Síndrome de Fahr / Fahr's syndrome

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Aneurismas incidentales en el contexto de hemorragias subaracnoideas perimesencefálicas / Incidental aneurysms and perimesencephalic subarachnoid haemorrhages

La hemorragia subaracnoidea perimesencefálica se refiere a la presencia de sangre alrededor del mesencéfalo y angiografía normal, cumpliendo con criterios clínicos y radiológicos bien establecidos. A diferencia de la hemorragia subaracnoidea de causa aneurismática, presenta un buen pronóstico, siendo la recuperación, en la mayoría de los casos, completa y satisfactoria. Por otra parte, entre el 2 y el 5% de la población desarrollará un aneurisma intracraneal en el curso de la vida, la mayoría de ellos asintomáticos, siendo posible encontrar un aneurisma incidental en pacientes que han sufrido una hemorragia perimesencefálica. La importancia de un diagnóstico adecuado regirá la conducta a seguir planteando un reto para los equipos de tratamiento vascular, debiendo analizar pormenorizadamente los hallazgos e individualizando las decisiones terapéuticas (AU)

A perimesencephalic subarachnoid hemorrhage refers to the presence of bleeding around the midbrain with a normal angiography, meeting the well established clinical criteria and radiological criteria. Unlike the aneurysmal subarachnoid hemorrhage, it has a good prognosis, the recovery in most cases being complete and satisfactory. On the other hand, between 2 and 5% of the population will develop an intracranial aneurysm in the course of their life, most of them asymptomatic, with the likelihood of finding an incidental aneurysm in patients who have suffered a perimesencephalic hemorrhage. The importance of a proper diagnosis governs the course to follow, being a challenge for vascular treatment teams who must analyse the findings in detail and individualize treatment decisions (AU)

[Incidental aneurysms and perimesencephalic subarachnoid haemorrhages]. / Aneurismas incidentales en el contexto de hemorragias subaracnoideas perimesencefálicas.

A perimesencephalic subarachnoid hemorrhage refers to the presence of bleeding around the midbrain with a normal angiography, meeting the well established clinical criteria and radiological criteria. Unlike the aneurysmal subarachnoid hemorrhage, it has a good prognosis, the recovery in most cases being complete and satisfactory. On the other hand, between 2 and 5% of the population will develop an intracranial aneurysm in the course of their life, most of them asymptomatic, with the likelihood of finding an incidental aneurysm in patients who have suffered a perimesencephalic hemorrhage. The importance of a proper diagnosis governs the course to follow, being a challenge for vascular treatment teams who must analyse the findings in detail and individualize treatment decisions.

Quiste dentígero asociado con mesiodens: Exposición de un caso, revisión de la literatura y diagnóstico diferencial / Dentigerous cyst associated with mesiodens: a case report, literature review and differential diagnostic

El quiste dentígero es uno de los quistes odontogénicos más frecuentes y están asociados con la corona de un diente no erupcionado o en desarrollo. Cuando se asocian con dientes supernumerarios son poco frecuentes y constituyen el 5-6% de todos los quistes dentígeros. En región maxilar, el 90% de los casos se asocian amesiodens. El objetivo de este artículo es exponer el caso de una adolescente de 14 años con un quiste dentígero asociado con un diente supernumerario (mesiodens) impactado en región maxilar anterior, su manejo dental y revisión de la literatura (AU)

Dentigerous cyst is one of the most prevalent types of odontogenic cyst and is associated with crown of an unerupted or developing tooth. Dentigerous cysts associated with supernumerary teeth are rare and estimated to constitute 5-6% of all dentigerous cysts. The vast majority, about 90%, are associated with a maxillarymesiodens. The purpose of this article to report the case of an 14-year-old a teenager with a dentigerous cyst associated with an impacted anterior maxillary supernumerary tooth, its dental management and literature review (AU)

Angiosarcoma epitelioide vertebral primario / Primary spinal epitheliod angiosarcoma

El angiosarcoma óseo primario es una rara eventualidad que representa, tan solo, el 1% de todos los angiosarcomas. Estos tumores asientan con mayor frecuencia en huesos largos. La afectación vertebral primaria se ha comunicado en un 10% de angiosarcomas. Como resultado de esta rareza se desconocen, en gran medida, sus características patológicas, clínicas y terapéuticas. Presentamos el caso de una paciente con angiosarcoma epitelioide vertebral primario en D8 que debutó con dolor dorsal y paraparesia con el objetivo de intentar contribuir a un mejor conocimiento de este raro proceso (AU)

