[A descriptive study of neurocysticercosis in a tertiary care hospital]. / Estudio descriptivo de neurocisticercosis en un hospital terciario.

INTRODUCTION: Neurocysticercosis is the most frequent parasitic disease affecting the central nervous system. It is a disease that is endemic to certain countries in South America. The phenomenon of immigration, however, has increased its prevalence in developed regions due to the arrival of immigrants from endemic areas. AIM: To present the clinical and demographic characteristics of the cases of neurocysticercosis attended in a tertiary care hospital in the city of Murcia. PATIENTS AND METHODS: We conducted a descriptive, retrospective study by reviewing the medical records of patients with a hospital diagnosis of neurocysticercosis over a nine-year period (1997-2005). Demographic and clinical data on these patients were collected. RESULTS: Twenty-three patients (three under 12 years of age) were found. Mean age: 29.6 years. Countries of origin: Ecuador and Bolivia. The most frequently observed clinical manifestations were: epileptic seizures (73.9%), headache (39.1%) and neurological focus (26.1%). Albendazole was employed in 91.3% of cases and corticoids in 73.9%. The most frequently used drug in patients who received antiepileptic therapy was phenytoin. Four patients required surgical treatment. During the follow-up period, 52.8% of the patients were asymptomatic. CONCLUSIONS: Neurocysticercosis is a disease that is becoming increasingly more prevalent in Spain and we should suspect its presence in patients from endemic areas who visit because of clinical symptoms involving the central nervous system.

Estudio descriptivo de neurocisticercosis en un hospital terciario / A descriptive study of neurocysticercosis in a tertiary care hospital

La neurocisticercosis es la enfermedad parasitaria más frecuente del sistema nervioso central. Setrata de una enfermedad endémica de ciertos países de Sudamérica. Sin embargo, debido al fenómeno de la inmigración, ha aumentado su prevalencia en zonas desarrolladas debido a la llegada de inmigrantes procedentes de áreas endémicas. Objetivo.Presentar las características clínicas y demográficas de los casos de neurocisticercosis atendidos en un hospital terciario de la ciudad de Murcia. Pacientes y métodos. Estudio descriptivo, retrospectivo mediante revisión de historias clínicas de pacientes con diagnóstico hospitalario de neurocisticercosis en un período de nueve años (1997-2005). Se recogen los datosdemográficos y clínicos de estos pacientes. Resultados. Se estudiaron 23 pacientes (tres menores de 12 años). Edad media: 29,6 años. Países de origen: Ecuador y Bolivia. Las manifestaciones clínicas más frecuentes fueron: crisis epilépticas (73,9%),cefalea (39,1%) y focalidad neurológica (26,1%). Se utilizó albendazol en el 91,3% de los casos y corticoides en el 73,9%. De los pacientes que recibieron tratamiento antiepiléptico, el fármaco más utilizado fue la fenitoína. cuatro pacientes precisarontratamiento quirúrgico. En el período de seguimiento estaban asintomáticos el 52,8% de los pacientes. Conclusiones. La neurocisticercosis es una enfermedad cada vez más prevalente en España y que debemos sospechar en pacientes procedentes de zonasendémicas que consulten por clínica de afectación del sistema nervioso central

Neurocysticercosis is the most frequent parasitic disease affecting the central nervous system. It is adisease that is endemic to certain countries in South America. The phenomenon of immigration, however, has increased its prevalence in developed regions due to the arrival of immigrants from endemic areas. Aim. To present the clinical and demographic characteristics of the cases of neurocysticercosis attended in a tertiary care hospital in the city of Murcia.Patients and methods. We conducted a descriptive, retrospective study by reviewing the medical records of patients with a hospital diagnosis of neurocysticercosis over a nine-year period (1997-2005). Demographic and clinical data on these patients were collected. Results. Twenty-three patients (three under 12 years of age) were found. Mean age: 29.6 years. Countries of origin: Ecuador and Bolivia. The most frequently observed clinical manifestations were: epileptic seizures(73.9%), headache (39.1%) and neurological focus (26.1%). Albendazole was employed in 91.3% of cases and corticoids in 73.9%. The most frequently used drug in patients who received antiepileptic therapy was phenytoin. Four patients requiredsurgical treatment. During the follow-up period, 52.8% of the patients were asymptomatic. Conclusions. Neurocysticercosis is a disease that is becoming increasingly more prevalent in Spain and we should suspect its presence in patients from endemicareas who visit because of clinical symptoms involving the central nervous system

Un caso de meningitis por virus Toscana en Murcia / A case of meningitis due to Toscana virus in Murcia

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[Gene therapy for Parkinson's disease]. / Terapia génica en la enfermedad de Parkinson.

