[Bromocriptine: could it be the cure for post-surgical akinetic mutism?] / Bromocriptina: podria ser la cura para el mutismo acinetico posquirurgico?

INTRODUCTION: Akinetic mutism is considered as an alteration of the motivational state of the person, which the patient is unable to initiate verbal or motor responses voluntary, even with preserved sensorimotor and surveillance functions. CASE REPORT: A 43 year-old male involved in a cerebellum arteriovenous fistula complicated with hydrocephalus, who responded dramatically to treatment with bromocriptine. CONCLUSION: Typically, akinetic mutism is described as a transient surgeries posterior fossa. However, it can also occur after multiple valvular failure in patients with hydrocephalus.

TITLE: Bromocriptina: podria ser la cura para el mutismo acinetico posquirurgico?Introduccion. El mutismo acinetico se considera una alteracion del estado motivacional de la persona, por el cual el paciente es incapaz de iniciar respuestas verbales o motoras de caracter voluntario, aun teniendo preservadas las funciones sensomotoras y de vigilancia. Caso clinico. Varon de 43 años, intervenido de una fistula arteriovenosa del cerebelo complicada con hidrocefalia, que respondio espectacularmente al tratamiento con bromocriptina. Conclusion. Tipicamente se ha descrito el mutismo acinetico como una complicacion transitoria de las cirugias de la fosa posterior. Sin embargo, tambien puede aparecer tras multiples fallos valvulares en pacientes con hidrocefalia.

[Giant cavernous sinus haemangioma. Case report]. / Hemangioma gigante del seno cavernoso. Caso clínico.

INTRODUCTION: Intracranial haemangioma is a benign vascular tumor which seldom affects the cavernous sinus region, being it frequently misdiagnosed as a meningioma. CASE REPORT: A 60-year-old woman presented with a subacute-onset third cranial nerve palsy. A giant extraaxial mass located in the middle cranial fossa which extended into the selar and supraselar areas was diagnosed. It showed homogeneous enhancement after contrast administration. The angiography revealed mild enhancement as well as indirect signs of mass effect, and a tumoral embolization was performed. Surgery and outcome. The tumor was partially removed through a left pterional approach, leaving an intraselar remnant. The patient had an uneventful postoperative course, and did not present new neurological deficits. CONCLUSIONS: The haemangioma must be considered in the differential diagnosis of cavernous sinus tumours. This entity represents a neurosurgical challenge, due to the complexity of this anatomical region and the trend of the tumour to bleed during its dissection.

Hemangioma gigante del seno cavernoso. Caso clínico / Giant cavernous sinus haemangioma. Case report

Introducción. El hemangioma intracraneal es unatumoración vascular benigna que raramente afecta alseno cavernoso, por lo que es frecuentemente diagnosticadode forma errónea como meningioma.Caso clínico. Presentamos el caso de una mujer de 60años que debutó con un cuadro de parálisis subagudadel tercer par craneal izquierdo. En neuroimagen, seevidenció una masa extraaxial localizada en la fosacraneal media, con extensión a las regiones selar ysupraselar, e intenso realce homogéneo tras la administraciónde contraste. La arteriografía mostró discretorealce en fase arterial y venosa, y efecto de masa. Serealizó una embolización del tronco meningohipofisarioizquierdo.Intervención y evolución. Se practicó una resecciónsubtotal del tumor mediante un abordaje pterionalizquierdo, persistiendo un pequeño resto intraselar. Eldiagnóstico anatomopatológico fue hemangioma. Trasla intervención la paciente no presentó nuevos déficitsneurológicos.Conclusión. El hemangioma debe ser tenido en consideraciónal realizar el diagnóstico diferencial de lostumores que afectan al seno cavernoso. Su tratamientoconstituye un reto quirúrgico para el neurocirujano,dada la complejidad de esta región anatómica y la grantendencia del tumor a sangrar durante su disección (AU)