Primary bone angiosarcoma is rare, and is only seen in 1% of all sarcomas. These tumours are more often found in the long bones. Primary spinal involvement has been reported in 10% of angiosarcomas. As a result of its rarity, its pathological, clinical and therapeutic characterists are largely unknown. We presnt the case of a patient with a primary vertebral epithelioid angiosarcoma in D8 which initially presented as dorsal pain and paraparesis, with the aim of contributing to a better knowlwedge of this rare process (AU)

[Spinal hematomas: the spinal apoplexy]. / Hematomas espinales: la apoplejía espinal.

INTRODUCTION: Spinal haematomas (SH) are a rare pathology. They can produce a rapid and irreversible neurological deterioration. METHOD: In this retrospective study, we review 8 cases of SH treated in our centre over the last five years. Data collected were: age, sex, predisposing factors, clinical status, radiological features, treatment and outcome. RESULTS: Five patients were female and three male. Age ranged between 13 and 81 years. Five patients were hypertensive. Four had a coagulation disorder. In three patients SH occurred after physical effort. One case appeared after a lumbar puncture and another was secondary to intramedullary cavernous angioma. All patients presented with pain followed by neurological symptoms. Four cases were epidural, two subdural and two were intramedullary. All epidural SH and one subdural SH, were located dorsal to medulla. Five patients were operated on due to progressive neurological deterioration. Only one of them showed neurological improvement. Patients who were not operated on, had a better neurological status and they improved spontaneously. Two of them were discharged without neurological symptoms. CONCLUSIONS: There is controversy over the physiopathology of SH. The triggering mechanisms are unknown. The vessel (artery or vein) and the anatomical compartment in which SH arise are also unknown. Prompt diagnosis and urgent surgical treatment are needed when the patient has neurological symptoms. In these cases, the most important prognostic factor is the preoperative neurological status.

Hematomas espinales: la apoplejía espinal / Spinal hematomas: the spinal apoplexy

Introducción: Los hematomas espinales (HE) son una afección muy poco frecuente, que puede causar un deterioro neurológico rápido e irreversible. Pacientes y método: Presentamos un estudio retrospectivo con 8 casos de HE tratados en nuestro servicio en los últimos 5 años. Se analizaron: edad, sexo, factores predisponentes, situación clínica, características radiológicas, tratamiento y evolución. Resultados: Los pacientes eran 5 mujeres y 3 varones. Las edades oscilaron entre 13 y 81 años. Cinco pacientes eran hipertensos; 4 presentaban alteraciones de la coagulación; en 3, el hematoma apareció en relación con un esfuerzo físico; en 1, tras una punción lumbar y en 1 fue secundario a un cavernoma intramedular. El inicio clínico fue en todos los casos con dolor, seguido de la instauración de síntomas neurológicos. Cuatro eran epidurales, dos subdurales y dos intramedulares. Todos los HE epidurales y uno subdural eran posteriores a la médula espinal. Cinco pacientes fueron intervenidos por deterioro neurológico progresivo. Sólo 1 presentó mejoría clínica. Los casos no intervenidos estaban en mejor situación neurológica y mejoraron espontáneamente, y 2 de ellos fueron dados de alta sin secuelas. Conclusiones: Los HE son procesos de fisiopatología controvertida. Se desconocen los mecanismos que los desencadenan, así como los vasos (arteriales o venosos) y el compartimento anatómico en que se originan. Se precisa un diagnóstico precoz y un tratamiento quirúrgico urgente cuando hay síntomas neurológicos progresivos. En estos casos, el factor pronóstico más importante es el estado neurológico preoperatorio (AU)

