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2.
Neurocirugia (Astur) ; 22(6): 567-73, 2011 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-22167288

RESUMO

INTRODUCTION: Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn't metastasize. It causes symptoms of Meniere's syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation. CASE REPORT: A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour. DISCUSSION: In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function.


Assuntos
Neoplasias da Orelha/patologia , Saco Endolinfático/patologia , Adolescente , Diagnóstico Diferencial , Neoplasias da Orelha/complicações , Neoplasias da Orelha/etiologia , Neoplasias da Orelha/cirurgia , Humanos , Masculino , Doença de Meniere/etiologia , Resultado do Tratamento , Doença de von Hippel-Lindau/complicações
3.
Neurocir. - Soc. Luso-Esp. Neurocir ; 22(6): 563-573, nov.-dic. 2011. ilus
Artigo em Espanhol | IBECS | ID: ibc-104743

RESUMO

Introducción. El adenocarcinoma papilar de saco endolinfático se asocia a la enfermedad de Von Hippel Lindau en el 15% de los casos, tiene un crecimiento lento pero elevada agresividad local, y no metastatiza. Clínicamente produce un Síndrome de Menière derivado de la compresión que provoca en el conducto endolinfático. Cuando debuta con pérdida de audición suele ser de forma brusca e irreversible. Se diagnostica con técnicas de imagen y el análisis de su estructura con inmunohistoquímica. Su tratamiento electivo es la exéresis quirúrgica, y su principal complicación el sangrado perioperatorio, que se puede evitar con embolización o radiación estereotáctica preoperatorio.Caso clínico. Presentamos un caso de un tumor de saco endolinfático en un paciente de 17 años aquejado de sordera unilateral y crisis de vértigo rotatorio, con antecedentes familiares de enfermedad de Von Hippel Lindau. Las pruebas complementarias mostraron una hipoacusia neurosensorial y una arreflexia vestibular derechas. En tomografía computarizada de peñascos se apreciaba una lesión en el acueducto vestibular. Se sometió al paciente a una petrosectomía con abordaje presigmoideo y preservación de laberinto, realizándose una exéresis total de la lesión. Se diagnosticó de tumor del saco endolinfático en el análisis anatomopatológico.Discusión. Ante un paciente con antecedentes familiares de enfermedad de Von Hippel Lindau y un cuadro clí-nico de vértigo incluso sin hipoacusia, o siendo esta leve, habría que pensar en la presencia de un tumor del saco endolinfático. La presentación clínica de sordera puede ser brusca e irreversible, incluso con imágenes negativas o poco concluyentes, por lo que una rápida actuación es importante para la preservación de esta función (AU)


Introduction. Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn’t metastasize. It causes symptoms of Meniere’s syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation.Case report. A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour.Discussion. In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function (AU)


Assuntos
Humanos , Masculino , Adolescente , Saco Endolinfático/patologia , Doença de von Hippel-Lindau/patologia , Tomografia Computadorizada por Raios X , Aqueduto Vestibular/patologia , Vertigem/etiologia , Perda Auditiva Neurossensorial/etiologia
4.
Rev Neurol ; 52(5): 275-82, 2011 Mar 01.
Artigo em Espanhol | MEDLINE | ID: mdl-21341222

RESUMO

INTRODUCTION AND AIM: Radiosurgery is among the treatment options for patients with vestibular schwannoma. We present the experience in our institution in the treatment of this disease with this technique. PATIENTS AND METHODS: A retrospective study was made including 20 patients (11 women and 9 men; median age: 55.15 years-old) with vestibular schwannoma who received linear accelerator radiosurgery treatment since April 2005 until December 2008. Follow-up period was between 12 and 42 months, considering clinical examination of cranial nerves VII (House-Brackmann scale) and VIII (Gardner-Robertson scale) as well as radiological findings (considering tumor volume). For statistical analysis, the Fisher's exact test and logistic regression test were used. RESULTS: Certain worsening of hearing function was present in 25% of the patients. Five patients had large tumors at the moment of the treatment (equal or larger than 3.5 cm3), from which four deteriorated from headache, unsteady gait, dizziness/vertigo, facial numbness and tinnitus, with statistical significance (p < 0.05). From the first year of treatment on, there was a tumor volume decrease tendency, with no tumor growth in the medium/long term follow-up, achieving a local control rate of 100%. CONCLUSIONS: Radiosurgery has become an alternative in the treatment of patients with vestibular schwannoma of appropriate size, with high safety level, using low radiation doses, low rate of complications and good tumor control rate in the medium term follow-up.


