Asociación entre los polimorfismos genéticos de nucleótido único en genes transportadores ABC con la epilepsia farmacorresistente en la población española / Association between single nucleotide genetic polymorphisms in ABC transporter genes with drug-resistant epilepsy in the Spanish population

Introducción: El 30% de los pacientes con epilepsia no responde al tratamiento farmacológico. La presencia de polimorfismos genéticos de nucleótido único (SNP) en el individuo puede influir en la variabilidad de respuesta al tratamiento farmacológico. La hipótesis de transportadores plantea que la presencia de SNP en los genes que codifican las proteínas ABC repercutiría en la biodisponibilidad de los fármacos anticrisis en el foco epileptógeno, lo que ocasionaría refractariedad. El objetivo del presente estudio fue evaluar la asociación de 13 polimorfismos en los genes ABCB1, ABCC2, ABCC5 y ABCG2 con la epilepsia farmacorresistente (EFR) en población española. Sujetos y métodos: Se realizó un estudio de casos y controles que incluyó a 327 pacientes con epilepsia: 227 farmacorresistentes y 100 farmacocontrolados según los criterios de la Liga Internacional contra la Epilepsia. En el ADN de leucocitos de sangre periférica extraído se estudiaron los polimorfismos en los genes transportadores ABC. Se utilizó la plataforma tecnológica iPlex® Gold y Mass ARRAY. Se compararon las frecuencias alélicas y genotípicas del grupo de casos y del de controles, el valor de p, la odds ratio y los intervalos de confianza al 95%. Resultados: La frecuencia alélica y genotípica del presente estudio fue similar a la comunicada en las bases de datos poblacionales. En los SNP estudiados no se encontraron diferencias significativas (p > 0,05) en todos los modelos de herencia analizados. Conclusiones: Nuestros resultados sugieren que no existe asociación entre los polimorfismos analizados en los genes ABC con la EFR en población española. Sin embargo, otros estudios adicionales confirmarán o descartarán estos resultados.(AU)

Introduction: Almost a third of all patients with epilepsy (30%) fail to respond to pharmacological treatment. The presence of single nucleotide polymorphisms (SNPs) in the individual may influence the variability of the response to drug treatment. The transporter hypothesis posits that the presence of SNPs in the genes encoding ABC proteins would affect the bioavailability of antiseizure drugs at the epileptogenic focus, giving rise to refractoriness. The aim of the present study was to evaluate the association of 13 polymorphisms in the ABCB1, ABCC2, ABCC5 and ABCG2 genes with drug-resistant epilepsy (DRE) in a Spanish population. Subjects and methods: A case-control study was conducted involving 327 patients with epilepsy: 227 resistant to drug therapy and 100 in whom their medication enabled them to control their symptoms, according to International League Against Epilepsy criteria. In the peripheral blood leukocyte DNA that was extracted, polymorphisms in the ABC transporter genes were studied. The iPlex® Gold and Mass ARRAY technology platform was used. The allele and genotypic frequencies of the case and control groups, p-value, odds ratio and 95% confidence intervals were compared. Results: The allele and genotypic frequency of the present study was similar to that reported in population-based databases. For the SNPs studied, no significant differences (p > 0.05) were found in any of the inheritance models analysed. Conclusions: Our results suggest that there is no association between the polymorphisms analysed in the ABC genes and DRE in the Spanish population. Nevertheless, further studies will confirm or refute these results.(AU)

[Association between single nucleotide genetic polymorphisms in ABC transporter genes with drug-resistant epilepsy in the Spanish population]. / Asociación entre los polimorfismos genéticos de nucleótido único en genes transportadores ABC con la epilepsia farmacorresistente en la población española.

INTRODUCTION: Almost a third of all patients with epilepsy (30%) fail to respond to pharmacological treatment. The presence of single nucleotide polymorphisms (SNPs) in the individual may influence the variability of the response to drug treatment. The transporter hypothesis posits that the presence of SNPs in the genes encoding ABC proteins would affect the bioavailability of antiseizure drugs at the epileptogenic focus, giving rise to refractoriness. The aim of the present study was to evaluate the association of 13 polymorphisms in the ABCB1, ABCC2, ABCC5 and ABCG2 genes with drug-resistant epilepsy (DRE) in a Spanish population. SUBJECTS AND METHODS: A case-control study was conducted involving 327 patients with epilepsy: 227 resistant to drug therapy and 100 in whom their medication enabled them to control their symptoms, according to International League Against Epilepsy criteria. In the peripheral blood leukocyte DNA that was extracted, polymorphisms in the ABC transporter genes were studied. The iPlex® Gold and Mass ARRAY technology platform was used. The allele and genotypic frequencies of the case and control groups, p-value, odds ratio and 95% confidence intervals were compared. RESULTS: The allele and genotypic frequency of the present study was similar to that reported in population-based databases. For the SNPs studied, no significant differences (p > 0.05) were found in any of the inheritance models analysed. CONCLUSIONS: Our results suggest that there is no association between the polymorphisms analysed in the ABC genes and DRE in the Spanish population. Nevertheless, further studies will confirm or refute these results.

TITLE: Asociación entre los polimorfismos genéticos de nucleótido único en genes transportadores ABC con la epilepsia farmacorresistente en la población española.Introducción. El 30% de los pacientes con epilepsia no responde al tratamiento farmacológico. La presencia de polimorfismos genéticos de nucleótido único (SNP) en el individuo puede influir en la variabilidad de respuesta al tratamiento farmacológico. La hipótesis de transportadores plantea que la presencia de SNP en los genes que codifican las proteínas ABC repercutiría en la biodisponibilidad de los fármacos anticrisis en el foco epileptógeno, lo que ocasionaría refractariedad. El objetivo del presente estudio fue evaluar la asociación de 13 polimorfismos en los genes ABCB1, ABCC2, ABCC5 y ABCG2 con la epilepsia farmacorresistente (EFR) en población española. Sujetos y métodos. Se realizó un estudio de casos y controles que incluyó a 327 pacientes con epilepsia: 227 farmacorresistentes y 100 farmacocontrolados según los criterios de la Liga Internacional contra la Epilepsia. En el ADN de leucocitos de sangre periférica extraído se estudiaron los polimorfismos en los genes transportadores ABC. Se utilizó la plataforma tecnológica iPlex® Gold y Mass ARRAY. Se compararon las frecuencias alélicas y genotípicas del grupo de casos y del de controles, el valor de p, la odds ratio y los intervalos de confianza al 95%. Resultados. La frecuencia alélica y genotípica del presente estudio fue similar a la comunicada en las bases de datos poblacionales. En los SNP estudiados no se encontraron diferencias significativas (p > 0,05) en todos los modelos de herencia analizados. Conclusiones. Nuestros resultados sugieren que no existe asociación entre los polimorfismos analizados en los genes ABC con la EFR en población española. Sin embargo, otros estudios adicionales confirmarán o descartarán estos resultados.

