. La distonía es un trastorno del movimiento caracterizado por contracciones musculares sostenidas que causan movimientos de torsión repetidos y posturas anómalas. Las distonías pueden clasificarse según su distribución, etiología, curso clínico y edad de inicio. Una correcta clasificación resulta muy útil de cara a evaluar las pruebas complementarías necesarias, el pronóstico y el tratamiento del proceso. El diagnóstico de distonía es fundamentalmente clínico y se confirma con electromiografía. Tanto los diversos estudios de laboratorio como de neuroimagen sirven fundamentalmente para ayudar en la clasificación etiológica y para descartar causas secundarias de distonía. Dentro de los diversos tratamientos existentes hoy en día (farmacoterapia sistémica, intratecal, infiltrativa, quirúrgica), cabe destacar el papel de la toxina botulínica como tratamiento de elección en la mayor parte de las distonías focales. Conclusiones. Es necesaria la realización de estudios epidemiológicos exhaustivos que permitan conocer con mayor exactitud la epidemiología de las distonías. A pesar del gran avance en los últimos años en cuanto a genética y neuroimagen, el diagnóstico de la distonía sigue siendo fundamentalmente clínico. La toxina botulínica es el tratamiento de elección en la mayor parte de las distonías focales (AU)

Summary. Introduction. Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. Aims. To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. Development. Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can beclassified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among he different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. Conclusions. Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias (AU)

[Time evolution of TNF-alpha, VCAM-1, IL-4, IL-10, neopterin and CD-30 in patients treated with interferon]. / Evolución temporal de TNF-a, VCAM-1, IL-4, IL-10, neopterina y CD-30 en pacientes tratados con interferón.

INTRODUCTION: Interferon (IFN) diminishes the outbreaks of multiple sclerosis (MS) and slows down its progression. Follow-up of patients is performed using clinical and resonance imaging parameters, and no biological markers are available that allow us to determine its efficiency. AIMS: 1. To discover the effects of IFN on the serum levels of TNF-alpha, IL-4, IL-10, VCAM-1, neopterin and CD-30 in patients with MS; 2. To determine how these modifications evolve over time; 3. To find out the clinical value of its determination in isolation. PATIENTS AND METHODS: We studied 19 patients with MS who were clinically stable and undergoing IFN therapy. Samples were obtained every 3 months over a 2.5 year period and always immediately before injecting the drug. The ELISA method was used to determine interleukins. RESULTS: Serum levels of neopterin, CD-30 and VCAM-1 were not modified, TNF-alpha levels oscillated regardless of the clinical status of the patient and IL-4 and IL-10 had a significant serum peak at 9-12 months after beginning treatment. CONCLUSIONS: The existence of a significant IL-4 and IL-10 peak between 6 and 12 months of therapy indicates that IFN reaches its possible immunomodulatory effect after several months and, therefore, a poor initial clinical response must not be a reason for discontinuing medication. The specific determination of the serum levels of IL is not useful in following up patients treated with IFN.

[Superficial siderosis of the central nervous system: report of two new cases]. / Siderosis superficial del sistema nervioso central: descripción de dos nuevos casos.

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). CASE REPORTS: We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. CONCLUSION: Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS.

Siderosis superficial del sistema nervioso central: descripción de dos nuevos casos / Superficial siderosis of the central nervous system: report of two new cases

Introducción. La siderosis superficial del sistema nervioso central (SNC) es una enfermedad rara, caracterizada por sordera, ataxia y disfunción piramidal. Se debe al depósito de hemosiderina en las membranas subpiales del cerebro, la médula espinal y los nervios craneales. La mayoría de los casos son secundarios a un sangrado crónico o recurrente en el espacio subaracnoideo. Se requiere la realización de una resonancia magnética (RM) para alcanzar el diagnóstico. Casos clínicos. Presentamos dos pacientes con un cuadro crónico de ataxia cerebelosa y pérdida auditiva progresivas. En ambos casos, la RM mostraba señales hipointensas en T2 en el cerebro, el cerebelo y la médula espinal, diagnósticas de siderosis superficial del SNC. En uno de los pacientes se demostró xantocromía en el análisis del líquido cefalorraquídeo. Los potenciales evocados auditivos revelaron, en ambos casos, una pérdida auditiva retrococlear. Se realizaron estudios vasculares exhaustivos sin que se pudiera demostrar la presencia de fuentes de sangrado. Uno de los pacientes, que recibía tratamiento con anticoagulantes orales, se benefició de la reducción del INR. Conclusión. Debido a su rareza, la sospecha clínica es esencial para el diagnóstico de esta enfermedad (AU)

Introduction. Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). Case reports. We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. Conclusion. Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS (AU)

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