[Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case]. / Hemangioblastoma sólido-quístico supratentorial afectando la hoz del cerebro. Presentación de un caso.

INTRODUCTION: hemangioblastomas are benign neoplasias that are originated in the central nervous system and constitute between 1.5-2.5% of intracranial tumors. The majority of them are infratentorial, mainly affecting the cerebellum (76%). Supratentorial lessions are rare, being in these cases the frontal, parietal or temporal lobes the most common locations. Meningeal involvement is infrequent. Only eight cases have been reported in the literature. In 30% of the cases, these tumors are associated with von Hippel Lindau syndrome (VHL). CASE REPORT: 67 year old woman without any medical or family history. She presented with 4 month evolution neurological symptoms. The cranial MRI scan showed a solitary solid-cystic lesion on the right paramedian frontal lobe, in contact with the falx cerebri. The pathological analysis showed a cellular proliferation composed of polygonal cells with clear cytoplasm due to the presence of intracytoplasmic vacuoles and round or oval nucleus without cytologic atypia. These cells were accompanied by a rich vascular network of capillary type and blood extravasation. She was diagnosed of supratentorial hemangioblastoma. CONCLUSION: the preoperative diagnosis of these neoplasms is difficult because the clinical suspicion is low in supratentorial location. Imaging techniques are useful but definitive diagnosis is made through pathologic examination. The use of immunohistochemical techniques is helpful for the differential diagnosis with lesions that are more common in this region. The importance of a correct diagnosis of these histologically benign tumors, lies on the possible association with VHL syndrome and its complications.

Hemangioblastoma sólido-quístico supratentorial afectando la hoz del cerebro. Presentación de un caso / Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case

Introducción. Los hemangioblastomas son neoplasias benignas que se originan en el sistema nervioso central y constituyen entre un 1,5-2,5% de los tumores intracraneales. Mayoritariamente son de localización infratentorial, afectando principalmente al cerebelo (76%). Las lesiones supratentoriales son sumamente raras, siendo en estos casos la localización más habitual el lóbulo frontal, parietal o temporal. La afectación meníngea es excepcional, habiéndose descrito sólo ocho casos en la literatura. En un 30% de los casos, estos tumores se asocian al síndrome de von Hippel Lindau (VHL).Caso clínico. Mujer de 67 años sin antecedentes patológicos ni familiares de interés que se presentó conclínica neurológica de 4 meses de evolución. El estudio de resonancia magnética craneal demostró una lesión única sólido-quística frontal paramedial derecha, encontacto con la hoz del cerebro, que se orientó como meningioma. El estudio anatomo-patológico de la pieza quirúrgica objetivó una proliferación celular constituida por células poligonales con citoplasma claro debido a la presencia de vacuolas intracitoplasmáticas y núcleo redondo u oval sin atipia citológica. Estas células estaban acompañadas de una rica red vascular de tipocapilar, con anastomosis y extravasación sanguínea. Se diagnosticó de hemangioblastoma supratentorial. Discusión. El diagnóstico preoperatorio de estas neoplasias es difícil debido a que la sospecha clínica esbaja cuando se halla en localización supratentorial. Las técnicas de imagen son de utilidad, realizándose el diagnóstico definitivo a través del estudio anatomopatológico. El uso de técnicas de inmunohistoquímica es de gran ayuda para el diagnóstico diferencial con lesiones que habitualmente se localizan en esta región. La importancia de un diagnóstico correcto de estos tumores histológicamente benignos, radica, entre otras cosas,en la posible asociación con en síndrome de VHL y suscomplicaciones (AU)

Hemangioblastomas are benign neoplasias that are originated in the central nervous systemand constitute between 1.5-2.5% of intracranial tumors.The majority of them are infratentorial, mainly affectingthe cerebellum (76%). Supratentorial lessions are rare,being in these cases the frontal, parietal or temporallobes the most common locations. Meningeal involvementis in frequent. Only eight cases have been reported in the literature. In 30% of the cases, these tumors areas sociated with von Hippel Lindau syndrome (VHL).Case report. 67 year old woman without any medicalor family history. She presented with 4 month evolution neurological symptoms. The cranial MRI scan showed a solitary solid-cystic lesion on the right paramedianfrontal lobe, in contact with the falx cerebri. The pathological analysis showed a cellular proliferation composed of polygonal cells with clear cytoplasm due to the presence of intracytoplasmic vacuoles and round oroval nucleus without cytologic atypia. These cells wereaccompanied by a rich vascular network of capillarytype and blood extravasation. She was diagnosed ofsupratentorial hemangioblastoma. Conclusion. The preoperative diagnosis of these neoplasmsis difficult because the clinical suspicion is low in supratentorial location. Imaging techniques are useful but definitive diagnosis is made through pathologic examination. The use of immunohistochemical techniquesis helpful for the differential diagnosis with lesions that are more common in this region. The importance of acorrect diagnosis of these histologically benign tumors,lies on the possible association with VHL syndrome and its complications (AU)