[Bases of deep brain stimulation]. / Bases de la estimulación cerebral profunda.

INTRODUCTION: Deep brain stimulation is an effective therapy that is being used in an increasing number of indications. The mechanisms by which it exerts its therapeutic effect are still largely unknown, although there is increasing evidence of its influence at various levels. AIM: To review the existing literature on the mechanism of action of deep brain stimulation. DEVELOPMENT: Deep brain stimulation acts on brain tissue that is stimulated at various levels: molecular, cellular and neural networks. Spatial, temporal and electrical factors are involved in its effectiveness, but it mainly seems to perform its function by replacing anomalous firing patterns, which are present in certain neurological and psychiatric diseases. Other mechanisms, such as neuroprotection or neurogenesis, remain under study. CONCLUSIONS: Although many of the effects by which deep brain stimulation acts on the brain are still unknown, it seems to be a complex treatment, with large-scale effects, in which the correction of circuitopathies seems to prevail as the main mechanism.

TITLE: Bases de la estimulación cerebral profunda.Introducción. La estimulación cerebral profunda es una terapia eficaz que está siendo utilizada en un número creciente de indicaciones. Los mecanismos mediante los cuales ejerce efecto terapéutico aún se desconocen en su mayor parte, si bien cada vez se dispone de más datos sobre su influencia en diversos niveles. Objetivo. Revisar la bibliografía existente sobre el mecanismo de acción de la estimulación cerebral profunda. Desarrollo. La estimulación cerebral profunda actúa sobre el tejido cerebral estimulado en varios niveles, molecular, celular y de redes neuronales. En su efectividad intervienen factores espaciales, temporales y eléctricos, pero fundamentalmente parece ejercer su función mediante la sustitución de patrones de disparo anómalos, presentes en ciertas enfermedades neurológicas y psiquiátricas. Otros mecanismos, como la neuroprotección o la neurogénesis, permanecen en estudio. Conclusiones. Aunque aún se desconocen muchos efectos por los cuales la estimulación cerebral profunda actúa en el cerebro, parece un tratamiento complejo, con efectos a gran escala, en los que parece primar la corrección de circuitopatías como mecanismo principal.

[Deep brain stimulation in drug-resistant epilepsy]. / Estimulación cerebral profunda en la epilepsia farmacorresistente.

INTRODUCTION: Deep brain stimulation (DBS) in drug-resistant epilepsy has been applied to several brain targets. However, its exact mechanism of action is not known, and the diversity of targets makes it difficult to know the degree of evidence that supports its use. DEVELOPMENT: A review of the literature on DBS for drug-resistant epilepsy was conducted. The efficacy of DBS in drug-resistant epilepsy seems to be mediated by a desynchronisation of neuronal activity at the epileptogenic focus or a modulation of the «circuitopathies¼ that exist in epilepsy, depending on the target. In DBS multiple cortical and subcortical structures have been used, but class I evidence exists only for DBS of the anterior nucleus of the thalamus. CONCLUSIONS: DBS in epilepsy is still under investigation, with class I evidence for DBS of the anterior nucleus of the thalamus. The rest of the targets have yielded variable results that must be confirmed with randomised designs in larger series.

TITLE: Estimulación cerebral profunda en la epilepsia farmacorresistente.Introducción. La estimulación cerebral profunda (ECP) en la epilepsia farmacorresistente se ha aplicado en varias dianas cerebrales. Sin embargo, su mecanismo de acción no se conoce con exactitud, y la diversidad de dianas hace difícil conocer el grado de evidencia que apoya su utilización. Desarrollo. Se realiza una revisión bibliográfica sobre la ECP para la epilepsia farmacorresistente. La eficacia de la ECP en la epilepsia farmacorresistente parece mediada por una desincronización de la actividad neuronal en el foco epileptógeno o una modulación de las circuitopatías que existen en la epilepsia, dependiendo de la diana. En la ECP se han utilizado múltiples estructuras corticales y subcorticales, pero solamente la ECP del núcleo anterior del tálamo tiene una evidencia de clase I. Conclusiones. La ECP en la epilepsia es aún objeto de investigación, con evidencia de clase I en la ECP del núcleo anterior del tálamo. El resto de las dianas ha arrojado resultados variables que deben confirmarse con diseños aleatorizados en series de mayor tamaño.

[Is it possible to extract intracranial pressure information based on the EEG activity?] / Es posible obtener informacion de la presion intracraneal a partir de la actividad electroencefalografica?

INTRODUCTION: The capability of the electroencephalography (EEG) of recording the bioelectrical activity of the brain has made of it a fundamental tool for the evaluation of the patient's neurological condition. In recent years, moreover, it has also begun to be used in obtaining information for other kind of variables, as the ones related with the cerebral hemodynamics. AIM: To study the potential relationship between the EEG activity and the intracranial pressure (ICP) in patients suffering from traumatic brain injury and subarachnoid hemorrhage, during their stay at the intensive care unit. PATIENTS AND METHODS: Twenty-one adult patients (10 women) were included in the present observational prospective cohort study. They suffered from either traumatic brain injury or subarachnoid hemorrhage, requiring continuous EEG and ICP monitoring. In every patient, Granger causality between spectral functions of the EEG and the ICP was evaluated. Temporal windows of 10 minute were used to evaluate whether a causal relationship between those variables exist or not. In all of the cases, several days of continuous recording and assessment were performed. RESULTS: In most patients and during most of the time, Granger causality turns out to be significant in the direction from the EEG to the ICP, meaning that the EEG dynamics actually leads the ICP dynamics. CONCLUSIONS: The present work provides useful information and shed light in discovering a hidden relationship between the ICP and EEG dynamics. The potential use of this relationship could lead to develop a medical device to measure ICP in a non-invasive fashion.