Parkinson's disease is a chronic and progressive disorder whose treatment does not prevent middle term appearance of invalidating motor and psychic complications. Gene therapy techniques which are increasingly applied in the field of neurodegenerative diseases are added to the possibility of treatment of this disease. Among the existing modalities, the in vivo strategies that use potent viral vectors are those which have obtained the best results in the different existing models of the disease. This article aims to review the information regarding the use of these latter techniques, the therapeutic trials that have been conducted and the advantages and disadvantages that the use of the different vectors have.

Terapia génica en la enfermedad de Parkinson / Gene therapy for Parkinson's disease

La enfermedad de Parkinson es un trastorno crónico y progresivo cuyo tratamiento no impide a medio plazo la aparición de complicaciones motoras y psíquicas invalidantes. Las técnicas de terapia génica de aplicación cada vez mayor en el campo de las enfermedades neurodegenerativas se suman a las posibilidades de tratamiento de esta patología. Entre las modalidades existentes, las estrategias in vivo que emplean potentes vectores virales son las que mejores resultados han obtenido en los distintos modelos existentes de la enfermedad. Este artículo pretende revisar la información referente al empleo de estas últimas técnicas, los ensayos terapéuticos que se han llevado a cabo y las ventajas e inconvenientes que tiene la utilización de los diferentes vectores

Parkinson's disease is a chronic and progressive disorder whose treatment does not prevent middle term appearance of invalidating motor and psychic complications. Gene therapy techniques which are increasingly applied in the field of neurodegenerative diseases are added to the possibility of treatment of this disease. Among the existing modalities, the in vivo strategies that use potent viral vectors are those which have obtained the best results in the different existing models of the disease. This article aims to review the information regarding the use of these latter techniques, the therapeutic trials that have been conducted and the advantages and disadvantages that the use of the different vectors have

Changes in the neuronal activity in the pedunculopontine nucleus in chronic MPTP-treated primates: an in situ hybridization study of cytochrome oxidase subunit I, choline acetyl transferase and substance P mRNA expression.

The pedunculopontine nucleus is a mesencephalic nucleus that has widespread and reciprocal connections with the basal ganglia. It has been implicated in the physiopathology of akinesia, rigidity, gait failure and sleep disorders associated with Parkinson's disease. In this study, in situ hybridization was used to examine the changes in neuronal metabolic activity (measuring cytochrome oxidase subunit I) and in the level of acetylcholine and Substance P synthesis in the pedunculopontine nucleus of monkeys chronically treated with MPTP. Significant reductions were observed in cytochrome oxidase subunit I (p = 0.001), choline acetyl transferase (p = 0.003) and substance P (p = 0.006) mRNA expression in parkinsonian animals compared with controls, indicating that pedunculopontine cholinergic neurons activity decreases with parkinsonism.

Changes in vascularization in substantia nigra pars compacta of monkeys rendered parkinsonian.

The degeneration of nigral dopaminergic neurons in Parkinson's disease is believed to be associated with a glial reaction and inflammatory changes. In turn, local factors may induce changes in vascularization and contribute to neuronal vulnerability. Among these factors, Vascular Endothelial Growth Factor (VEGF) is released in adults under pathological conditions and is thought to induce angiogenesis. In order to determine whether changes in brain vasculature are observed in the affected brain regions in parkinsonism, we quantitatively analysed the VEGF-expressing cells and blood vessels in the substantia nigra of monkeys rendered parkinsonian by MPTP injection and compared the results with those obtained in control monkeys. Using stereological methods, we observed an increase in the number of VEGF-expressing neurons and an increase of the number of blood vessels and their volume occupying the substantia nigra pars compacta of monkeys rendered parkinsonian by chronic MPTP intoxication. These changes in vascularization may therefore modify the neuronal availability of blood nutrients, blood cells or toxic substances and neuronal susceptibility to parkinsonism.

Novel HMBS founder mutation and significant intronic polymorphism in Spanish patients with acute intermittent porphyria.

Acute intermittent porphyria (AIP) is an autosomal dominant disorder of heme biosynthesis, caused by a partial deficiency of hydroxymethylbilane synthase (HMBS). Knowledge of the nature of the HMBS mutations causing AIP in Spanish families is very limited. Here we report a novel 669_698del of the HMBS gene in twenty-two individuals from five independent Spanish AIP families, settled in Murcia (southeastern region of Spain). All mutation carriers shared a common disease associated haplotype indicating an ancestral founder effect. Identification of the 669_698del founder mutation allowed rapid and simple molecular diagnosis of AIP in families from this region in Spain. In addition, 771 + 58C>T in intron 12 on the non-669_698del allele was identified in six AIP patients, which promoted homozygous AIP misdiagnosis.