Introduction. Intracranial haemangioma is a benignvascular tumor which seldom affects the cavernoussinus region, being it frequently misdiagnosed as ameningioma.Case report. A 60-year-old woman presented witha subacute-onset third cranial nerve palsy. A giantextraaxial mass located in the middle cranial fossawhich extended into the selar and supraselar areas wasdiagnosed. It showed homogeneous enhancement aftercontrast administration. The angiography revealedmild enhancement as well as indirect signs of masseffect, and a tumoral embolization was performed. Surgeryand outcome. The tumor was partially removedthrough a left pterional approach, leaving an intraselarremnant. The patient had an uneventful postoperativecourse, and did not present new neurological deficits.Conclusions. The haemangioma must be consideredin the differential diagnosis of cavernous sinus tumours.This entity represents a neurosurgical challenge, due tothe complexity of this anatomical region and the trendof the tumour to bleed during its dissection (AU)

Hemangioblastoma quístico de la unión bulbomedular asociado a enfermedad de von Hippel-Lindau. Presentación de dos casos y revisión de la bibliografía / Cystic hemangioblastoma of the junction of the medulla and upper spinal cord associated to von hippel-lindau disease. Two case reports and a review of the literature

Introducción. Los hemangioblastomas son tumores benignos muy vascularizados que pueden presentarse de forma esporádica o bien asociados al síndrome de von Hippel-Lindau en un 20% de los casos. Sólo un 5-20% de los hemangioblastomas se localizan en el tronco cerebral, y los de la unión bulbomedular son los diagnosticados con menor frecuencia. Casos clínicos. Dos pacientes jóvenes fueron diagnosticados de enfermedad de von Hippel-Lindau tras comenzar con un hemangioblastoma quístico de la unión bulbomedular como primera manifestación patológica. Ambos fueron intervenidos mediante craneotomía suboccipital, y se les extirpó completamente ambas lesiones sin presentar déficit neurológicos posquirúrgicos. Uno de los pacientes requirió una reintervención años después por una nueva recidiva de hemangioblastoma sintomático en la médula dorsal. El estudio genético llevado a cabo en ambos pacientes confirmó la presencia de una mutación en el gen VHL, responsable del cuadro sindrómico que padecían. Conclusión. La presencia de hemangioblastomas quísticos de la unión bulbomedular asociados a la enfermedad de von Hippel-Lindau es muy infrecuente. A pesar de la dificultad técnica existente para su extirpación, la incorporación de las últimas técnicas de neuroimagen y microcirugía ha permitido una reducción significativa de la morbimortalidad asociada al tratamiento quirúrgico de estos pacientes (AU)

Introduction. Hemangioblastomas are very highly vascularised benign tumours that can present either sporadically or in association with von Hippel-Lindau syndrome in 20% of cases. Only 5-20% of hemangioblastomas are located in the brainstem, and those that occur at the junction of the medulla and upper spinal cord are the ones that are less commonly diagnosed. Case report. Two young patients were diagnosed with von Hippel-Lindau disease after beginning with a cystic hemangioblastoma at the junction of the medulla and upper spinal cord as the first pathological manifestation. Both of them underwent a suboccipital craniotomy and the two lesions were completely removed without any kind of post-operative neurological deficits. One of the patients required a reintervention some years later due to a new recurrence of a symptomatic hemangioblastoma in the dorsal medulla. The genetic study conducted in both patients confirmed the presence of a mutation in the VHL gene, which is responsible for the syndrome they were suffering from. Conclusions. The presence of cystic hemangioblastomas of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease is very rare. Despite the technical difficulties involved in removing them, the incorporation of the latest neuroimaging and microsurgery techniques has made it possible to significantly reduce the morbidity and mortality rate associated to the surgical treatment of such patients (AU)

[Cystic hemangioblastoma of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease. Two case reports and a review of the literature]. / Hemangioblastoma quistico de la union bulbomedular asociado a enfermedad de von Hippel-Lindau. Presentacion de dos casos y revision de la bibliografia.