TITLE: Es posible obtener informacion de la presion intracraneal a partir de la actividad electroencefalografica?Introduccion. El electroencefalograma (EEG) permite obtener informacion directa de la actividad bioelectrica del cerebro y es una herramienta fundamental para la evaluacion de la condicion neurologica del paciente. En los ultimos años ha comenzado a emplearse tambien para obtener indirectamente informacion sobre la hemodinamica cerebral y las variables que intervienen en la autorregulacion del flujo sanguineo cerebral. Objetivo. Estudiar la posible relacion entre la actividad electroencefalografica y la presion intracraneal (PIC) en pacientes con traumatismo craneoencefalico y hemorragia subaracnoidea ingresados en cuidados intensivos. Pacientes y metodos. Se incluyo a 21 pacientes (10 mujeres) mayores de 18 años con traumatismo craneoencefalico o hemorragia subaracnoidea que requerian monitorizacion de la PIC y a los que se les registro el EEG de forma continua. Se determino la causalidad de Granger entre la PIC con respecto a las variables espectrales del EEG para ventanas temporales de 10 minutos durante la estancia en cuidados intensivos. Resultados. La causalidad de Granger mostro una alta correlacion entre la PIC con las bandas del EEG. En la mayoria de los pacientes existe una causalidad de Granger significativa en la direccion del EEG hacia la PIC en gran parte del tiempo de monitorizacion, de forma que las variables del EEG precedian a la PIC. Conclusiones. El presente trabajo expone la relacion temporal subyacente entre la dinamica de la PIC y la actividad bioelectrica cerebral registrada mediante EEG en pacientes con traumatismo craneoencefalico y hemorragia subaracnoidea. El potencial uso de esta relacion podria permitir estimar la PIC de manera no invasiva.

[Medullar thoracic compression by tophaceous gout: presentation of a case and review of the literature]. / Compresion medular dorsal por tofos gotosos: presentacion de un caso y revision de la bibliografia.

INTRODUCTION: Spine involvement in gout is an extremely uncommon complication. Dorsalgia and quadriplegia are some manifestations that may occur, although these symptoms are seen more frequently in other more prevalent pathologies, such as spinal tumors. CASE REPORT: We present an unusual case of thoracic spinal cord compression at T10-T11 level caused by the extradural deposit of tophaceous material in a 52-year-old woman with uncontrolled chronic tophaceous gout. In addition to intensive medical treatment, the patient required surgery (hemilaminectomy and spinal decompression) and subsequent rehabilitation. Overall and neurological evolution were satisfactory.

TITLE: Compresion medular dorsal por tofos gotosos: presentacion de un caso y revision de la bibliografia.Introduccion. La afectacion de la columna vertebral en la gota es una complicacion extremadamente infrecuente. La dorsalgia y la cuadriplejia son algunas manifestaciones que se pueden presentar, aunque estos sintomas se ven con mas frecuencia en otras patologias mas prevalentes, como los tumores medulares. Caso clinico. Se presenta un caso inusual de compresion medular dorsal en D10-D11 causado por el deposito extradural de material tofaceo en una paciente de 52 años con gota tofacea cronica incontrolada. Ademas de un tratamiento medico intensivo, la paciente requirio cirugia (hemilaminectomia y descompresion medular) y rehabilitacion posterior. La evolucion general y neurologica fue satisfactoria.

[Haemorrhagic pineal cysts: two clinical cases successfully treated by means of microsurgery]. / Quistes pineales hemorragicos: dos casos clinicos tratados con exito mediante microcirugia.

INTRODUCTION: Pineal cysts are a relatively frequent incidental finding in imaging tests; yet, pineal apoplexy is considered to be rare and is associated to severe symptoms. CASE REPORTS: We report the cases of a 25-year-old male and a female aged 15 years who visited the emergency department with signs and symptoms of intracranial hypertension. The existence of a pineal haemorrhage was confirmed by imaging tests. They were successfully treated by means of microsurgery. CONCLUSION: In our experience, and backed by the literature, we believe that the best treatment for this infrequent pathology is the microsurgical approach. Nevertheless, we do not rule out the possibility that, sometime in the future, endoscopic techniques may play an important role in the treatment of pineal apoplexy.

TITLE: Quistes pineales hemorragicos: dos casos clinicos tratados con exito mediante microcirugia.Introduccion. Los quistes pineales son un hallazgo incidental relativamente frecuente en las pruebas de imagen; sin embargo, la apoplejia pineal se considera rara y se asocia a sintomas graves. Casos clinicos. Varon de 25 a˜os y mujer de 15 a˜os, que acudieron a urgencias con clinica de hipertension intracraneal; mediante pruebas de imagen se confirmo la existencia de una hemorragia pineal. Se les trato con exito mediante microcirugia. Conclusion. En nuestra experiencia, y avalado por la bibliografia, creemos que el mejor tratamiento de esta infrecuente patologia es el abordaje microquirurgico. Sin embargo, no se excluye la posibilidad de que, en un futuro, las tecnicas endoscopicas puedan tener un lugar importante en el tratamiento de la apoplejia pineal.

Classification of structural lesions in magnetic resonance imaging. Surgical implications in drug-resistant epilepsy patients.

