Lumbalgia mecánica como forma de presentación de vértebra limbus anterior / Mechanical low back pain as a presentation of anterior limbus vertebra

No disponible

Asociación lupus eritematoso sistémico y esclerosis tuberosa, un caso / Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report

La esclerosis tuberosa (ET), también llamada enfermedad de Pringle Bourneville, es una facomatosis con posible afectación dérmica, neurológica, renal y pulmonar. Se caracteriza por el desarrollo de proliferaciones benignas en numerosos órganos, que dan lugar a las diferentes manifestaciones clínicas. Se asocia a la mutación de 2 genes: TSC1 (hamartina) y TSC2 (tuberina), con la alteración funcional del complejo diana de la rapamicina (mTOR). La activación de la señal mTOR ha sido descrita recientemente en el lupus eritematoso sistémico (LES), y su inhibición podría resultar beneficiosa en pacientes con nefritis lúpica. Presentamos el caso de una paciente que 30 años después del inicio de LES con afectación renal grave (glomerulonefritis tipo IV), resuelta con pulsos intravenosos de ciclofosfamida, comenzó con manifestaciones clínicas del complejo esclerosis tuberosa (CET). Consideramos de interés la coexistencia de estas 2 entidades, ya que solo hemos encontrado 2 casos similares en la literatura (AU)

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest (AU)

Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report. / Asociación lupus eritematoso sistémico y esclerosis tuberosa, un caso.

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest.

Nefropatía aguda por oxalato y pancreatitis crónica / Acute oxalate nephropathy and chronic pancreatitis

No disponible

Degeneration of the brainstem.

Magnetic resonance imaging (MRI) is the most sensitive imaging technique for assessing brainstem involvement in neurodegenerative diseases. An MRI can assess the degree of atrophy of the brainstem and alterations in signal intensity on T2-weighted images in the affected areas, which are the main imaging findings found in these diseases. Besides, diffusion-weighted MRI and proton MR spectroscopy are tools that play an important role in the characterization of these entities. Our purpose is to describe the neurodegenerative diseases that predominantly affect the brainstem highlighting the imaging findings most useful for diagnosis.

Síndrome de hipotensión intracraneal: revisión de hallazgos en resonancia magnética / Intracranial hypotension syndrome: a review of the magnetic resonance findings

Introducción. El síndrome de hipotensión intracraneal (SHI) es un síndrome de etiología y presentación clínica variable, causada, en la mayoría de casos, por fuga de líquido cefalorraquídeo a través del saco tecal. La cefalea ortostática asociada a los hallazgos típicos en resonancia magnética (RM), secundarios a la depleción del líquido cefalorraquídeo, constituye a clave para el diagnóstico. Objetivo. Mostrar los hallazgos radiológicos que en un contexto clínico adecuado permiten identificar y diagnosticar esta entidad. Desarrollo. La disminución del volumen del líquido cefalorraquídeo desempeña un papel importante en el SHI, que lleva a un aumento del volumen de sangre compensatorio, fundamentalmente dependiente del sistema venoso. La RM es una técnica sensible en el diagnóstico del SHI. No obstante, los hallazgos por separado son inespecíficos. Entre los hallazgos en RM se encuentran el realce dural difuso y homogéneo, la presencia de pequeñas colecciones subdurales bilaterales, desplazamiento caudal de las estructuras encefálicas (pseudo-Chiari), dilatación de venas corticales y medulares, y el reciente signo de la distensión venosa. Este último signo constituye un hallazgo altamente sensible de SHI, que tiende adesaparecer tras la mejoría clínica del paciente incluso antes de la desaparición del realce paquimeníngeo, y que podría utilizarse como marcador de respuesta al tratamiento. Conclusión. El SHI es una entidad de difícil diagnóstico clínico en la que se han descrito hallazgos típicos en la RM con los que el neurólogo y el radiólogo deben estar familiarizados (AU)

Introduction. Intracranial hypotension syndrome (IHS) is a syndrome with a variable aetiology and clinical presentation that is, in most cases, caused by leakage of cerebrospinal fluid (CSF) through the thecal sac. Orthostatic headache associated to the typical magnetic resonance imaging (MRI) findings, secondary to depletion of CSF, is the key to a correct diagnosis. Aims. To show the imaging findings that, within a suitable clinical context, allow this condition to be identified and diagnosed. Development. Decreased CSF volume plays an important role in IHS, which leads to an increase in the compensatory volume of blood, essentially dependent on the venous system. MRI is a sensitive technique in the diagnosis of IHS. Yet, separate findings are unspecific. The MRI findings include diffuse and homogeneous dural enhancement, the presence of small bilateral subdural collections, caudal displacement of the encephalic structures (pseudo-Chiari), dilatation of thecortical and medullar veins, and the recent sign of venous distension. This last sign is a highly sensitive finding of IHS, which tends to disappear following the patient’s clinical improvement even before the disappearance of the pachymeningeal enhancement, and could be used as a marker for response to treatment. Conclusions. IHS is a condition that is difficult to diagnose clinically for which several typical MRI findings have been reported; both neurologists and radiologists must be familiar with these findings (AU)

[Intracranial hypotension syndrome: a review of the magnetic resonance findings]. / Sindrome de hipotension intracraneal: revision de hallazgos en resonancia magnetica.

