Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the "no evidence of disease activity" parameter.

INTRODUCTION: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions. OBJECTIVE: To determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the "no evidence of disease activity" (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters. PATIENTS AND METHODS: We performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions. RESULTS: The study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti-JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy. CONCLUSIONS: Natalizumab is highly effective as measured by the NEDA long-term remission parameter.

Evolución de una serie de pacientes con esclerosis múltiple remitente-recurrente tratados con natalizumab durante 7 años mediante el parámetro no evidencia enfermedad activa / Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the “no evidence of disease activity” parameter

Introducción: La efectividad y seguridad de natalizumab en pacientes con esclerosis múltiple remitente recurrente (EMRR) se demostró en ensayos clínicos. Sin embargo, por las limitaciones de estos es importante saber cómo se comporta en condiciones de práctica clínica a largo plazo.ObjetivoConocer la eficacia a largo plazo de natalizumab en pacientes con EMRR mediante la evaluación anual del no evidence of disease activity (NEDA), que incluye número de brotes, discapacidad medida con EDSS y parámetros de RM cerebral.Pacientes y métodosEstudio retrospectivo y multicéntrico (n = 3) de pacientes con EMRR tratados con una o más dosis de natalizumab. Se evaluó el estado NEDA cada año y la seguridad a partir del porcentaje de pacientes que discontinuaron y que presentaron efectos adversos.ResultadosIncluimos 89 pacientes, la mayoría recibieron tratamiento durante 2 a 4 años, con una duración del seguimiento de hasta 7 años. Natalizumab reduce significativamente la progresión radiológica y clínica de la enfermedad, así como la tasa anual de brotes, demostrándose su eficacia con el parámetro NEDA, 75,28% al primer año y 66,67% al séptimo año. Veinticinco pacientes (28,1%) han abandonado el estudio en una mediana de tiempo de 4 años, 14 pacientes (56%) por aparición de anticuerpos contra el virus JC, como causa única o asociada a otro motivo, 4 abandonos (16%) fueron por ineficacia, un paciente falleció a causa de LMP.ConclusionesNatalizumab presenta una alta eficacia medida mediante el parámetro de remisión NEDA a largo plazo. (AU)

Introduction: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions.ObjectiveTo determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the “no evidence of disease activity” (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters.Patients and methodsWe performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions.ResultsThe study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti–JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy.ConclusionsNatalizumab is highly effective as measured by the NEDA long-term remission parameter. (AU)

Ataxia y nistagmo de origen infrecuente / Ataxia and nystagmus of unusual origin

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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.

Respuesta a: Leucoencefalopatía con sustancia blanca evanescente de inicio en edad adulta / Hospital Povisa

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Leucoencefalopatía con sustancia blanca evanescente: caso clínico de inicio en adulto / No disponible

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Eficacia del topiramato en un paciente con cefalea en racimos crónica / The effectiveness of topiramate in a patient with chronic cluster headache

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Utilidad del levetiracetam en solución oral en el estado epiléptico / The value of an oral solution of levetiracetam in epileptic status

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[Leber's optic neuropathy: a case report]. / Neuropatía óptica de Leber: caso clínico.

INTRODUCTION: Leber's optic neuropathy is a hereditary disease that mainly affects young males and is produced by specific mutations of the mitochondrial DNA, which affect the complex I of the mitochondrial respiratory chain. CASE REPORT: An 18-year-old male who presented with a 3-week history of progressive loss of sight in the right eye. Magnetic resonance imaging of the brain revealed numerous hyperintense lesions in the periventricular and subcortical white matter, and the visual evoked potentials showed bilateral optic neuropathy that was mild on the left side and severe on the right side. A spinal tap was performed and oligoclonal bands were detected in the cerebrospinal fluid. In the weeks that followed vision continued to get worse on both sides and the patient had hyalinised vessels in the papilla, with lower amplitude responses bilaterally in the electroretinogram. A genetic study was conducted that revealed a primary mutation 11778 in gene MTND4 and secondary mutation 15257 in gene MTCYB, which were compatible with a diagnosis of Leber's optic neuropathy. CONCLUSIONS: The absence of inflammation of the optic disc, which could lead to the suspicion of a retrobulbar neuritis, must act as a warning to the physician that he or she is possibly before a case of Leber's optic neuropathy, especially when the loss of vision is still progressing, when there is early bilateral involvement or if there is a family history of optic neuritis or multiple sclerosis.