INTRODUCTION: Hemangioblastomas are very highly vascularised benign tumours that can present either sporadically or in association with von Hippel-Lindau syndrome in 20% of cases. Only 5-20% of hemangioblastomas are located in the brainstem, and those that occur at the junction of the medulla and upper spinal cord are the ones that are less commonly diagnosed. CASE REPORTS: Two young patients were diagnosed with von Hippel-Lindau disease after beginning with a cystic hemangioblastoma at the junction of the medulla and upper spinal cord as the first pathological manifestation. Both of them underwent a suboccipital craniotomy and the two lesions were completely removed without any kind of post-operative neurological deficits. One of the patients required a reintervention some years later due to a new recurrence of a symptomatic hemangioblastoma in the dorsal medulla. The genetic study conducted in both patients confirmed the presence of a mutation in the VHL gene, which is responsible for the syndrome they were suffering from. CONCLUSIONS: The presence of cystic hemangioblastomas of the junction of the medulla and upper spinal cord associated to von Hippel-Lindau disease is very rare. Despite the technical difficulties involved in removing them, the incorporation of the latest neuroimaging and microsurgery techniques has made it possible to significantly reduce the morbidity and mortality rate associated to the surgical treatment of such patients.

[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.

[Refractory epilepsy as the presenting symptom of a brucellar brain abscess]. / Epilepsia refractaria como presentacioón de absceso cerebral brucelósico.

INTRODUCTION: Brucellosis is a zoonotic disease that is occasionally transmitted to human beings from infected animal reservoirs. It is an important condition in endemic areas. One infrequent complication of systemic brucellosis is the infection of the central or the peripheral nervous systems. CASE REPORT: A 54-year-old male who was being studied prior to surgery for refractory epilepsy, with clinical expression in the form of complex partial seizures. Neuroimaging findings revealed an expansive lesion in the right temporal lobe, which direct serological, histopathological and microbiological evidence showed to be a chronic brucellar abscess. After combined treatment involving complete surgical resection followed by a cycle of standard antimicrobial therapy, the patient was seizure-free at one year of follow-up. CONCLUSIONS: Despite its low frequency, infection by Brucella must be considered in the differential diagnosis of intracranial expansive lesions, as well as in the case of patients whose presenting symptoms are epileptic seizures. To perform the diagnosis it is especially important to be aware of the wide range of clinical and radiological manifestations that can be produced, and which do not always correlate. Identification of risk factors on the patient record is also a crucial step.

Epilepsia refractaria como presentación de absceso cerebral brucelósico / Refractory epilepsy as the presenting symptom of a brucellar brain abscess

Introducción. La brucelosis es una enfermedad zoonósicaque se transmite de forma ocasional al ser humano desde reservoriosanimales infectados. Representa una entidad de relevanciaen áreas endémicas. Una complicación poco frecuente de la brucelosissistémica es la infección del sistema nervioso, tanto centralcomo periférico. Caso clínico. Varón de 54 años en estudio prequirúrgicode epilepsia refractaria, con expresión clínica en forma decrisis parciales complejas, en el cual se demostró mediante técnicasde neuroimagen una lesión expansiva en el lóbulo temporal derecho,filiada como absceso crónico brucelósico mediante demostraciónserológica, histopatológica y microbiológica directa. Tras eltratamiento combinado mediante resección quirúrgica completa,seguido de un ciclo de tratamiento antimicrobiano, el paciente quedólibre de crisis tras un año de seguimiento. Conclusiones. A pesarde su baja frecuencia, la infección por Brucella debe considerarseen el diagnóstico diferencial de las lesiones expansivas intracraneales,así como en los pacientes cuya forma de presentación son lascrisis epilépticas. Para la realización del diagnóstico es de especialimportancia el conocimiento de la amplia variedad de manifestacionesclínicas y radiológicas a las que puede dar lugar, y que nosiempre encuentran correlato, así como la identificación de factoresde riesgo en la historia clínica