INTRODUCTION: The presence of a structural lesion in the preoperative magnetic resonance imaging (MRI) of drug-resistant epilepsy patients has been usually associated with a favourable surgical outcome. We present our experience in our Epilepsy Surgery Unit. PATIENTS AND METHODS: Clinical records from 265 patients, operated on from 1990-2010 in our institution, were reviewed. Patients were classified, according to MRI findings, into three groups: surgical lesion (SL), tumors or vascular malformations requiring surgery 'per se'; orientative lesion (OL), dysplasia, atrophy or mesial temporal sclerosis; and (NL) group, with normal MRI. Seizure outcomes were analysed in relation to this classification. RESULTS: Period 1990-2000, 151 patients: 87% of SL, 65% of OL and 57% of NL patients were in Engel class I or II at the two-year follow-up. Among temporal lobe epilepsy cases (TLE), 87% of SL, 67% of OL and 56% of NL patients achieved seizure control. Differences were statistically significative. Period 2001-2010, 114 patients: 100% of SL, 90% of OL, and 81% of NL patients were in Engel's class I or II. Both TLE and extratemporal (ETLE) SL patients obtained a 100% seizure control. Among the OL patients, 95% with TLE and 43% of ETLE achieved seizure control. In the NL group, the percentages were 88% in TLE, and 50% in ETLE. CONCLUSIONS: In our series, SL was a predictor of a favorable outcome. In TLE patients, good results were achieved despite normal MRI. Patients with ETLE and NL did not have a worse outcome than those with OL. A classification in SL, OL and NL seems more helpful for predicting the surgical outcome than the traditional classification lesion versus non-lesion MRI. Radiological findings must be carefully evaluated in the context of a complete epilepsy surgery evaluation.

TITLE: Clasificacion de las lesiones estructurales en resonancia magnetica. Implicaciones quirurgicas en pacientes con epilepsia farmacorresistente.Introduccion. En la seleccion quirurgica del paciente con epilepsia farmacorresistente, el papel de la resonancia magnetica (RM) no se ha cuantificado hasta el momento. Presentamos la experiencia en nuestra Unidad de Cirugia de la Epilepsia. Pacientes y metodos. Se estudiaron retrospectivamente los pacientes intervenidos por epilepsia farmacorresistente. Distinguimos dos periodos: 1990-2000 (RM de 0,5 T) y 2001-2008 (RM de 1,5 T). La RM preoperatoria se clasifico en tres grupos: RM con lesion quirurgica (LQ), RM orientativa (LO) y RM normal (NL). Tambien se efectuo una clasificacion anatomopatologica similar. Se correlacionaron las distintas clasificaciones y los resultados quirurgicos. Resultados. Periodo 1990-2000: 151 pacientes. El 70% quedo en las clases de Engel I o II. Segun la RM, los resultados fueron: LQ, 87%; LO, 65%; y NL, 57%. Las diferencias fueron estadisticamente significativas. Periodo 2001-2008: 114 pacientes. El 89% quedo en las clases de Engel I o II. Segun la RM: LQ, 100%; LO, 90%; y NL, 81%. Las diferencias fueron estadisticamente significativas. Los pacientes con epilepsia del lobulo temporal y extratemporal con LQ tuvieron un 100% de control; con LO, el 95% con epilepsia del lobulo temporal y el 43% con estado epileptico; en aquellos pacientes sin lesion (NL), el 88% con epilepsia del lobulo temporal se controlo frente al 50% con estado epileptico. Conclusiones. La RM es una herramienta eficaz en la seleccion de candidatos quirurgicos en la epilepsia. La LQ asocia muy buen pronostico. En la epilepsia del lobulo temporal se pueden obtener muy buenos resultados (80-90% de control) a pesar de una RM normal. En el estado epileptico, las LO pueden tener peor resultado que la NL en la RM.

[Frontal aneurysmal bone cyst. A case report and review of the literature]. / Quiste oseo aneurismatico frontal. Caso clinico y revision de la bibliografia.

INTRODUCTION: Cranial aneurysmal bone cyst (ABC) is a rare pathological finding that is usually diagnosed in young patients. It is defined as a benign lesion made up of intervillous spaces limited by connective bone tissue septa and osteoclast-type giant cells; these histological characteristics are common to extracranial ABC. Clinically, in most patients, they manifest as cranial tumours which may be painful and vary in size. About 100 cases of cranial ABC have been reported in the literature. We present a literature review of the cases of frontal ABC reported to date, including one that was recently treated in our own service. CASE REPORT: A 29-year-old female, with no history of traumatic brain injury, who was examined due to the presence of a fixed, painless frontal cranial lesion on the right-hand side. The results of a radiological study revealed the presence of a lytic cranial lesion with well-delimited edges and marginal sclerosis; peripheral and linear contrast enhancement was also observed in the magnetic resonance imaging of the brain. The patient was submitted to a surgical intervention and the entire lesion was removed. There were no incidents in the post-operative period and the definitive pathological diagnosis was cranial ABC. CONCLUSIONS: Cranial ABC is a pathological condition with well-defined histological and radiological characteristics, despite the fact that its aetiopathogenesis is still not fully understood. Complete excision of the tumour is considered to be the preferred treatment, which often leads to full recovery of the patient and also offers a good long-term prognosis.

[Unilateral subthalamic stimulation in the treatment of advanced Parkinson's disease]. / Estimulación subtalámica unilateral en el tratamientode la enfermedad de Parkinson avanzada.

INTRODUCTION: The use of unilateral subthalamic stimulation (USTS) in the treatment of advanced Parkinson's disease can be effective and offer a series of advantages on comparing this technique with bilateral subthalamic stimulation (BSTS). PATIENTS AND METHODS: We studied 35 consecutive patients: 22 with BSTS and 13 with USTS (six left and seven right). The epidemiological features and the scores on the functional assessment scales -Hoehn and Yahr, Schwab and England, and the Unified Parkinson's Disease Rating Scale (UPDRS) I to IV- were not significantly different in the two groups, except for the Hoehn and Yahr in off (USTS: 3.3 +/- 0.3; BSTS: 4.1 +/- 0.2; p = 0.004). RESULTS: The overall percentages of improvement six months after surgery were UPDRS I: 12%; II: 21.6%; III-medication off/stimulator on: 30.6% (with respect to the baseline off state); III-on/on: 8.8% (with respect to the baseline on state); IV: 48.9%. Reduction in the levodopa equivalent dose: USTS: 26.3%; BSTS: 17%. These percentages of improvement were not significantly different in the two groups. If we consider the motor scale in medication-off and stimulator-on in the sixth month and we compare it with the same in the off state before surgery, the axial motor symptoms improved by 17.1% (USTS) and 25% (BSTS); in the extremities, USTS: 39.1% in the limbs contralateral to the implanted electrode, and 14.5% in the ipsilaterals; BSTS: right extremities, 32.6%; left extremities, 31.5%. No significant differences were found on comparing the electrical power consumed by the electrodes in the two groups of patients in the sixth month of treatment. CONCLUSIONS: USTS was effective in improving the axial symptoms. In our series, the reduction in medication following surgery was similar to that of the patients with BSTS.