Neuropatía óptica de Leber: caso clínico / Leber’s optic neuropathy: a case report

Introducción. La neuropatía óptica de Leber es una enfermedad hereditaria que afecta sobre todo a varones jóvenes y se produce por mutaciones puntuales del ADN mitocondrial, que afectan al complejo I de la cadena respiratoria mitocondrial. Caso clínico. Varón de 18 años de edad, que presenta pérdida progresiva de la visión por el ojo derecho de 3 semanas de evolución. La resonancia magnética cerebral evidencia múltiples lesiones hiperintensas en la sustancia blanca periventricular y subcortical, y los potenciales evocados visuales manifiestan neuropatía óptica bilateral leve en lado izquierdo y grave en el lado derecho. Se realiza punción lumbar y se detectan bandas oligoclonales en el líquido cefalorraquídeo. En las semanas siguientes continúa el empeoramiento de la visión bilateral, muestra vasos hialinizados en la papila, con respuestas de amplitud disminuida en el electrorretinograma bilateralmente. Se realiza estudio genético y se detecta la mutación primaria11778 en el gen MTND4, y la mutación secundaria 15257en el gen MTCYB, compatibles con el diagnóstico de neuropatíaóptica de Leber. Conclusiones. La ausencia de inflamación del discoóptico, que podría conducir a la sospecha de una neuritis retrobulbar, debe alertar al clínico acerca de la posibilidad de que se trate de una neuropatía óptica de Leber, sobre todo cuando la pérdida de la visión progresa todavía, cuando existe afectación bilateral precoz, o si existen antecedentes familiares de neuritis óptica oesclerosis múltiple (AU)

Introduction. Leber's optic neuropathy is a hereditary disease that mainly affects young males and is produced by specific mutations of the mitochondrial DNA, which affect the complex I of the mitochondrial respiratory chain. Case report. An 18-year-old male who presented with a 3-week history of progressive loss of sight in the right eye. Magnetic resonance imaging of the brain revealed numerous hyperintense lesions in the periventricular and subcortical white matter, and the visual evoked potentials showed bilateral optic neuropathy that was mild on the left side and severe on the right side. A spinal tap was performed and oligoclonal bands were detected in the cerebrospinal fluid. In the weeks that followed vision continued to get worse on both sides and the patient had hyalinised vessels in the papilla, with lower amplitude responses bilaterally in the electroretinogram. A genetic study was conducted that revealed a primary mutation 11778 in gene MTND4and secondary mutation 15257 in gene MTCYB, which were compatible with a diagnosis of Leber's optic neuropathy. Conclusions. The absence of inflammation of the optic disc, which could lead to the suspicion of a retrobulbar neuritis, must act as a warning to the physician that he or she is possibly before a case of Leber's optic neuropathy, especially when the loss of vision is still progressing, when there is early bilateral involvement or if there is a family history of optic neuritis or multiple sclerosis (AU)

Parkisonismo de etiología tumoral / Toumoral Parkinsonims

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[Does brain blood flow HMPAO SPECT withhold the vascular etiopathogenic theory of transient global amnesia?]. / Apoya el SPECT de perfusión cerebral la teoría etiopatogénica vascular de la amnesia global transitoria?