INTRODUCTION: Intracranial hypotension syndrome (IHS) is a syndrome with a variable aetiology and clinical presentation that is, in most cases, caused by leakage of cerebrospinal fluid (CSF) through the thecal sac. Orthostatic headache associated to the typical magnetic resonance imaging (MRI) findings, secondary to depletion of CSF, is the key to a correct diagnosis. AIMS: To show the imaging findings that, within a suitable clinical context, allow this condition to be identified and diagnosed. DEVELOPMENT: Decreased CSF volume plays an important role in IHS, which leads to an increase in the compensatory volume of blood, essentially dependent on the venous system. MRI is a sensitive technique in the diagnosis of IHS. Yet, separate findings are unspecific. The MRI findings include diffuse and homogeneous dural enhancement, the presence of small bilateral subdural collections, caudal displacement of the encephalic structures (pseudo-Chiari), dilatation of the cortical and medullar veins, and the recent sign of venous distension. This last sign is a highly sensitive finding of IHS, which tends to disappear following the patient's clinical improvement even before the disappearance of the pachy-meningeal enhancement, and could be used as a marker for response to treatment. CONCLUSIONS: IHS is a condition that is difficult to diagnose clinically for which several typical MRI findings have been reported; both neurologists and radiologists must be familiar with these findings.

Anatomy of the brainstem: a gaze into the stem of life.

The brainstem has an ectodermal origin and is composed of 4 parts: the diencephalon, mesencephalon, pons, and medulla oblongata. It serves as the connection between the cerebral hemispheres with the medulla and the cerebellum and is responsible for basic vital functions, such as breathing, heartbeat blood pressure, control of consciousness, and sleep. The brainstem contains both white and gray matter. The gray matter of the brainstem (neuronal cell bodies) is found in clumps and clusters throughout the brainstem to form the cranial nerve nuclei, the reticular formation, and pontine nuclei. The white matter consists of fiber tracts (axons of neuronal cells) passing down from the cerebral cortex--important for voluntary motor function--and up from peripheral nerves and the spinal cord--where somatosensory pathways travel--to the highest parts of the brain. The internal structure of brainstem, although complex, presents a systematical arrangement and is organized in 3 laminae (tectum, tegmentum, and basis), which extend its entire length. The motor pathway runs down through the basis, which is located at the most anterior part. The cranial nerve nuclei are settled into the middle layer (the tegmentum), just in front of the 4th ventricle and are placed, from medial to lateral, on the basis of their function: somatic motor, visceral motor, visceral sensory, and somatic sensory. All the somatosensory tracts run upward to the thalamus crossing the tegmentum in front of the cranial nerve nuclei. The tectum, formed by the quadrigeminal plate and the medullary velum, contains no cranial nuclei, no tracts and no reticular formation. The knowledge of precise anatomical localization of a lesion affecting the brainstem is crucial in neurological diagnosis and, on this basis, is essential to be familiar with the location of the mayor tracts and nuclei appropriately. Nowadays, current magnetic resonance imaging techniques, although still macroscopic, allow the fine internal structure of the brainstem to be viewed directly and make it possible to locate the main intrinsic structures that justify the symptoms of the patient. In this article we discuss the anatomy of the brainstem and highlight the features and landmarks that are important in interpreting magnetic resonance imaging.

[Isolated mesencephalic stroke related to a ruptured intracranial dermoid cyst]. / Ictus isquémico mesencefálico aislado secundario a ruptura de quiste dermoide.

Dermoids cysts are embrionary benign lesions that comprise approximately 0.04-0.25% of all intracranial tumors. Occasionally they break and spread their content into subarachnoid space and/or lateral ventricles causing several acute or delayed symptoms. Debut of this type of tumor as acute stroke is poorly reflected in literature. We present a 26-year-old woman with a isolated mesencephalic infarct secondary to spontaneous rupture of a dermoid cyst. We discuss the possible pathophysiological mechanisms for this condition and review the literature.

Ictus isquémico mesencefálico aislado secundario a ruptura de quiste dermoide / Isolated mesencephalic stroke related to a ruptured intracranial dermoid cyst

Los quistes dermoides son lesiones benignas deorigen embrionario que representan del 0.04 a 0,25%de todos los tumores intracraneales. Estos quistesocasionalmente pueden romperse diseminándose elcontenido graso intraquístico al espacio subaracnoideoy/o los ventrículos laterales. En este caso puede provocardiversas manifestaciones clínicas de forma agudao retardada. El debut de este tipo de tumor con unictus agudo está escasamente reflejado en la literatura.Presentamos el caso de una mujer de 26 años con uninfarto mesencefálico aislado secundario a la rupturade un quiste dermoide. Discutimos el mecanismofisiopatológico supuesto y realizamos una revisión delos casos recogidos en la literatura (AU)