Encefalopatía posterior reversible en una niña de 12 años con síndrome nefrótico y tratamiento con metilprednisolona / Posterior reversible encephalopathy in a 12-year-old female with nephrotic syndrome and methylprednisolone therapy

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Estado actual de la porfiria aguda intermitente en la región de Murcia / The current state of acute intermittent porphyria in the region of Murcia

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Measurement of motor disability in MPTP-treated macaques using a telemetry system for estimating circadian motor activity.

The parkinsonian symptoms of primates after MPTP exposure can be measured by several visual methods (classical motor scores). However, these methods have a subjective bias, especially as regards the evaluation of the motor activity. Computerized monitoring systems represent an unbiased method for measuring the motor disability of monkeys after MPTP administration. In this work the motor activity of monkeys before and after MPTP administration is measured and compared with the activity of a control intact group by means of a telemetry system. A pronounced decrease in motor activity was observed after MPTP administration. These results suggest the monitoring method used is suited for characterizing the motor incapacity and possible improvements following treatments to test different therapies to control Parkinson's disease in MPTP models involving primates.

[Plasmapheresis: its use in multiple sclerosis and other demyelinating processes of the central nervous system. An observation study]. / Plasmaféresis: su utilidad en esclerosis múltiple y otros procesos desmielinizantes del sistema nervioso central. Estudio observacional.

INTRODUCTION: We present a retrospective observation study aimed at analyzing the value of plasmapheresis in the management of patients with multiple sclerosis (MS) and other acute demyelinating processes affecting the central nervous system (CNS) who show severe exacerbations that do not respond well to conventional therapy with corticoids. PATIENTS AND METHODS: A total of 11 patients were included in the study: nine with MS, one disseminated acute encephalomyelitis and one case of transverse myelitis. All of them presented an acute or subacute neurological deficit, which prevented them from carrying out their day to day activities, with or without repercussions on the EDSS, and with the risk of suffering a severe residual disability after not responding to intravenous methylprednisolone pulses. Each patient was submitted to three exchanges per week, for 2 weeks, with association of orally administered prednisone and they were then evaluated after the last session and at one, six and twelve months. RESULTS: Following plasmapheresis all the patients experienced a significant drop in disability and seven of them (77.7% of the total number with MS) even improved during the first month with respect to their basal situation ( an extension of the Lazarus effect ). After a year s follow up, 100% of the patients still maintained the basal situation that was recovered from before exacerbation, and only two relapses were recorded. The patients with MS presented a transient exacerbation after the second exchange. New therapy with immunosuppressants, immunomodulators or both was associated in eight cases. CONCLUSIONS: We consider plasmapheresis to be a safe, effective therapeutic procedure in the management of patients with MS and other demyelinating processes affecting the CNS. Its use should be considered as first choice in severe relapses and in swiftly progressing forms that do not respond to intravenous methylprednisolone.

Plasmaféresis: su utilidad en la esclerosis múltiple y otros procesos desmielinizantes del sistema nervioso central. Estudio observacional / Plasmaféresis: su utilidad en la esclerosis múltiple y otros procesos desmielinizantes del sistema nervioso central. Estudio observacional

Introducción. Presentamos un estudio observacional, retrospectivo, cuyo objetivo es analizar la utilidad de la plasmaféresis en el manejo de pacientes con esclerosis múltiple (EM) y otros procesos desmielinizantes agudos del sistema nervioso central (SNC) que presentan exacerbaciones graves rebeldes a la terapia convencional con corticoides. Pacientes y métodos. Incluimos 11 pacientes: nueve con EM, uno con encefalomielitis aguda diseminada y uno con mielitis transversa. Todos presentaban un déficit neurológico agudo o subagudo que les impedía la realización de sus actividades habituales, con o sin repercusión sobre la EDSS (escala ampliada del grado de discapacidad de Kurtzke), y con riesgo de sufrir discapacidad residual grave tras no responder a pulsos de metilprednisolona intravenosa. Cada paciente se sometió a tres recambios por semana, durante 2 semanas, con asociación de prednisona oral, y se evaluó tras la última sesión, al mes, a los seis y a los 12 meses. Resultados. Tras la plasmaféresis, todos experimentaron una reducción de la discapacidad significativa, y siete de ellos (77,7 por ciento del total con EM) incluso mejoraron respecto a su situación basal durante el primer mes (`ampliación del efecto Lázaro'). Tras un año de seguimiento, el 100 por ciento de los pacientes mantenía su recuperada situación basal anterior al empeoramiento, y se registraron únicamente dos brotes. Los pacientes con EM presentaron un `empeoramiento transitorio' tras el segundo recambio. Se asoció un nuevo tratamiento inmunosupresor, inmunomodulador, o ambos, en ocho casos. Conclusiones. Consideramos que la plasmaféresis es un procedimiento terapéutico eficaz y seguro en el manejo del paciente con EM y otros procesos desmielinizantes del SNC. Su utilización se debería considerar como de elección en los brotes graves y en formas con rápida progresión que no responden a la metilprednisolona intravenosa (AU)