INTRODUCTION: Transient global amnesia (TGA) is a neurological disorder that consists in a sudden loss of anterograd memory and temporospatial disorientation during less than 24 hours. Several precipitating factors have been reported. Conventional neuroimaging scans usually are negative. Different etiopathogenic theories have been postulated but the vascular etiology is the most commonly accepted. CASE REPORTS: Three patients with a typical presentation of TGA are studied. In all of them two brain blood flow HMPAO SPECT were performed, within the first 48 hours from the onset and three months after as an evolutive control. The first patient showed a left temporal perfusion defect and temporoparietal hypoperfusion. The second showed frontotemporal hypoperfusion, temporal mesial defect and hypoperfusion in basal ganglia, all in the left side. The third patient showed thalamic hyperperfusion and cerebellum hypoperfusion, both in the left. In all of them, control SPECT normalized. CONCLUSION: Three etiopatogenic theories about TGA have been reported: epilepsy, migraine and blood flow impairment. In TGA neuroanatomic image and neurophysiologic studies usually do not show significative alterations. Conversely, functional studies as brain blood flow HMPAO SPECT, do show changes being the most common bilateral temporobasal hypoperfusion, although this is not the only pattern described. Causes of this variable behaviour remain unclear but can be related to different clinic expressions and, over all, to time of evolution from onset. The three cases in this study show three different perfusion patterns reported in TGA and all of them withhold the vascular etiopathogenic theory.

¿Apoya el SPECT de perfusión cerebral la teoría etiopatogénica vascular de la amnesia global transitoria? / Does brain blood flow hmpao spect withhold the vascular etiopathogenic theory of transient global amnesia?

Introducción. La amnesia global transitoria (AGT) es un desorden neurológico consistente en una pérdida brusca de memoria anterógrada predominantemente y una desorientación temporoespacial cuya duración es inferior a 24 horas. Se han definido múltiples factores desencadenantes. Las pruebas de imagen convencionales suelen ser negativas. Existen varias teorías etiopatogénicas, de las que la más aceptada es la de origen vascular. Casos clínicos. Se presentan tres pacientes con cuadro típico de AGT a los que se realizó un SPECT de perfusión con HMPAO dentro de las primeras 48 del inicio del episodio y un control evolutivo a los tres meses. El primero mostró un defecto de perfusión temporal izquierdo e hipoperfusión temporoparietal ipsilateral. El segundo, una hipoperfusión frontotemporal, un defecto temporal mesial e hipoperfusión de los ganglios basales, todos ellos izquierdos. El tercer paciente mostró una hiperperfusión talámica e hipoperfusión cerebelosa, ambos izquierdos. En todos ellos, el SPECT de control mostró la normalización de los hallazgos. Conclusión. Las tres teorías etiopatogénicas de la AGT postulan un origen epileptógeno, migrañoso o vascular. En la AGT, las pruebas de imagen neuroanatómica y neurofisiológicas no suelen aportar datos significativos, pero las pruebas funcionales del SPECT de perfusión sí suelen mostrar alteraciones, la más frecuente de las cuales es la hipoperfusión temporobasal bilateral, aunque no es el único patrón descrito. Las causas del patrón variable de hipoperfusión no están claras, aunque pueden estar relacionadas con cambios en la expresividad clínica y, sobre todo, en el tiempo de evolución desde el inicio de la clínica. Los tres casos mostrados ilustran tres patrones de perfusión diferentes descritos en la AGT, y todos ellos apoyan la teoría etiopatogénica vascular (AU)

Introduction. Transient global amnesia (TGA) is a neurological disorder that consists in a sudden loss of anterograd memory and temporoespatial disorientation during less than 24 hours. Several precipitating factors have been reported. Conventional neuroimaging scans usually are negative. Different etiopathogenic theories have been postulated but the vascular etiology is the most commonly accepted. Case reports. Three patients with a typical presentation of TGA are studied. In all of them two brain blood flow HMPAO SPECT were performed, within the first 48 hours from the onset and three months after as an evolutive control. The first patient showed a left temporal perfusion defect and temporoparietal hypoperfusion. The second showed frontotemporal hypoperfusion, temporal mesial defect and hypoperfusion in basal ganglia, all in the left side. The third patient showed thalamic hyperperfusion and cerebellum hypoperfusion, both in the left. In all of them, control SPECT normalized. Conclusion. Three etiopatogenic theories about TGA have been reported: epilepsy, migraine and blood flow impairment. In TGA neuroanatomic image and neurophisiologic studies usually do not show significative alterations. Conversely, functional studies as brain blood flow HMPAO SPECT, do show changes being the most common bilateral temporobasal hypoperfusion, although this is not the only pattern described. Causes of this variable behaviour remain unclear but can be related to different clinic expressions and, over all, to time of evolution from onset. The three cases in this study show three different perfusion patterns reported in TGA and all of them withhold the vascular etiopathogenic theory (AU)