[Treatment of hydrocephalus secondary to intraventricular haemorrhage in preterm infants. A review of the literature]. / Tratamiento de la hidrocefalia secundaria a hemorragia intraventricular en el prematuro. Revisión bibliográfica.

INTRODUCTION: The current incidence of intraventricular haemorrhage in low-weight preterm infants is 20%. The percentage of patients who are going to develop hydrocephalus secondary to this haemorrhage varies greatly. In the last 20 years different medical and surgical treatments have been put forward to prevent both the occurrence of haemorrhage and the development of hydrocephalus. DEVELOPMENT: In this study we review the literature on the different treatments used to control and treat hydrocephalus. From the medical point of view, treatment with pharmacological agents such as azetazolamide or furosemide offers more drawbacks than advantages as far as their capacity to prevent hydrocephalus is concerned. Treatments involving fibrinolytic agents offer a high risk of triggering new haemorrhages but in recent years their use has been taken up again in combination with ventricular drains. As regards surgical treatment of hydrocephalus due to haemorrhage, despite the risk of infection, it is more advisable to use ventricular drains than to introduce subgaleal reservoirs. At the present time, the best definitive treatment for hydrocephalus in preterm infants is still the ventriculoperitoneal shunt. The most suitable time is when the weight of the newborn infant exceeds 1500 g and the cerebrospinal fluid offers a protein count above 200 mg/dL. CONCLUSIONS: The long-term neurological development of these children depends mainly on the severity of the haemorrhage, but poorer prognoses are observed in cases in which complications arise from the control examinations of the valve shunt systems.

Tratamiento de la hidrocefalia secundaria a hemorragia intraventricular en el prematuro. Revisión bibliográfica / Treatment of hydrocephalus secondary to intraventricular haemorrhage in preterm infants. A review of the literature

Introducción. La hemorragia intraventricular en los prematuros de poco peso tiene una incidencia actual de un 20%. El porcentaje de pacientes que van a desarrollar hidrocefalia secundaria a esta hemorragia es muy variable. En los últimos 20 años se han descrito diferentes tratamientos médicos y quirúrgicos para evitar que se produzca la hemorragia y que se desarrolle una hidrocefalia. Desarrollo. En este trabajo se ha realizado una revisión bibliográfica de los diferentes tratamientos para controlar y tratar la hidrocefalia. Desde el punto de vista médico se ha comprobado que los tratamientos con fármacos como la acetazolamida o furosemida presentan más desventajas que beneficios en cuanto a la capacidad para prevenir la hidrocefalia. Los tratamientos con fibrinolíticos tienen un alto riesgo de provocar nuevas hemorragias, pero en los últimos años se ha reiniciado su utilización combinada con drenajes ventriculares. En cuanto al tratamiento quirúrgico de la hidrocefalia por la hemorragia, a pesar del riesgo de infección, es más aconsejable el drenaje ventricular que la colocación de reservorios subgaleales. En el momento actual, el mejor tratamiento definitivo para la hidrocefalia del prematuro continua siendo la derivación ventriculoperitoneal. El momento más oportuno es cuando el neonato presente un peso superior a 1.500 g y el líquido cefalorraquídeo tenga una cifra de proteínas inferior a 200 mg/dL. Conclusión. El desarrollo neurológico a largo plazo de estos niños depende principalmente de la gravedad de la hemorragia, pero se observa un peor pronóstico en aquellos casos en los que existen complicaciones derivadas de las revisiones de los sistemas derivativos valvulares

Introduction. The current incidence of intraventricular haemorrhage in low-weight preterm infants is 20%. The percentage of patients who are going to develop hydrocephalus secondary to this haemorrhage varies greatly. In the last 20 years different medical and surgical treatments have been put forward to prevent both the occurrence of haemorrhage and the development of hydrocephalus. Development. In this study we review the literature on the different treatments used to control and treat hydrocephalus. From the medical point of view, treatment with pharmacological agents such as azetazolamide or furosemide offers more drawbacks than advantages as far as their capacity to prevent hydrocephalus is concerned. Treatments involving fibrinolytic agents offer a high risk of triggering new haemorrhages but in recent years their use has been taken up again in combination with ventricular drains. As regards surgical treatment of hydrocephalus due to haemorrhage, despite the risk of infection, it is more advisable to use ventricular drains than to introduce subgaleal reservoirs. At the present time, the best definitive treatment for hydrocephalus in preterm infants is still the ventriculoperitoneal shunt. The most suitable time is when the weight of the newborn infant exceeds 1500 g and the cerebrospinal fluid offers a protein count above 200 mg/dL. Conclusions. The long-term neurological development of these children depends mainly on the severity of the haemorrhage, but poorer prognoses are observed in cases in which complications arise from the control examinations of the valve shunt systems

Tratamiento quirúrgico de los quistes aracnoideos sintomáticos en niños / Surgical treatment of symptomatic arachnoid cysts in children