Assuntos
Neuroma Acústico/cirurgia , Radiocirurgia/métodos , Adulto , Idoso , Nervo Facial/fisiologia , Nervo Facial/fisiopatologia , Nervo Facial/cirurgia , Feminino , Audição/fisiologia , Testes Auditivos , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/patologia , Estudos Retrospectivos , Resultado do Tratamento , Nervo Vestibulococlear/fisiologia , Nervo Vestibulococlear/fisiopatologia , Nervo Vestibulococlear/cirurgia
6.
Rev Neurol ; 49(6): 300-6, 2009.
Artigo em Espanhol | MEDLINE | ID: mdl-19728276

RESUMO

INTRODUCTION AND AIMS: One of the therapeutic options for chronic adult hydrocephalus that has become widely used in our service is the lumboperitoneal shunt with low-pressure Spetzler catheter and in an outpatient regimen. We report on the first 30 patients treated in this way with a follow-up of between one and five years. PATIENTS AND METHODS: Diagnosis was reached after studying the patient history and a clinical examination; Hakim and Adams' triad was found to be a primary and highly predictive factor, together with flow magnetic resonance imaging and the use of the ambulatory tap test. Patients who responded to the latter were submitted to placement of a shunt in a short operation performed with local anaesthetic and sedation. An evaluation of the three symptoms was carried out before and after the intervention according to a mixed scale (Rankin-Stein and Langfitt-Vall d'Hebron) and graphic tests based on the minimental test. RESULTS: At three months, 21 patients (70%) had improved and 14 of them (65%) maintained this improvement at three years; eight of these have been monitored for four and five years. Five patients died due to intercurrent illnesses. CONCLUSIONS: After comparing the complications that occurred with other case mixes involving both lumboperitoneal and ventriculoperitoneal shunts, we discuss the usefulness of this method (which is preferred by patients and relatives) in comparison to ventriculoperitoneal shunts, where the complications are more severe because they invade the brain and due to the need for general anaesthesia and longer hospitalisation. In addition, the method is more economical than the alternatives.


Assuntos
Procedimentos Cirúrgicos Ambulatórios , Hidrocefalia/cirurgia , Derivação Ventriculoperitoneal/métodos , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Feminino , Seguimentos , Humanos , Região Lombossacral , Masculino , Pessoa de Meia-Idade , Peritônio
7.
Rev. neurol. (Ed. impr.) ; 49(6): 300-306, 15 sept., 2009. ilus, graf, tab
Artigo em Espanhol | IBECS | ID: ibc-72682

RESUMO

Introducción y objetivo. Dentro de las opciones terapéuticas de la hidrocefalia crónica del adulto, ha ido imponiéndoseen nuestro servicio la derivación lumboperitoneal con catéter de baja presión de Spetzler y en régimen ambulatorio.Presentamos los primeros 30 pacientes así tratados con un seguimiento de entre uno y cinco años. Pacientes y métodos. Realizamosel diagnóstico mediante la anamnesis y exploración clínica, constatando la tríada de Hakim y Adams como factor primordialy de gran valor predictivo, así como la resonancia magnética de flujo y el uso de la punción lumbar de prueba (taptest) ambulatoria. Los pacientes que respondieron a ésta fueron sometidos al implante del shunt en una breve intervención conanestesia local y sedación. Se realizó antes y después una valoración de los tres síntomas según una escala mixta (Rankin-Stein y Langfitt-Vall d’Hebron) y pruebas gráficas basadas en el test minimental. Resultados. A los tres meses habían mejorado21 pacientes (70%) y mantuvieron la mejoría a los tres años 14 pacientes (65%); ocho de éstos han sido controlados cuatroy cinco años. Cinco pacientes fallecieron por enfermedades intercurrentes. Conclusiones. Tras compararse las complicacioneshabidas con otras casuísticas tanto de derivación lumboperitoneal como de derivación ventriculoperitoneal, se comentala utilidad del método, preferido por los pacientes y familiares, frente al de derivación ventriculoperitoneal, donde las complicacionestienen mayor gravedad al invadir el cerebro y ante la necesidad de anestesia general e ingreso más prolongado.El método, además, es más económico que los alternativos(AU)