Introduction: Spinal haematomas (SH) are a rare pathology. They can produce a rapid and irreversible neurological deterioration. Method: In this retrospective study, we review 8 cases of SH treated in our centre over the last five years. Data collected were: age, sex, predisposing factors, clinical status, radiological features, treatment and outcome. Results: Five patients were female and three male. Age ranged between 13 and 81 years. Five patients were hypertensive. Four had a coagulation disorder. In three patients SH occurred after physical effort. One case appeared after a lumbar puncture and another was secondary to intramedullary cavernous angioma. All patients presented with pain followed by neurological symptoms. Four cases were epidural, two subdural and two were intramedullary. All epidural SH and one subdural SH, were located dorsal to medulla. Five patients were operated on due to progressive neurological deterioration. Only one of them showed neurological improvement. Patients who were not operated on, had a better neurological status and they improved spontaneously. Two of them were discharged without neurological symptoms. Conclusions: There is controversy over the physiopathology of SH. The triggering mechanisms are unknown. The vessel (artery or vein) and the anatomical compartment in which SH arise are also unknown. Prompt diagnosis and urgent surgical treatment are needed when the patient has neurological symptoms. In these cases, the most important prognostic factor is the preoperative neurological status (AU)

Complicación espinal hemorrágica secundaria a una punción lumbar diagnóstica / Spinal cord hemorrhage complicating diagnostic lumbar puncture

No disponible

No disponible

Utilidad de la resonancia magnética con espectroscopia para el diagnóstico no invasivo del tuberculoma cerebral / No disponible

No disponible

[Haemorrhage in arteriovenous malformations of the brain following radiosurgery: the importance of the latency period]. / Hemorragia de las malformaciones arteriovenosas cerebrales tras la radiocirugía: importancia del período de latencia.

INTRODUCTION: Arteriovenous malformations are abnormal communications between arteries and veins. Treatment can consist in surgery, embolisation and/or radiosurgery. AIM: To assess the influence of radiosurgery on the risk of bleeding and related factors. PATIENTS AND METHODS: Retrospective study of 79 patients who were treated with a 6 MV linear accelerator. Clinical, morphological and dosimetric parameters were collected and a statistical analysis was performed to investigate their relationship with bleeding during the latency period. RESULTS: The mean age was 33.7 years, and 56% of patients were females. The mean volume was 6.16 cm(3). Bleeding occurred before treatment in 52.6% of cases. Altogether 25% had been embolised and 7.6% had been treated earlier using radiosurgery. Six patients suffered a haemorrhage after treatment. Bleeding occurred in 21% of those who had undergone embolisation versus 3.6% in non-embolised individuals (p < 0.02). Bleeding occurred in 33.3% of those who had been treated radiosurgically on more than one occasion, and only 5.7% if they were treated just once (p = 0.02). Bleeding took place in 28.6% of lesions above 10 cm(3) and in 3.2% when they were smaller (p < 0.01). Bleeding occurred in 16.1% of patients if they received less than 17 Gy, and in 2.22% if they received 17 or more (p < 0.01). All haemorrhages took place in treatments with more than one isocentre (p < 0.01) and with a higher homogeneity index (p < 0.01). CONCLUSIONS: Radiosurgery does not modify the risk of bleeding. The factors associated to higher percentages of bleeding are also related to poorer closure outcomes and longer latency periods.

Hemorragia de las malformaciones arteriovenosas cerebrales tras la radiocirugía: importancia del período de latencia / Haemorrhage in arteriovenous malformations of the brain following radiosurgery: The importance of the latency period

Introducción. Las malformaciones arteriovenosas son comunicaciones anómalas entre arterias y venas. El tratamiento puede realizarse mediante cirugía, embolización y/o radiocirugía. Objetivo. Evaluar la influencia de la radiocirugíasobre el riesgo de sangrado y los factores relacionados con éste. Pacientes y métodos. Estudio retrospectivo de 79 pacientes tratados con acelerador lineal de 6 MV. Se recogieron parámetros clínicos, morfológicos y dosimétricos, analizando estadísticamentesu relación con el sangrado durante el período de latencia. Resultados. La edad media fue de 33,7 años, y el 56%fueron mujeres. El volumen medio fue de 6,16 cm3. El 52,6% sangró antes del tratamiento. El 25% había sido embolizado y el 7,6% había recibido radiocirugía previamente. Seis pacientes sufrieron hemorragia tras el tratamiento. Sangró el 21% de los embolizados, frente al 3,6% de los no embolizados (p = 0,02). Entre los tratados con más de una radiocirugía sangró el33,3%, y sólo el 5,7% si se trataron una sola vez (p = 0,02). Sangró el 28,6% de las lesiones mayores de 10 cm3 y el 3,2% de las menores (p < 0,01). Sangró el 16,1% si recibieron menos de 17 Gy, y el 2,22% si recibieron 17 o más (p < 0,01). Todas las hemorragias se produjeron en tratamientos con más de un isocentro (p < 0,01) y con índice de homogeneidad más alto (p < 0,01). Conclusiones. La radiocirugía no modifica el riesgo de sangrado. Los factores asociados con mayores porcentajes de hemorragia se relacionan también con peores resultados de cierre y períodos de latencia más prolongados