Introducción. Los quistes aracnoideos (QA) deben tratarse quirúrgicamente cuando son sintomáticos. Actualmente persiste todavía la controversia sobre la mejor técnica quirúrgica: derivación cistoperitoneal (CPS) o fenestración del quiste, bien mediante craneotomía o por técnicas endoscópicas. Pacientes y métodos. Se presentan los resultados de una serie de 18 pacientes con edad inferior a los 10 años que se han tratado de QA sintomáticos. 12 pacientes presentaron aumento del perímetro craneal, 4 cefaleas y 2 niños crisis comiciales. La localización ha sido supratentorial en 11 casos y 7 intratentorial. Resultados. En 12 casos el tratamiento realizado ha sido la derivación cistoperitoneal o ventriculoperitoneal. En 5 pacientes se ha realizado tratamiento endoscópico del quiste y en 1 caso desbridamiento mediante craneotomía. De los 18 niños, 7 han precisado una segunda intervención para resolver la clínica, bien por mal funcionamiento valvular o por insuficiencia del tratamiento endoscópico. Como complicaciones se han producido 2 hematomas subdurales, que han precisado tratamiento quirúrgico. No se ha producido mortalidad ni morbilidad. Conclusiones. Los avances en técnicas endoscópicas pueden ser el tratamiento ideal frente al desbridamiento por craneotomía, aunque hay que tener en cuenta el alto porcentaje de no resolución en niños de menos de 15 meses. La CPS soluciona el problema de estos quistes con menor riesgo quirúrgico, pero tiene un alto índice de reintervenciones, además de la dependencia del shunt. En la revisión bibliográfica realizada se comprueba que se publican todavía series tratadas tanto con fenestración del quiste como mediante derivación (AU)

Introduction. When symptomatic, arachnoid cysts (AC) must be treated surgically. The best surgical technique, however, is at the present time still subject to controversy –implantation of a cyst-peritoneal shunt (CPS) or fenestration of the cyst, either by means of a craniotomy or by using endoscopic techniques. Patients and methods. This paper reports the findings from a series of 18 patients under 10 years of age who were treated for symptomatic ACs. An increase in the cranial perimeter was observed in 12 patients, 4 had headaches and 2 children suffered convulsive crises. In 11 cases the location was supratentorial and in 7 it was found to be infratentorial. Results. Treatment involved a cyst-peritoneal or ventriculo peritoneal shunt in 12 cases. Endoscopic treatment of the cyst was carried out in 5 of the patients and in 1 case craniotomy debridement was performed. Seven of the 18 children required a second intervention to resolve the clinical condition, either due to poor valve functioning or because the endoscopic treatment was insufficient. Complications included 2 subdural haematomas, which required surgical treatment. No mortality or morbidity occurred. Conclusions. The progress being accomplished in endoscopic techniques can make them the ideal form of treatment rather than craniotomy debridement techniques, although the high percentage of no-resolution in children below the age of 15 months must be taken into account. CPS solves the problem of these cysts with a lower degree of surgical risk, but it has a high rate of reintervention, as well as the dependence on the shunt. In the review of the literature we carried out it was seen that reports are still published concerning series treated by both cyst fenestration and by means of shunts (AU)

[Surgical treatment of symptomatic arachnoid cysts in children]. / Tratamiento quirúrgico de los quistes aracnoideos sintomáticos en niños.

INTRODUCTION: When symptomatic, arachnoid cysts (AC) must be treated surgically. The best surgical technique, however, is at the present time still subject to controversy -implantation of a cyst-peritoneal shunt (CPS) or fenestration of the cyst, either by means of a craniotomy or by using endoscopic techniques. PATIENTS AND METHODS: This paper reports the findings from a series of 18 patients under 10 years of age who were treated for symptomatic ACs. An increase in the cranial perimeter was observed in 12 patients, 4 had headaches and 2 children suffered convulsive crises. In 11 cases the location was supratentorial and in 7 it was found to be infratentorial. RESULTS: Treatment involved a cyst-peritoneal or ventriculoperitoneal shunt in 12 cases. Endoscopic treatment of the cyst was carried out in 5 of the patients and in 1 case craniotomy debridement was performed. Seven of the 18 children required a second intervention to resolve the clinical condition, either due to poor valve functioning or because the endoscopic treatment was insufficient. Complications included 2 subdural haematomas, which required surgical treatment. No mortality or morbidity occurred. CONCLUSIONS: The progress being accomplished in endoscopic techniques can make them the ideal form of treatment rather than craniotomy debridement techniques, although the high percentage of no-resolution in children below the age of 15 months must be taken into account. CPS solves the problem of these cysts with a lower degree of surgical risk, but it has a high rate of reintervention, as well as the dependence on the shunt. In the review of the literature we carried out it was seen that reports are still published concerning series treated by both cyst fenestration and by means of shunts.

[Pharmacoresistant temporal-lobe epilepsy. Exploration with foramen ovale electrodes and surgical outcomes]. / Epilepsia farmacorresistente del lóbulo temporal. Exploración con electrodos del foramen oval y resultados quirúrgicos.

AIM: To report our experience in the surgical treatment of temporal-lobe epilepsy. PATIENTS AND METHODS: An analysis was performed of the outcomes of 137 patients submitted to surgery between 1990 and 2001, with a follow-up of more than two years. A study was conducted of the percentages of successful detection by the different complementary tests--MRI, EEG, interictal SPECT, video-EEG with foramen ovale electrodes (FOE), neuropsychological study (NPS)--and the precision with which they reported the epileptogenic focus. RESULTS AND CONCLUSIONS: Successful surgical outcomes (Engel grades I-II): 73.4%. No surgical mortality occurred, although some mild, reversible morbidity was observed. Surgical outcomes were not affected by sex, age, age of onset and the length of time the patient had had the disease, or the frequency of the seizures. No association was found between seizures in the immediate post-operative period and a poorer long term control of the epilepsy. MR images were normal in 25% of patients; in these cases the surgical outcomes (Engel grades I-II at two years: 62%) were significantly poorer than in cases of tumours/cavernomas (86%); RMI studies of other types of lesions gave intermediate results (72%). With respect to the capacity of the different tests to lateralise/locate the epileptogenic focus, video-EEG-FOE proved to be the best, followed by MRI, SPECT, EEG and NPS.