Introduction and aims. One of the therapeutic options for chronic adult hydrocephalus that has become widely usedin our service is the lumboperitoneal shunt with low-pressure Spetzler catheter and in an outpatient regimen. We report on thefirst 30 patients treated in this way with a follow-up of between one and five years. Patients and methods. Diagnosis wasreached after studying the patient history and a clinical examination; Hakim and Adams’ triad was found to be a primary andhighly predictive factor, together with flow magnetic resonance imaging and the use of the ambulatory tap test. Patients whoresponded to the latter were submitted to placement of a shunt in a short operation performed with local anaesthetic andsedation. An evaluation of the three symptoms was carried out before and after the intervention according to a mixed scale(Rankin-Stein and Langfitt-Vall d’Hebron) and graphic tests based on the minimental test. Results. At three months, 21patients (70%) had improved and 14 of them (65%) maintained this improvement at three years; eight of these have beenmonitored for four and five years. Five patients died due to intercurrent illnesses. Conclusions. After comparing thecomplications that occurred with other case mixes involving both lumboperitoneal and ventriculoperitoneal shunts, we discussthe usefulness of this method (which is preferred by patients and relatives) in comparison to ventriculoperitoneal shunts, wherethe complications are more severe because they invade the brain and due to the need for general anaesthesia and longerhospitalisation. In addition, the method is more economical than the alternatives(AU)


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Hidrocefalia/diagnóstico , Hidrocefalia/cirurgia , Procedimentos Cirúrgicos Ambulatórios/métodos , Demência/complicações , Demência/diagnóstico , Anestesia Local/métodos , Hidrocefalia/fisiopatologia , Hidrocefalia , Anamnese/métodos
8.
Neurocirugia (Astur) ; 20(2): 152-8, 2009 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-19448959

RESUMO

Skull defects and even meningeal defects are responsible for the majority of pneumocephalus cases. Sometimes, several factors can produce intracranial gas under pressure (tension pneumocephalus) with severe neurological impairment. We present a case of a 66 year old woman with a double ventriculo-peritoneal shunt that was admitted to the emergency service with an intracranial hypertension syndrome. A scalp wound was found on the physical examination and a brain CT showed a tension pneumocephalus. The scalp wound was just located on the border of a cranioplasty made after surgical removal of a parasagital meningioma eight years ago. Evacuation of pneumocephalus, reparation of cranial and meningeal defects and modification of factors contributing to the tension pneumocephalus (ventricular-peritoneal shunts programmed to low pressure) were performed. When we found a patient with a tension pneumocephalus we must think not only about a skull or meningeal defect but also look for other elements that produce gas inside skull under pressure (shunts, cerebrospinal fluid leak between others). Therefore, therapeutic approach has three parts: pneumocephalus drainage, to find and repair entrance of gas and to modify factors that turn pneumocephalus in a tension pneumocephalus.


Assuntos
Traumatismos Cranianos Fechados/complicações , Pneumocefalia/etiologia , Couro Cabeludo/lesões , Derivação Ventriculoperitoneal/efeitos adversos , Idoso , Feminino , Traumatismos Cranianos Fechados/patologia , Humanos
9.
Neurocir. - Soc. Luso-Esp. Neurocir ; 20(2): 152-158, mar.-abr. 2009. tab, ilus
Artigo em Espanhol | IBECS | ID: ibc-60965

RESUMO

El neumoencéfalo se produce la mayoría de lasveces por una solución de continuidad en el cráneo eincluso en las meninges. En ocasiones, ciertos factorespueden hacer que éste adopte las características deun neumoencéfalo a tensión provocando una clínicaneurológica severa. Presentamos el caso de una pacientede 66 años portadora de una doble válvula de derivaciónventrículo-peritoneal que acude a Urgencias con uncuadro de hipertensión intracraneal y que tras la exploraciónfísica y la tomografía computarizada se detectauna herida en cuero cabelludo y un neumoencéfalo atensión. Este defecto en piel se hallaba justo en la zonadel borde de una plastia craneal colocada tras la resecciónde un meningoma parasagital 8 años antes. Se realizódrenaje, reparación de la solución de continuidady modificación de los factores que agravaban el cuadrodel neumoencéfalo (la presencia de unas válvulas dederivación con presiones de salida muy bajas).La presencia de un neumoencéfalo a tensión debehacernos pensar en encontrar no sólo el punto deacceso del aire al interior del cráneo sino también lascausas que han favorecido que el neumoencéfalo adoptecaracterísticas de alta presión (sistemas de derivacióno fístulas de LCR entre otros). De esta forma la orientaciónterapéutica adecuada tiene que ir destinada a lamodificación de estos factores agravantes, además dehacia el drenaje del neumoencéfalo y cierre del defectocraneal (AU)