Introduction. We present a retrospective observation study aimed at analyzing the value of plasmapheresis in the management of patients with multiple sclerosis (MS) and other acute demyelinating processes affecting the central nervous system (CNS) who show severe exacerbations that do not respond well to conventional therapy with corticoids. Patients and methods. A total of 11 patients were included in the study: nine with MS, one disseminated acute encephalomyelitis and one case of transverse myelitis. All of them presented an acute or subacute neurological deficit, which prevented them from carrying out their day-to-day activities, with or without repercussions on the EDSS, and with the risk of suffering a severe residual disability after not responding to intravenous methylprednisolone pulses. Each patient was submitted to three exchanges per week, for 2 weeks, with association of orally-administered prednisone and they were then evaluated after the last session and at one, six and twelve months. Results. Following plasmapheresis all the patients experienced a significant drop in disability and seven of them (77.7% of the total number with MS) even improved during the first month with respect to their basal situation (‘an extension of the Lazarus effect’). After a year’s follow-up, 100% of the patients still maintained the basal situation that was recovered from before exacerbation, and only two relapses were recorded. The patients with MS presented a ‘transient exacerbation’ after the second exchange. New therapy with immunosuppressants, immunomodulators or both was associated in eight cases. Conclusions. We consider plasmapheresis to be a safe, effective therapeutic procedure in the management of patients with MS and other demyelinating processes affecting the CNS. Its use should be considered as first choice in severe relapses and in swiftly progressing forms that do not respond to intravenous methylprednisolone (AU)

Réplica. Plexitis braquial como manifestación de la arteritis de células gigantes / Reply. Brachial plexitis as a manifestation of giant cell arteritis

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[Paraneoplastic cerebellar degeneration as initial presentation of a pancreatic small-cell carcinoma]. / Degeneración cerebelosa paraneoplásica como inicio de un carcinoma indiferenciado de células pequeñas de páncreas.

INTRODUCTION: Oat (small) cell carcinoma is the type of tumour most frequently associated to neurologic paraneoplastic syndromes. It is usually located in the lungs although it has been described in some other locations. Cerebellar symptoms may appear alone, associated to anti Yo antibodies ( Breast and gynaecologic carcinomas), or as manifestation of a more generalized paraneoplastic encephalopathy, associated to signs and symptoms of some other neurologic systems affected. CASE REPORT: A 52 year old patient consulted due to a pancerebellar clinical picture, which started about two months before, and later associated to polineuropathy. Abdominal CT showed a 4 cm mass in the head of the pancreas. Pathologic evaluation demonstrated a poorly differentiated small cell pancreatic tumour. Anti Hu antibodies in high titres were found both in serum and cerebrospinal fluid. DISCUSSION: The association of anti Hu immunity and paraneoplastic encephalomyelitis has been observed in patients with neuroblastoma, seminomas, colorectal, breast and prostate carcinomas and some types of sarcoma. Only about 1% of pancreatic malignancies correspond to small cell type. We have not found any previous report about the association between a paraneoplastic syndrome and pancreatic poorly differentiated small cell carcinoma.

Degeneración cerebelosa paraneoplásica como iniciode un carcinoma indiferenciado de células pequeñas de páncreas / Paraneoplastic cerebellar degeneration as initial presentation of a pancreatic small-cell carcinoma

Introducción. El carcinoma de células pequeñas es el tumor que con más frecuencia produce cuadros paraneoplásicos neurológicos. Su localización más frecuente es la pulmonar, aunque se ha descrito en otras localizaciones. La afectación cerebelosa puede aparecer de forma pura, asociada a anticuerpos anti-Yo (carcinoma de mama y ginecológicos), o formando parte de una encefalopatía paraneoplásica , asociada a otros signos y síntomas de afectación del sistema nervioso. Caso clínico. Varón de 52 años, con un cuadro pancerebeloso de dos meses de evolución y posterior asociación de una polineuropatía. En la tomografía computarizada abdominal aparece una masa de 4 cm en la cabeza pancreática. El estudio anatomopatológico determina la presencia de un carcinoma indiferenciado de células pequeñas de origen pancreático. Se identifican anticuerpos anti-Hu en altos títulos en suero y en líquido cefalorraquídeo. Discusión. Se han descrito pacientes con inmunidad anti-Hu y encefalomielitis paraneoplásica asociada a neuroblastoma, seminomas, adenocarcinoma de colon, cáncer de mama, cáncer de próstata y sarcomas. Sólo el 1 por ciento de los tumores malignos de páncreas corresponden al carcinoma indiferenciado de células pequeñas. En la bibliografía revisada no aparecen referencias publicadas que determinen la presencia de un síndrome paraneoplásico y el descubrimiento de un cáncer pancreático indiferenciado de células pequeñas (AU)