F. Isamat

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) and its relation to the multiple hamartoma syndrome (Cowden disease)

SummaryThe clinical manifestations, surgical treatment and postoperative results of three patients with gangliocytomas of the cerebellum (Lhermitte-Duclos disease) are presented. Particular attention is placed in one of the cases, that of a young woman with a short clinical history of episodic symptoms of intracranial hypertension, dizziness and ataxia, with a concomitant frontal meningioma and in the general context of a multiple harmartoma syndrome (Cowden disease). The possible relationship between both diseases is contemplated, since they can be the extremes of a wide spectrum of a peculiar form of phakomatosis.

A golgi and electron microscopic study of a dysplastic gangliocytoma of the cerebellum

SummaryThe fine structure of a dysplastic gangliocytoma of the cerebellum is studied by means of the Golgi method and electron microscopic examination.Thick proximally unbranched dendrites with terminal arborizations and varicose influorescences in the form of a basket are stained with the Golgi method. Axons are always descendant to the inner myelinated layer of the redistributed cerebellar cortex, while ascendant collaterals are observed at the level of the outer myelinated layer.Clear and dense-core vesicles and synapses are common in the cellular profiles under electron microscopic examination. From these data and because of the lack of putative connections through the white matter, an organized, self-regulated, catecholamine-mediated complex may be postulated.

On posttraumatic intracavernous false aneurysms and arteriovenous fistulas

SummaryPosttraumatic high-flow communications between the intracavernous internal carotid artery (ICA) and the cavernous sinus may give rise to two different pathological entities. A connection between the ICA and one of the adjacent intracavernous veins will result in a carotico-cavernous fistula (CCF). A false aneurysm will develop if the arterialized rent from the injured ICA flows directly into the bare perivenous spaces within the cavernous sinus, without a shunt with one of the veins. A CCF and a false aneurysm can be present in the same patient. Angiographically these two entities may be demonstrated, and clinically have a somewhat different behaviour. Lifethreatening massive epistaxis is the most serious complication, particularly in false intracavernous aneurysms. Most high-flow CCFs are the result of a small side-wall laceration of the ICA, while large ICA injuries or even complete transection of the artery are responsible for the origin of false aneurysms.

Variabilidad clínica, radiológica e histológica del neurocitoma central. A propósito de nueve casos

Resumen Desde la descripcion inicial de Rassoun en 1982 del neurocitoma central (NC) como un tumor que se origina en el septum peIlucidum, afecta a individuos jovenes y cursa con un comportamiento benigno, numerosos casos han sido recogidos. Sin embargo, a medida que el conocimiento de esta entidad va en aumento se han identificado variantes de esta descripcion: debut en la edad adulta o en pacientes ancianos, origen fuera del ventriculo lateral y comportamientos biologicos agresivos. Presentamos nuestra experiencia con nueve casos, discutiendo sus peculiaridades clinicas, radiologicas y las dificultades quirurgicas en su tratamiento. Sin embargo, es en el estudio anatomopatologico del NC donde mas puntos oscuros quedan por resolver, sobre todo en lo que respecta su patogenesis, patron de inmunofiuorescencia y su relacion con otras neoplasias, como el neuroblastoma, el neuroblastoma diferenciado asi como algunos puntos de contacto, no precisamente marginales con tumores aparentemente dispares del NC como el tumor neuroectodermico disembrioplasico, el astrocitoma pilocitico y de un numero creciente de formas mixtas de NC con elementos gliales, rabdoides o lipomatosos. Dado que no existe una clara definicion ni diferenciacion entre estas entidades, una redefinicion de los tumores neuronales es imprescindible.

Neurosurgery: An act of contrition

Once upon a time there was a child who went to the Jesuits school of Barcelona. There he was told that the correct thing to do at the end of each week was to retrospectively evaluate right and wrong of the week gone. Right should be kept as a positive gain, but without excessive glorification. Wrong had to be analysed before adopting a final decision, since on occasions and when properly spiced up it could be highly beneficial. Only for the hopelessly wrong the answer was an act of contrition. It was certainly good advice, but there were plenty of far more fascinating things to do on a Saturday! The boy grew up, he went to medical school and later on became a neurosurgeon. And today he is addressing to you the seventeenth European Lecture of the European Association of Neurosurgical Societies (EANS). Mr. President, ladies and gentlemen, my dear friends. I am very much aware of the great distinction it is to be asked to give this Lecture. I am very honoured, indeed. But beyond going any further let me stand to give homage to the neurosurgeon who began the saga of all these Lectures, the inaugural European Lecturer, an outstanding man and a very dear friend, Douglas J. Miller. The EANS lost at the end of last August a modest giant, a solid scientist, a skillful surgeon and a much loved educator. I remember that my first duty as former President of our Association was to attend the European Course in Neurosurgery orga-

Meningiomas de línea media intracraneal. Aspectos clinicoterapéuticos

Resumen Presentamos 103 meningiomas confirmados histologicamente que se localizaban en la linea media intracraneal que fueron intervenidos quirurgicamente en nuestro servicio. Consideramos su localizacion, presentacion clinica, radiologia, tipo de cirugia practicada y evolucion posterior. La edad media de nuestra serie fueron 52 anos y el 70% de nuestros pacientes eran mujeres. Predominan los meningiomas parasagitales y constituyen el 40% del total. En esta localizacion, el 62% se situan en el tercio medio. Solo 1 paciente tenia un meningioma de linea media infratentorial a nivel de la convexidad cerebelosa. Se discuten los resultados obtenidos en terminos de morbilidad, mortalidad y secuelas psicofisicas, comparandolos a los de otras largas series en las que se incluyen meningiomas de cualquier localizacion intracraneal.