Skull defects and even meningeal defects are responsiblefor the majority of pneumocephalus cases.Sometimes, several factors can produce intracranialgas under pressure (tension pneumocephalus) withsevere neurological impairment. We present a case of a66 year old woman with a double ventriculo-peritonealshunt that was admitted to the emergency service withan intracranial hypertension syndrome. A scalp woundwas found on the physical examination and a brain CTshowed a tension pneumocephalus. The scalp woundwas just located on the border of a cranioplasty madeafter surgical removal of a parasagital meningiomaeight years ago. Evacuation of pneumocephalus, reparationof cranial and meningeal defects and modificationof factors contributing to the tension pneumocephalus(ventricular-peritoneal shunts programmed to lowpressure) were performed.When we found a patient with a tension pneumocephaluswe must think not only about a skull or meningealdefect but also look for other elements that producegas inside skull under pressure (shunts, cerebrospinalfluid leak between others). Therefore, therapeuticapproach has three parts: pneumocephalus drainage,to find and repair entrance of gas and to modify factorsthat turn pneumocephalus in a tension pneumocephalus (AU)


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Derivação Ventriculoperitoneal/efeitos adversos , Ferimentos e Lesões , Pneumocefalia/diagnóstico , Pneumocefalia/etiologia
10.
Rev Neurol ; 47(6): 310-4, 2008.
Artigo em Espanhol | MEDLINE | ID: mdl-18803159

RESUMO

INTRODUCTION: High-grade malignant gliomas have a high mortality. Stereotactic biopsy is the gold standard technique to obtain diagnostic information about this kind of tumours but it can be dangerous. New technologies as the magnetic resonance spectroscopy (MRS) are an alternative option. AIM: We suggest the possibility of replacing stereotactic biopsy for new imaging technologies in patients with high-grade malignant gliomas and a very limited life expectancy due to tumoral irresectability, among other factors. We systematically analyze the literature checking the diagnostic accuracy and complications of stereotactic biopsy as well as of new technologies like the MRS. DEVELOPMENT: The average morbidity of stereotactic biopsy is 3.2% and the average mortality is 0.83%. The percentage of cases with a diagnosis (diagnostic yield) is 96%, but accuracy of that diagnosis is 79%. As regards MRS, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for identifying high-grade gliomas are 89.8, 88.2, 95.3 and 79.7%, respectively. CONCLUSIONS: Stereotactic biopsy presents a quite high morbi-mortality with a real accuracy diagnostic of 75.8% after considering the diagnostic yield. With regard to MRS, there are very few studies about accuracy diagnostic but, however; there are many about sensitivity, specificity, PPV and NPV, being these last values very high. In spite of that, we cannot conclude that stereotactic biopsy can be substituted for the MRS because there are not enough studies to support this conclusion.


Assuntos
Biópsia/métodos , Glioma , Neoplasias do Sistema Nervoso , Análise Espectral/métodos , Técnicas Estereotáxicas , Glioma/diagnóstico , Glioma/patologia , Humanos , Neoplasias do Sistema Nervoso/diagnóstico , Neoplasias do Sistema Nervoso/patologia , Valor Preditivo dos Testes , Sensibilidade e Especificidade
11.
Rev. neurol. (Ed. impr.) ; 47(6): 310-314, 16 sept., 2008. tab
Artigo em Es | IBECS | ID: ibc-69903

RESUMO

Introducción. Los gliomas de alto grado de malignidad se caracterizan por una alta mortalidad. La biopsia estereotáctica es la prueba de referencia para alcanzar un diagnóstico, pero puede llegar a ser peligrosa. Una alternativa diagnóstica es la espectroscopia por resonancia magnética (ERM). Objetivo. Sugerimos la posibilidad de sustituir la biopsia estereotáctica por nuevas pruebas de imagen en pacientes con una corta esperanza de vida por tumores de alto grado de malignidad irresecables, entre otros factores. Para ello analizamos de forma sistemática la agudeza diagnóstica, tanto de la biopsia estereotácticacomo de la ERM. Desarrollo. La morbilidad media de la biopsia estereotáctica es del 3,2%, y la mortalidad del0,83%. El porcentaje de casos en los que se alcanza un diagnóstico (rendimiento diagnóstico) es del 96%, pero la agudeza de ese diagnóstico es del 79%. Con respecto a la ERM, la sensibilidad, especificidad y los valores predictivos positivos (VPP) y negativos (VPN) para la detección de gliomas de alto grado son del 89,8, 88,2, 95,3 y 79,7%, respectivamente. Conclusiones. La biopsia estereotáctica presenta una alta morbimortalidad, con una agudeza diagnóstica tras considerar el rendimiento diagnóstico del 75,8%. Con respecto a la ERM, existen muy pocos estudios sobre agudeza diagnóstica, pero muchos sobre sensibilidad, especificidad, VPP y VPN, y estos valores son muy altos. A pesar de estos resultados, no podemos concluir quela biopsia estereotáctica sea sustituida por la ERM, ya que no existen suficientes estudios que apoyen esta conclusión