Pinealoma y síntomas psiquiátricos. A propósito de un caso / Pineal tumor and psychiatric symptoms. A case report

Las manifestaciones clínicas de los tumores cerebrales son polimorfas. En cualquiera de los casos pueden aparecer manifestaciones psiquiátricas diversas, bien como consecuencia directa de la lesión tumoral, bien indirectamente por el efecto de la masa, la hipertensión intracraneal, el proceso inflamatorio, el tratamiento o los trastornos metabólicos secundarios a los síndromes paraneoplásicos. Se presenta 1 caso en el que la relación entre un tumor intracraneal y la psicopatología de la paciente puede establecerse retrospectivamente por la secuencia temporal, aunque la mejoría de la clínica psiquiátrica no es completa una vez extirpada la lesión. El caso no queda resuelto extirpando de manera pulcra y estéril la irrupción de una afección orgánica sin tener en cuenta las circunstancias y la personalidad previa del individuo y cómo una alteración cerebral en distintas zonas la modifica, a veces de manera irreversible

The clinical manifestations of cerebral tumors are polymorphous. All cerebral tumors can cause diverse psychiatric manifestations, whether as a direct consequence of the tumor, as an indirect consequence of the mass, intracranial hypertension, the inflammatory process, treatment, or metabolic disorders secondary to paraneoplastic syndromes. We present a case in which the relationship between an endocranial tumor and the patient's psychopathology was retrospectively established by the temporal association, although the psychiatric symptoms did not completely disappear on removal of the tumor. This case illustrates that removal of an organic lesion is not sufficient for symptom resolution without taking into account the patient's prior circumstances and personality and how personality can be modified, sometimes irreversibly, by a cerebral lesion

Estimulación del nervio vago y psicosis. A propósito de un caso / Vagus nerve stimulation and psychosis. A single case report

La aparición de alteraciones del comportamiento en el contexto de una disminución en el número de crisis en pacientes epilépticos es un hecho que ya fue descrito por Landolt a mediados del siglo XX en su teoría de la normalización forzada. Aquí proponemos, a través de un caso clínico, una serie de mecanismos que pretenden explicar a grandes rasgos la fisiopatología de este fenómeno. Entre ellos destaca la teoría del aumento en el estado de alerta provocado por los mecanismos inhibitorios secundarios a una epilepsia de varios años de evolución. Por otra parte, se plantea la posibilidad de que la estimulación intermitente del nervio vago izquierdo (ENV), procedimiento empleado como terapia coadyuvante en el manejo de pacientes epilépticos refractarios a politerapia farmacológica, pueda provocar una respuesta inhibitoria de características similares, que pudiese por sí misma originar una serie de cuadros psiquiátricos. Finalmente se barajan diversas alternativas en relación con el proceso fisiopatológico que conlleva la cronificación de dicha patología, entre las cuales destaca un deterioro cognitivo asociado al desarrollo de síntomas negativos en pacientes con epilepsia del lóbulo temporal

The appearance of behavioral disorders in the context of a decrease in the number of seizures in epileptic patients is a fact that was already described in Landolt's theory of forced normalization in the middle of the XX century. Through this clinical case, we propose several mechanisms that aim to give a general explanation to the physiopathology of this condition. Among them, the theory which suggests increased alertness as a result of inhibitory mechanisms secondary to a long lasting epileptic condition stands out. In addition, we consider the possibility that left vagus nerve stimulation (VNS), a procedure used as a second line treatment in epileptic patients refractary to multiple drug therapy, may cause an inhibitory reaction of similar characteristics as the previously described, and could therefore lead to different psychiatric disorders. Lastly, we bring up several alternatives that will try to throw some light on the physiopathological condition that leads to the chronification of this disease, the theory regarding a cognitive deterioration associated to the appearance of negative symptoms in patients with temporal lobe epilepsy standing out

[Vagus nerve stimulation and psychosis. A single case report]. / Estimulación del nervio vago y psicosis. A propósito de un caso.

The appearance of behavioral disorders in the context of a decrease in the number of seizures in epileptic patients is a fact that was already described in Landolt's theory of forced normalization in the middle of the XX century. Through this clinical case, we propose several mechanisms that aim to give a general explanation to the physiopathology of this condition. Among them, the theory which suggests increased alertness as a result of inhibitory mechanisms secondary to a long lasting epileptic condition stands out. In addition, we consider the possibility that left vagus nerve stimulation (VNS), a procedure used as a second line treatment in epileptic patients refractory to multiple drug therapy, may cause an inhibitory reaction of similar characteristics as the previously described, and could therefore lead to different psychiatric disorders. Lastly, we bring up several alternatives that will try to throw some light on the physiopathological condition that leads to the chronification of this disease, the theory regarding a cognitive deterioration associated to the appearance of negative symptoms in patients with temporal lobe epilepsy standing out.