Controversias alrededor de los meningiomas de la región del seno cavernoso. Un análisis de la literatura y algunas conclusiones derivadas del manejo de 31 casos consecutivos

Resumen Entre 1988 Y1997 hemos intervenido en nuestro servicio 31 pacientes portadores de meningiomas que afectaban la region del seno cavernoso (SC) y los hemos clasificado en intrinsecos, entendiendo por tales los originados dentro de los confines anatomicos del SC y en paracavernosos, cuando el SC estaba invadido por meningiomas originados en regiones anatomicas adyacentes. La serie esta constituida por 11 pacientes con meningiomas intrinsecos y 20 meningiomas paracavernosos. Todos los pacientes fueron intervenidos mediante una craneotomia pterional asociada a una osteotomia orbitocigomatica o frontoorbitaria; en nueve casos, las extensiones del tumor a la region petroclival obligaron a realizar un abordaje combinado supra-infratentorial. En los meningiomas intrinsecos se consiguio una reseccion completa en dos casos (18%), mejoraron un 50% de las neuropatias craneales y se presentaron un 45% de nuevas neuropatias. En los meningiomas paracavernosos las resecciones completas fueron posibles en 11 casos (55%), mejoraron un 42% de neuropatias craneales y se presentaron un 30% de nuevos deficits. Con un seguimiento entre uno y nueve anos, cuatro pacientes presentaron progresion tumoral en focos de tumor residual y se identificaron un 7.5% de recidivas tumorales; ocho pacientes recibieron tratamiento complementario (4 radiocirugia estereotaxica). Del analisis de los puntos de controversia alrededor de los meningiomas del seno cavernoso (definicion, indicaciones y tipos de tratamiento, manejo de la carotida, pronostico de los pares craneales, manejo de la recidiva y de la progresion tumoral) podemos concluir en base a nuestra experiencia y segun la literatura disponible, que es preciso una definicion homogenea de esta entidad MSC que permita el analisis y comparacion de las series, asi como el uso de un sistema estandarizado de recogida de resultados en particular respecto a la funcion de los pares craneales. El tratamiento debe establecerse de forma individualizada y en aquellos casos con clinica progresiva o signos de compresion cerebral, mediante cirugia radical excepto en casos de carotida englobada donde seria preferible la cirugia subtotal asociada a radiocirugia. Los periodos de seguimiento forzosamente cortos de las series radioquirurgicas, no permiten concluir que la radiocirugia sea una terapia definitiva como primera forma de tratamiento en los MSC.

Jean Brihaye, a European neurosurgeon

Jean Brihaye, Professor of Neurosurgery at the Institut Bordet, Brussels, Belgium, is and has been one of the most significant men engaged in the brilliant concept: to promote the free interchange of neurosurgical knowledge, experience and understanding among neurosurgeons from all over Europe. His vivacity and intelligence, his natural ability for solving awkward situations, his sense of fairness, his persuasive manners and his deap love of mankind, have made Brihaye a classical humanist of the twentieth century and a profound neurosurgeon beyond the bounds of theoretical dogma, tt is no wonder that he has been engaged with the European Association of Neurosurgical Societies (E.A.N.S.) since the idea of the association was born in Rome in 1963. It is also no wonder that during the International Congress of Neurosurgery held in Toronto in 1985, Jean Brihaye was elected by the Executive Committee of the E.A.N.S. honorary president of the association. The first man that has ever had this honour. At the founding meeting of the E.A.N.S. in Prague (June 1971) Brihaye was chosen as its secretary. He remained in this post for 8 years, until 1978. During this time his dedication to the philosophy of the E.A.N.S. has continued. He combined neurosurgical work in Brussels with frequent travelling all around Europe to personally meet neurosurgeons from member societies, to attend their meetings and to stimulate integration within the E.A.N.S. No efforts were spared by the E.A.N.S. to achieve a uniform postgraduate European training programme in neurosurgery and the minimum facilities and requirements of a neurosurgical service. And, of course, Jean Brihaye was an essential figure in this accomplishment. In 1979 he became President of the E.A.N.S., and he organized the 7th European Congress in 1983, in Brussels. If as secretary his dedicated work for the association was absolutely unique, as a president he had the intelligent ability to foresee the future needs of European neurosurgery. Precision and clarity have delineated his activities. Jean Brihaye has proved to be a great European. Many of us have had the privilege to meet him, admiration and friendship have been the result. A group of his friends is devoting this issue of Acta Neurochirurgica to him. I do hope that the bright and lively eyes of Jean Brihaye when reading all these papers will see more than the scientific contents. Our hearts were filled with gratitude as we wrote them.