Introduction. High-grade malignant gliomas have a high mortality. Stereotactic biopsy is the gold standard technique to obtain diagnostic information about this kind of tumours but it can be dangerous. New technologies as the magnetic resonance spectroscopy (MRS) are an alternative option. Aim. We suggest the possibility of replacing stereotactic biopsy for new imaging technologies in patients with high-grade malignant gliomas and a very limited life expectancy due to tumoral irresectability, among other factors. We systematically analyze the literature checking the diagnostic accuracy and complications of stereotactic biopsy as well as of new technologies like the MRS. Development. The average morbidity of stereotactic biopsy is 3.2% and the average mortality is 0.83%. The percentage of cases with a diagnosis (diagnostic yield) is 96%, but accuracyof that diagnosis is 79%. As regards MRS, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for identifying high-grade gliomas are 89.8, 88.2, 95.3 and 79.7%, respectively. Conclusions. Stereotactic biopsy presents a quite high morbi-mortality with a real accuracy diagnostic of 75.8% after considering the diagnostic yield. Withregard to MRS, there are very few studies about accuracy diagnostic but, however; there are many about sensitivity, specificity, PPV and NPV, being these last values very high. In spite of that, we cannot conclude that stereotactic biopsy can be substituted for the MRS because there are not enough studies to support this conclusion


Assuntos
Humanos , Técnicas Estereotáxicas , Biópsia/métodos , Análise Espectral/métodos , Glioma/diagnóstico , Neoplasias Encefálicas/diagnóstico , Espectroscopia de Ressonância Magnética/métodos , Sensibilidade e Especificidade
13.
Brain Res ; 869(1-2): 244-50, 2000 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-10865083

RESUMO

The subventricular zone (SVZ) of the adult mouse brain retains the capacity to generate new neurons from stem cells. The neuronal precursors migrate tangentially along the rostral migratory stream (RMS) towards the olfactory bulb, where they differentiate as periglomerular and granular interneurons. In this study, we have investigated whether nitric oxide (NO), a signaling molecule in the nervous system with a role in embryonic neurogenesis, may be produced in the proximity of the progenitor cells in the adult brain, as a prerequisite to proposing a functional role for NO in adult neurogenesis. Proliferating and immature precursor cells were identified by immunohistochemistry for bromo-deoxyuridine (BrdU) and PSA-NCAM, respectively, and nitrergic neurons by either NADPH-diaphorase staining or immunohistochemical detection of neuronal NO synthase (NOS I). Nitrergic neurons with long varicose processes were found in the SVZ, intermingled with chains of cells expressing PSA-NCAM or containing BrdU. Neurons with similar characteristics surrounded the RMS all along its caudo-rostral extension as far as the core of the olfactory bulb. No expression of NOS I by precursor cells was detected either in the proliferation or in the migration zones. Within the olfactory bulb, many small cells in the granular layer and around the glomeruli expressed either PSA-NCAM or NOS I and, in some cases, both markers. Colocalization was also found in a few isolated cells at a certain distance from the neurogenesis areas. The anatomical disposition shown indicates that NO may be released close enough to the neuronal progenitors to allow a functional influence of this messenger in adult neurogenesis.


Assuntos
Encéfalo/crescimento & desenvolvimento , Diferenciação Celular/fisiologia , Divisão Celular/fisiologia , Molécula L1 de Adesão de Célula Nervosa , Neurônios/metabolismo , Óxido Nítrico/metabolismo , Células-Tronco/metabolismo , Animais , Encéfalo/citologia , Encéfalo/metabolismo , Movimento Celular/fisiologia , Masculino , Camundongos , Camundongos Endogâmicos , Moléculas de Adesão de Célula Nervosa/metabolismo , Neurônios/citologia , Bulbo Olfatório/citologia , Bulbo Olfatório/crescimento & desenvolvimento , Bulbo Olfatório/metabolismo , Ácidos Siálicos/metabolismo , Células-Tronco/citologia
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