[The economic impact of epilepsy]. / Impacto económico de la epilepsia.

AIMS: In this study we review the economic impact involved in suffering from this disease in an attempt to determine how it affects both the individual and society, and the potential benefits deriving from its prevention and treatment. DEVELOPMENT: The World Health Organisation and the World Bank have pointed out that 90% of the costs generated by epilepsy are produced in developing countries. Yet in most developed countries the economic impact of the disease remains partially hidden for patients by the existence of publicly funded health service. As regards spending on pharmaceutical products in Spain, the subgroup made up of the antiepileptic drugs accounted for 1.36% of the total spending throughout the year 2001. Nevertheless, the main economic consequence for most patients is the limitation they suffer in their occupational activities, which is inversely proportional to the degree of control over their seizures and considerably higher than in the general population. Moreover, in epilepsy we must not forget the costs linked to its numerous psychological and social consequences. CONCLUSIONS: As happens in other areas of health care, the way epilepsy is attended depends to a large extent on economic factors. Further studies are therefore needed to provide us with a better understanding of the role played by economics in the field of health care.

Impacto económico de la epilepsia / The economic impact of epilepsy

Objetivo. Se realiza una revisión del impacto económico que supone padecer esta enfermedad, para tratar de conocer cómo afecta al individuo y a la sociedad, así como los beneficios potenciales de su prevención y tratamiento. Desarrollo. La Organización Mundial de la Salud y el Banco Mundial apreciaron que el 90 por ciento de los costes que genera la epilepsia se producen en los países en vías de desarrollo. En cambio, en la mayoría de los países desarrollados, el impacto económico de la enfermedad queda parcialmente enmascarado para los pacientes por la existencia de un sistema de salud financiado públicamente. En cuanto al gasto en productos farmacéuticos en España, el subgrupo formado por los fármacos antiepilépticos supuso un 1,36 por ciento del gasto total durante el año 2001. No obstante, la principal consecuencia económica para la mayoría de los pacientes es la limitación que sufren en su actividad laboral, que es inversamente proporcional al grado de control de las crisis y sensiblemente mayor que en la población general. Además, en la epilepsia, no podemos olvidar los costes relacionados con las numerosas consecuencias psicológicas y sociales. Conclusiones. Al igual que ocurre en otras áreas de la salud, la forma de atender la epilepsia depende en gran medida de factores económicos. Por ello, son necesarios más estudios que nos ayuden a comprender mejor el papel de la economía en el ámbito sanitario (AU)

Aims. In this study we review the economic impact involved in suffering from this disease in an attempt to determine how it affects both the individual and society, and the potential benefits deriving from its prevention and treatment. Development. The World Health Organisation and the World Bank have pointed out that 90% of the costs generated by epilepsy are produced in developing countries. Yet in most developed countries the economic impact of the disease remains partially hidden for patients by the existence of publicly funded health service. As regards spending on pharmaceutical products in Spain, the subgroup made up of the antiepileptic drugs accounted for 1.36% of the total spending throughout the year 2001. Nevertheless, the main economic consequence for most patients is the limitation they suffer in their occupational activities, which is inversely proportional to the degree of control over their seizures and considerably higher than in the general population. Moreover, in epilepsy we must not forget the costs linked to its numerous psychological and social consequences. Conclusions. As happens in other areas of health care, the way epilepsy is attended depends to a large extent on economic factors. Further studies are therefore needed to provide us with a better understanding of the role played by economics in the field of health care (AU)