Introduction. Brucellosis is a zoonotic disease that is occasionally transmitted to human beings from infectedanimal reservoirs. It is an important condition in endemic areas. One infrequent complication of systemic brucellosis is theinfection of the central or the peripheral nervous systems. Case report. A 54-year-old male who was being studied prior tosurgery for refractory epilepsy, with clinical expression in the form of complex partial seizures. Neuroimaging findingsrevealed an expansive lesion in the right temporal lobe, which direct serological, histopathological and microbiologicalevidence showed to be a chronic brucellar abscess. After combined treatment involving complete surgical resection followedby a cycle of standard antimicrobial therapy, the patient was seizure-free at one year of follow-up. Conclusions. Despite itslow frequency, infection by Brucella must be considered in the differential diagnosis of intracranial expansive lesions, as wellas in the case of patients whose presenting symptoms are epileptic seizures. To perform the diagnosis it is especially importantto be aware of the wide range of clinical and radiological manifestations that can be produced, and which do not alwayscorrelate. Identification of risk factors on the patient record is also a crucial step

[Treatment of a case of essential tremor with subthalamic stimulation]. / Tratamiento de un caso de temblor esencial con estimulacion subtalamica.

INTRODUCTION: Chronic stimulation of the subthalamic nucleus (STN) has proved itself to be useful in treating Parkinson's disease and especially in dealing with the tremor suffered by patients. Yet there is very little experience to support the use of STN as an alternative therapy in non Parkinsonian tremors. CASE REPORT: Our study considered the case of a patient who had been diagnosed as suffering from drug resistant essential tremor which was predominant in the distal region of the upper right limb and was treated by unilateral stimulation of the STN. RESULTS: The patient's clinical state improved significantly from the first weeks onwards. After a year and a half of therapy, the patient had achieved a stable improvement of 82.4% on the tremor scale and medication was reduced by 41.7%. CONCLUSIONS: SNT stimulation appears as a suitable target for the treatment of drug resistant essential tremor.

[Treatment of a case of generalised dystonia using subthalamic stimulation]. / Tratamiento de un caso de distonía generalizada mediante estimulación subtalámica.

INTRODUCTION: Generalised dystonia is an entity that does not usually respond well to medical treatment. Different surgical targets have therefore been used in the treatment of dystonia, including several thalamic nuclei or the internal globus pallidus. The subthalamic nucleus plays a fundamental role in the physiology of the basal ganglia. It could therefore be considered to be a good potential target for stimulation. CASE REPORT: A patient who was confined to a wheelchair and who had not responded to a number of different medical treatment protocols or to a bilateral thalamotomy was treated with bilateral deep brain stimulation in the subthalamic nucleus. Tetrapolar electrodes were placed in both subthalamic nuclei in two stages. The patient showed a significant improvement from the very beginning of the post-operative period. After six months' progression, the patient was able to walk unaided and the dystonic seizures diminished significantly. Unfortunately, the patient died from choking. We used a bipolar stimulation protocol at 50 Hz with 210 micros pulses, which do not reach the levels of maximum charge density that are considered to be harmful. CONCLUSION: The subthalamic nucleus can be a good surgical target for deep brain stimulation in cases of generalised dystonia; it responds well to stimulation at intermediate frequencies with safe charge densities.

Tratamiento de un caso de distonía generalizada mediante estimulación subtalámica / Treatment of a case of generalised dystonia using subthalamic stimulation