Introduction. Arteriovenous malformations are abnormal communications between arteries and veins. Treatmentcan consist in surgery, embolisation and/or radiosurgery. Aim. To assess the influence of radiosurgery on the risk of bleeding and related factors. Patients and methods. Retrospective study of 79 patients who were treated with a 6 MV linear accelerator. Clinical, morphological and dosimetric parameters were collected and a statistical analysis was performed to investigate their relationship with bleeding during the latency period. Results. The mean age was 33.7 years, and 56% of patients were females. The mean volume was 6.16 cm3. Bleeding occurred before treatment in 52.6% of cases. Altogether 25% had been embolised and 7.6% had been treated earlier using radiosurgery. Six patients suffered a haemorrhage after treatment. Bleeding occurred in 21% of those who had undergone embolisation versus 3.6% in non-embolised individuals (p < 0.02).Bleeding occurred in 33.3% of those who had been treated radiosurgically on more than one occasion, and only 5.7% if they were treated just once (p = 0.02). Bleeding took place in 28.6% of lesions above 10 cm3 and in 3.2% when they were smaller (p < 0.01). Bleeding occurred in 16.1% of patients if they received less than 17 Gy, and in 2.22% if they received 17 or more (p < 0.01). All haemorrhages took place in treatments with more than one isocentre (p < 0.01) and with a higher homogeneityindex (p < 0.01). Conclusions. Radiosurgery does not modify the risk of bleeding. The factors associated to higher percentagesof bleeding are also related to poorer closure outcomes and longer latency periods

Coexpression of NRAMP1, iNOS, and nitrotyrosine in bovine tuberculosis.

In murine models the inducible nitric oxide synthase (iNOS) and the natural resistance associated macrophage protein (NRAMP1) play major roles in host defense against mycobacteria. iNOS regulates nitric oxide (NO) production, which is noxious for ingested mycobacteria, and NRAMP1 displays pleiotropic antimicrobial effects, including upregulation of iNOS expression. Little is known about the role of these molecules in bovine tuberculosis (TB). In this work we demonstrate by Western blot a high expression of NRAMP1 in peripheral blood mononuclear cells (PBMCs), alveolar macrophages (obtained by bronchioalveolar lavage), and lymph node granulomas from 8 Holstein-Freisian cattle with autopsy-proven bovine TB. Immunohistochemistry revealed the abundant expression of NRAMP1 and iNOS in lymph node and lung granulomas. Immunoreactivity was abundant in the cytoplasm of many epithelioid macrophages and multinucleated giant cells of the Langhans type. A striking accumulation of nitrotyrosine (NT), an indicator of iNOS activity and local NO production, was observed in granuloma cells, particularly in multinucleated Langhans cells. This study shows that the expression of NRAMP1 and iNOS is costimulated in granulomas, which are protective T-cell reactions against mycobacteria.

[Histiocytic necrotizing lymphadenitis or Kikuchi's disease. CT radiologic features]. / Linfadenitis necrotizante histiocitaria o enfermedad de Kikuchi. Apariencia radiológica mediante TC.

Histiocytic necrotizing lymphadenitis, Kikuchi Fujimoto's disease (KFD) is characterised by fever and lymphadenopathy, usually large cervical, unilateral lymph nodes. Such clinical presentation demands a work-up to exclude serious medical conditions like malignancy and infections. Foci of necrosis with lymphocytic Histiocytic predominance in association with scarce polymorphonuclear cells on lymph node examination, confirm the diagnosis of KFD. Many different patterns of computed tomographic (CT) appearance of KFD have been reported. We describe the CT scan finding in two patients with this disease. All our cases showed, after two and three weeks of evolution respectively, enlarged lymph nodes with hypodense centres and peripheral ring enhancement. These radiological alterations correlated with the central lymph node necrosis found in the anatomopathological studies. In conclusion, KFD should be considered in patients with fever, cervical lymph node enlargement and CT scan showing hypointense centres and peripheral ring enhancement.