Epilepsia farmacorresistente del lóbulo temporal. Exploración con electrodos del foramen oval y resultados quirúrgicos / Pharmacoresistant temporal-lobe epilepsy. Exploration with foramen ovale electrodes and surgical outcomes

Objetivo. Presentar nuestra experiencia en el tratamiento quirúrgico de la epilepsia del lóbulo temporal. Pacientes y métodos. Se analizan resultados en 137 pacientes, intervenidos quirúrgicamente entre 1990 y 2001, con seguimiento mayor de dos años. Se estudia el porcentaje de aciertos de las pruebas complementarias-RM, EEG, SPECT interictal, vídeo-EEG con electrodos del foramen oval (EFO), estudio neuropsicológico (NPS)- y la precisión con que informaron del foco epileptógeno. Resultados y conclusiones. Éxitos quirúrgicos (grados I-II de Engel): 73,4%. No hubo mortalidad quirúrgica, aunque sí escasa morbilidad, leve yreversible. No modificaron los resultados quirúrgicos ni el sexo, edad, edad de comienzo y tiempo de desarrollo de la enfermedad, ni la frecuencia de las crisis. No encontramos asociación entre crisis en el postoperatorio inmediato y peor control de la epilepsia alargo plazo. La RM fue normal en el 25% de los pacientes; en ellos, los resultados quirúrgicos (grados I-II de Engel a los dos años: 62%) fueron significativamente peores que en los casos detumor/cavernoma (86%); ante otras lesiones en la RM, el resultado fue intermedio (72%). En relación a la capacidad lateralizadora/localizadora del foco epileptógeno de las diferentes pruebas, destaca el vídeo-EEG-EFO, seguido de la RM, SPECT, EEG y NPS

Aim. To report our experience in the surgical treatment of temporal-lobe epilepsy. Patients and methods. Ananalysis was performed of the outcomes of 137 patients submitted to surgery between 1990 and 2001, with a follow-up of more than two years. A study was conducted of the percentages of successful detection by the different complementary tests - MRI,EEG, interictal SPECT, video-EEG with foramen ovale electrodes (FOE), neuropsychological study (NPS) - and the precision with which they reported the epileptogenic focus. Results and conclusions. Successful surgical outcomes (Engel grades I-II):73.4%. No surgical mortality occurred, although some mild, reversible morbidity was observed. Surgical outcomes were not affected by sex, age, age of onset and the length of time the patient had had the disease, or the frequency of the seizures. No association was found between seizures in the immediate post-operative period and a poorer long term control of the epilepsy.MR images were normal in 25% of patients; in these cases the surgical outcomes (Engel grades I-II at two years: 62%) were significantly poorer than in cases of tumours/cavernomas (86%); RMI studies of other types of lesions gave intermediate results (72%). With respect to the capacity of the different tests to lateralise/locate the epileptogenic focus, video-EEG-FOE proved to be the best, followed by MRI, SPECT, EEG and NPS

[Lumbar spinal surgery in elderly patients]. / Cirugía de columna lumbar en pacientes de edad avanzada.

INTRODUCTION: In the geriatric population, pain with sciatic irradiation requires a differential diagnosis to enable a distinction to be made mainly between a herniated disc, lateral recess stenosis or lumbar stenosis. In addition, in many cases the degenerative problems are often associated with lumbar listhesis or instability. Furthermore, these patients present very diverse associated cardiovascular, pulmonary or metabolic pathologies which can make surgery complicated and, above all, prolong post-operative recovery, as well as increasing morbidity and mortality. PATIENTS AND METHODS: We reviewed a group of 50 patients aged between 70 and 87 who had been submitted to surgery between 1997 and 2003; 27 were females and 23 males. 76% of them presented associated systemic pathologies and 22% had a history of previous spinal surgery. In 15 cases clinical symptoms were gait disorders involving claudication, there were three cases of paraparesis with cauda equina syndrome, 19 lumbagos with bilateral sciatica and 16 cases of lumbago with unilateral sciatica. Unilateral decompression hemilaminectomy was performed in 16 patients (group I) with microdiscectomy in 13 cases, laminectomy of one or several vertebrae (group II) was carried out in 17 patients and another 17 patients were submitted to decompression laminectomy plus arthrodesis with transpedicular instrumentation (group III). RESULTS: Overall a significant improvement was observed in 86% of patients. Detected complications involved two serious deep infections (4%), one of which was secondary to cerebrospinal fluid fistula, and the other occurred in an instrumented patient. No instabilities secondary to the laminectomy were observed in non-instrumented patients. No intraoperative anaesthetic or surgical complications were produced. Patients are followed up simultaneously during the post-operative period by both Internal Medicine and Neurosurgery. CONCLUSIONS: In the geriatric population there is a high incidence of degenerative problems, not only involving canal stenosis but also herniated discs. Surgical intervention must be the least aggressive possible, bearing in mind that a laminectomy does not presuppose post-surgical stability. The associated pathologies presented by these patients make a good immediate post-operative follow-up indispensable if morbidity and mortality rates are to be reduced.

Cirugía de la columna lumbar en pacientes de edad avanzada / Lumbar spinal surgery in elderly patients

Introducción. En la población geriátrica el dolor con irradiación ciática requiere hacer un diagnóstico diferencial, principalmente, entre hernia discal, estenosis del receso lateral o estenosis lumbar. Los problemas degenerativos suelen asociarse, además, en muchos casos, con inestabilidad o listesis lumbar. Por otra parte, estos pacientes presentan asociadas muy diversas patologías cardiovasculares, pulmonares o metabólicas, que pueden complicar la propia cirugía y, sobre todo, prolongar la recuperación posoperatoria y aumentar la morbimortalidad. Pacientes y métodos.Hemos revisado un grupo de 50 pacientes intervenidos entre los años 1997 y 2003, con edades comprendidas entre los 70 y los 87 años, 27 mujeres y 23 hombres. El 76 por ciento de ellos presentaba patología sistémica asociada y un 22 por ciento tenía un antecedente de cirugía de columna previa. En 15 casos la clínica fue de claudicación en la marcha; en 3, de paraparesia con síndrome de cola de caballo; en 19, de lumbalgia y ciática bilateral, y en 16, de lumbalgia con ciática unilateral. En 16 pacientes se realizó hemilaminectomía descompresiva unilateral (grupo I), con microdiscectomía en 13 casos; en 17 pacientes, laminectomía de uno o varias vértebras (grupo II), y en otros 17 pacientes, laminectomía descompresiva más artrodesis con instrumentación transpedicular (grupo III). Resultados. Se observa una mejoría significativa global del 86 por ciento de los pacientes.Las complicaciones detectadas han sido dos infecciones profundas graves (4 por ciento), una de ellas secundaria a una fístula de líquido cefalorraquídeo y otra en un paciente instrumentado. No se han observado inestabilidades secundarias a la laminectomía en los pacientes no instrumentados. No se han producido complicaciones intraoperatorias anestésicas ni quirúrgicas. Los pacientes fueron seguidos en el posoperatorio por Medicina Interna y Neurocirugía de forma simultánea. Conclusiones. En la población geriátrica existe una alta incidencia de problemas degenerativos; no sólo de estenosis de canal, sino también de hernia discal. La intervención que se plantea debe ser lo menos agresiva posible, teniendo en cuenta que una laminectomía no presupone inestabilidad posquirúrgica. Por las patologías asociadas que presentan estos pacientes, se necesita un buen seguimiento posoperatorio inmediato para disminuir la morbimortalidad (AU)