Introducción. La distonía generalizada es una entidad que no suele responder bien al tratamiento médico. Por ello, se han empleado diferentes blancos quirúrgicos para su tratamiento, entre ellos diversos núcleos talámicos o el globo pálido interno. El núcleo subtalámico desempeña un papel fundamental en la fisiología de los ganglios basales. Por ello, podría considerarse como un buen blanco potencial para la estimulación. Caso clínico. Se trató con estimulación cerebral profunda bilateral en el núcleo subtalámico a un paciente postrado en silla de ruedas, que no había respondido a múltiples protocolos de tratamiento médico ni a una talamotomía bilateral. Se colocaron estimuladores en ambos núcleos subtalámicos, en dos tiempos. El paciente presentó una mejoría significativa, ya desde el período posoperatorio inmediato. Tras seis meses de evolución, el paciente era independiente para la marcha, y disminuyeron significativamente las crisis distónicas. Desgraciadamente, el paciente falleció a causa de un atragantamiento. Se empleó un protocolo de estimulación bipolar a 50 Hz, con pulsos de 210 micorseg, que no alcanzan los niveles de densidad máxima de carga que se consideran lesivos. Discusión. El núcleo subtalámico puede ser un buen blanco quirúrgico para la estimulación cerebral profunda en caso de distonía generalizada; responde bien a la estimulación a frecuencias intermedias con densidades de carga seguras (AU)

Introduction. Generalised dystonia is an entity that does not usually respond well to medical treatment. Different surgical targets have therefore been used in the treatment of dystonia, including several thalamic nuclei or the internal globus pallidus. The subthalamic nucleus plays a fundamental role in the physiology of the basal ganglia. It could therefore be considered to be a good potential target for stimulation. Case report. A patient who was confined to a wheelchair and who had not responded to a number of different medical treatment protocols or to a bilateral thalamotomy was treated with bilateral deep brain stimulation in the subthalamic nucleus. Tetrapolar electrodes were placed in both subthalamic nuclei in two stages. The patient showed a significant improvement from the very beginning of the post-operative period. After six months’ progression, the patient was able to walk unaided and the dystonic seizures diminished significantly. Unfortunately, the patient died from choking. We used a bipolar stimulation protocol at 50 Hz with 210 µs pulses, which do not reach the levels of maximum charge density that are considered to be harmful. Conclusion. The subthalamic nucleus can be a good surgical target for deep brain stimulation in cases of generalised dystonia; it responds well to stimulation at intermediate frequencies with safe charge densities (AU)

La moderna psicocirugía: un nuevo enfoque de la neurocirugía en la enfermedad psiquiátrica / Modern-day psychosurgery: a new approach to neurosurgery in psychiatric disease

Objetivo. Se pretende ofrecer una revisión actualizada de las diferentes posibilidades quirúrgicas en el manejo de ciertos trastornos psiquiátricos refractarios al tratamiento conservador (farmacoterapia, psicoterapia, terapia electroconvulsiva). Desarrollo. Para esta investigación, revisamos los trabajos publicados por los centros con mayor experiencia en esta cirugía, fundamentalmente en Norteamérica y Europa, desde sus inicios en los años 30, con la polémica leucotomía prefrontal, hasta la aparición de las modernas técnicas estereotáxicas. Se analizan las bases anatomofisiológicas, las principales indicaciones clínicas, las técnicas quirúrgicas utilizadas y los resultados, así como las perspectivas de futuro de este tratamiento neuroquirúrgico. Conclusiones. La evolución más destacable de la psicocirugía en los últimos años ha sido la combinación de una selección más rigurosa de los pacientes con la mayor especificidad del tratamiento efectuado sobre las estructuras cerebrales implicadas en la enfermedad psiquiátrica. Los procedimientos psicoquirúrgicos más empleados en la actualidad son la cingulotomía, la capsulotomía anterior, la tractotomía subcaudada, la leucotomía límbica y la hipotalamotomía posteromedial, con respuestas favorables en torno al 35-70 por ciento de los casos. Los diagnósticos psiquiátricos donde se pueden encontrar mejores resultados son el trastorno obsesivo-compulsivo, los estados de ansiedad crónica y la depresión mayor. Los avances actuales en las técnicas de neuroimagen, el mayor conocimiento neurofisiológico y las revolucionarias técnicas de neuromodulación, en especial la estimulación cerebral profunda, ofrecen expectativas todavía más prometedoras a la neurocirugía psiquiátrica (AU)

Aims. To obtain an up-to-date review of the different possible surgical approaches in the management of certain psychiatric disorders that are refractory to conservative treatment (pharmacotherapy, psychotherapy, electroconvulsive therapy). Method. In order to conduct this research we reviewed the work published by centres with the most experience in this type of surgery, mainly in North America and Europe, since its beginnings in the 1930s, with the controversy concerning prefrontal leucotomy, until the appearance of modern stereotactic techniques. We analyse the anatomophysiological bases, their main clinical indications, the surgical techniques used and their results, as well as perspectives for the future of this neurosurgical treatment. Conclusions. The most noteworthy progress in psychosurgery in recent years has been the combination of a more rigorous selection of patients and the higher degree of specificity with which treatment is performed on the brain structures involved in psychiatric disease. The most widely employed psychosurgical procedures at present are cingulotomy, anterior capsulotomy, subcaudate tractotomy, limbic leucotomy and postero-medial hypothalamotomy, with favourable responses in about 35-70% of cases. The psychiatric diagnoses where the best results are to be found are obsessive-compulsive disorder, chronic anxiety states and major depression. Current progress in neuroimaging techniques, increased neurophysiological knowledge and the revolutionary neuromodulation techniques, especially deep brain stimulation, offer an even more promising future for psychiatric neurosurgery (AU)

[Modern day psychosurgery: a new approach to neurosurgery in psychiatric disease]. / La moderna psicocirugía: un nuevo enfoque de la neurocirugía en la enfermedad psiquiátrica.

AIMS: To obtain an up to date review of the different possible surgical approaches in the management of certain psychiatric disorders that are refractory to conservative treatment (pharmacotherapy, psychotherapy, electroconvulsive therapy). METHOD: In order to conduct this research we reviewed the work published by centres with the most experience in this type of surgery, mainly in North America and Europe, since its beginnings in the 1930s, with the controversy concerning prefrontal leucotomy, until the appearance of modern stereotactic techniques. We analyse the anatomophysiological bases, their main clinical indications, the surgical techniques used and their results, as well as perspectives for the future of this neurosurgical treatment. CONCLUSIONS: The most noteworthy progress in psychosurgery in recent years has been the combination of a more rigorous selection of patients and the higher degree of specificity with which treatment is performed on the brain structures involved in psychiatric disease. The most widely employed psychosurgical procedures at present are cingulotomy, anterior capsulotomy, subcaudate tractotomy, limbic leucotomy and postero medial hypothalamotomy, with favourable responses in about 35 70% of cases. The psychiatric diagnoses where the best results are to be found are obsessive compulsive disorder, chronic anxiety states and major depression. Current progress in neuroimaging techniques, increased neurophysiological knowledge and the revolutionary neuromodulation techniques, especially deep brain stimulation, offer an even more promising future for psychiatric neurosurgery.

[Island of Reil and pharmacoresistance in epilepsy]. / Insula de Reil y epilepsia farmacorresistente.

INTRODUCTION: Over the last few years surgery has been developing, with promising results, methods to treat an important number of cases of partial epilepsy that are related, to different extents, with the lobe of the insula and display pharmacoresistance. Better knowledge of the anatomofunctional particularities of this region of the cortex, new neuroimaging and neurophysiological techniques, together with the use of the surgical microscope, stereotactic support and neuronavigation, have had a strong influence on the development of this type of surgery. CASE REPORT: In this paper we report three cases of patients with epilepsy and who were diagnosed as suffering from structural lesions of the insular region: two cavernomas and an oligodendroglioma. The preoperative study was carried out using magnetic resonance imaging, angiography and video EEG. The surgical procedure consisted in a transsylvian approach with electrocorticography, which provided us with the clinical results we have studied. CONCLUSIONS: The correct approach to the diagnosis of the epileptogenic insular lesions must include a complete neurophysiological study and preoperative planning with angiography. This allows their surgical resection through the transsylvian approach and enables us to obtain, with a very low degree of morbidity, clearly satisfactory results as regards the decrease in the number of seizures in these patients