Introduction. In the geriatric population, pain with sciatic irradiation requires a differential diagnosis to enable a distinction to be made mainly between a herniated disc, lateral recess stenosis or lumbar stenosis. In addition, in many cases the degenerative problems are often associated with lumbar listhesis or instability. Furthermore, these patients present very diverse associated cardiovascular, pulmonary or metabolic pathologies which can make surgery complicated and, above all, prolong post-operative recovery, as well as increasing morbidity and mortality. Patients and methods. We reviewed a group of 50 patients aged between 70 and 87 who had been submitted to surgery between 1997 and 2003; 27 were females and 23 males. 76% of them presented associated systemic pathologies and 22% had a history of previous spinal surgery. In 15 cases clinical symptoms were gait disorders involving claudication, there were three cases of paraparesis with cauda equina syndrome, 19 lumbagos with bilateral sciatica and 16 cases of lumbago with unilateral sciatica. Unilateral decompression hemilaminectomy was performed in 16 patients (group I) with microdiscectomy in 13 cases, laminectomy of one or several vertebrae (group II) was carried out in 17 patients and another 17 patients were submitted to decompression laminectomy plus arthrodesis with transpedicular instrumentation (group III). Results. Overall a significant improvement was observed in 86% of patients. Detected complications involved two serious deep infections (4%), one of which was secondary to cerebrospinal fluid fistula, and the other occurred in an instrumented patient. No instabilities secondary to the laminectomy were observed in non-instrumented patients. No intraoperative anaesthetic or surgical complications were produced. Patients are followed up simultaneously during the post-operative period by both Internal Medicine and Neurosurgery. Conclusions. In the geriatric population there is a high incidence of degenerative problems, not only involving canal stenosis but also herniated discs. Surgical intervention must be the least aggressive possible, bearing in mind that a laminectomy does not presuppose post-surgical stability. The associated pathologies presented by these patients make a good immediate postoperative follow-up indispensable if morbidity and mortality rates are to be reduced (AU)

[Dural fistula in the craniocervical junction. A case report and review of the literature]. / Fistula dural en la union craneocervical. Presentacion de caso clinico y revision de la bibliografia.

INTRODUCTION: Spinal dural fistulas consist in an artery that penetrates into the dura mater and drains into a perispinal vein. They are most commonly located in the dorsal or lumbar region; a craniocervical position is infrequent. The clinical features presented by these patients involve an progressive ascending myelopathy caused by severe venous hypertension, although they can also start with symptoms of a subarachnoid haemorrhage. CASE REPORT: A 62 year old male with symptoms of progressive myelopathy due to a dural fistula dependent on meningeal branches of the right PICA, which was treated surgically by fistula clipping performed using an extreme lateral suboccipital approach. In the literature that was reviewed there were 35 cases of dural fistulas located at the craniocervical junction. CONCLUSIONS: The existence of clinical features compatible with cervical myelopathy and an anodyne cervical resonance justify the need to perform medullar arteriography owing to a suspected vascular malformation. An early diagnosis of a dural fistula and its proper treatment lead to a diminished morbidity and mortality rate in these patients.

Fístula dural en la unión craneocervical. Presentación de un caso clínico y revisión de la bibliografía / Dural fistula in the craniocervical junction. A case report and review of the literature

Introducción. Las fístulas durales medulares consisten en una arteria que penetra en la duramadre y drena en una vena perimedular. Las localizaciones más frecuentes son dorsal y lumbar; la craneocervical es infrecuente. La clínica que presentan estos pacientes es de mielopatía progresiva ascendente por hipertensión venosa grave, aunque también puede iniciarse con un cuadro de hemorragia subaracnoidea. Caso clínico. Varón de 62 años, con cuadro de mielopatía progresiva por fístula dural dependiente de las ramas meníngeas de la arteria cerebelosa posteroinferior derecha, que se trató quirúrgicamente con la realización de un pinzamiento de la fístula mediante abordaje extremolateral suboccipital. En la bibliografía revisada hay publicados 35 casos de fístulas durales localizadas en la unión craneocervical. Conclusiones. La existencia de una clínica compatible con mielopatía cervical y una resonancia cervical anodina justifica la realización de una arteriografía medular por sospecha de una malformación vascular. El diagnóstico precoz de una fístula dural y su correcto tratamiento supone una disminución de la morbimortalidad de estos pacientes (AU)

Introduction. Spinal dural fistulas consist in an artery that penetrates into the dura mater and drains into a perispinal vein. They are most commonly located in the dorsal or lumbar region; a craniocervical position is infrequent. The clinical features presented by these patients involve an progressive ascending myelopathy caused by severe venous hypertension, although they can also start with symptoms of a subarachnoid haemorrhage. Case report. A 62-year-old male with symptoms of progressive myelopathy due to a dural fistula dependent on meningeal branches of the right PICA, which was treated surgically by fistula clipping performed using an extreme lateral suboccipital approach. In the literature that was reviewed there were 35 cases of dural fistulas located at the craniocervical junction. Conclusions. The existence of clinical features compatible with cervical myelopathy and an anodyne cervical resonance justify the need to perform medullar arteriography owing to a suspected vascular malformation. An early diagnosis of a dural fistula and its proper treatment lead to a diminished morbidity and mortality rate in these patients (AU)