Dermoids cysts are embrionary benign lesions thatcomprise approximately 0.04-0.25% of all intracranialtumors. Occasionally they break and spread their contentinto subarachnoid space and/or lateral ventriclescausing several acute or delayed symptoms. Debut ofthis type of tumor as acute stroke is poorly reflected inliterature. We present a 26-year-old woman with a isolatedmesencephalic infarct secondary to spontaneousrupture of a dermoid cyst. We discuss the possiblepathophysiological mechanisms for this condition andreview the literature (AU)

Sustitución valvular en un paciente con enfermedad de Chagas. Consideraciones anestésicas / No disponible

No disponible

[Effect of pentoxifylline on survival, cardiac function and both portal and systemic hemodynamics in advanced alcoholic cirrhosis--a randomized double-blind placebo-controlled trial]. / Effecto de la pentoxifilina en la supervivencia, la función cardiaca y en la hemodinámica portal y sistémica de la cirrosis alcohólica avanzada.

OBJECTIVE: To assess the effect of pentoxiphylline (a potent inhibitor of tumor necrosis factor alpha) on survival, on systemic and portal hemodynamics, and on cardiac function in patients with alcoholic cirrhosis. DESIGN: A randomized double-blind placebo-controlled trial. SETTING: A single center using parallel groups of patients to compare pentoxiphylline with placebo. PATIENTS: We recruited 24 patients with alcoholic cirrhosis (8 Child-Pugh B and 16 Child-Pugh C). INTERVENTIONS: Patients were randomly assigned to receive pentoxiphylline (400 mg tid; n = 12) or placebo (n = 12) over a 4-week period. OUTCOME MEASURES: The primary outcome was to extend short-term and long-term survival. Secondary outcomes included hemodynamic benefits (improvement in cardiac function and/or systemic vascular resistance index, or decrease in portal pressure). RESULTS: Portal pressure and cardiac function remained unchanged and there were no significant differences in short-term or long-term survival between treatment and placebo groups. The group on pentoxiphylline increased systemic vascular resistance and decreased cardiac indices (from 1,721 +/- 567 to 2,082 +/- 622 dyn.sec(-1) cm(-5) m(-2) and from 4.17 +/- 1.4 to 3.4 +/- 0.9 l.m(-2), p = 0.05). CONCLUSIONS: Although pentoxiphylline seems to provide some short-term hemodynamic benefits in patients with advanced alcoholic cirrhosis, this drug has no effect on survival or portal pressure in these patients.

Efecto de la pentoxifilina en la supervivencia, la función cardiaca y en la hemodinámica portal y sistémica de la cirrosis alcohólica avanzada / No disponible

Objetivo: valorar el efecto de la pentoxifilina (un potente inhibidordel factor de necrosis tumoral alfa) en la supervivencia, en lahemodinámica sistémica y portal y en la función cardiaca en la cirrosisalcohólica avanzada.Diseño: estudio aleatorizado, doble-ciego, controlado con placebo.Contexto: estudio unicéntrico utilizando grupos de pacientesen paralelo para comparar pentoxifilina y placebo.Pacientes: se incluyeron 24 pacientes con cirrosis alcohólica(8 en estadio B de Child-Pugh y 16 en estadio C de Child-Pugh).Intervención: los pacientes fueron aleatorizados a recibirpentoxifilina (400 mg, 3 veces al día, n = 12) o placebo (n = 12)durante 4 semanas.Determinaciones: el objetivo principal fue la supervivencia acorto/largo plazo. Los objetivos secundarios fueron observar beneficioshemodinámicos (mejoría en la función cardiaca y/o en elíndice de resistencias vasculares sistémicas o disminución de lapresión portal).Resultados: la presión portal y la función cardiaca no se modificarony no hubo diferencias en la supervivencia a corto y largoplazo entre los grupos tratados y placebo. Los índices de resistenciavascular sistémica y cardiaco cambiaron en el grupo de pentoxifilina(de 1.721 ± 567 a 2.082 ± 622 Din.seg1 cm-5 m-2 y de4,17 ± 1,4 a 3,4 ± 0,9 lm-2, p = 0,05).Conclusiones: aunque la pentoxifilina parece producir algúnbeneficio hemodinámico a corto plazo en pacientes con cirrosis alcohólicaavanzada, no tiene efecto sobre la tasa de supervivencia, lafunción cardiaca ni sobre la presión portal en estos pacientes

Objective: to assess the effect of pentoxiphylline (a potent inhibitorof tumor necrosis factor alpha) on survival, on systemicand portal hemodynamics, and on cardiac function in patientswith alcoholic cirrhosis.Design: a randomized double-blind placebo-controlled trial.Setting: a single center using parallel groups of patients tocompare pentoxiphylline with placebo.Patients: we recruited 24 patients with alcoholic cirrhosis (8Child-Pugh B and 16 Child-Pugh C).Interventions: patients were randomly assigned to receivepentoxiphylline (400 mg tid; n = 12) or placebo (n = 12) over a 4-week period.Outcome measures: the primary outcome was to extendshort-term and long-term survival. Secondary outcomes includedhemodynamic benefits (improvement in cardiac function and/orsystemic vascular resistance index, or decrease in portal pressure).Results: portal pressure and cardiac function remained unchangedand there were no significant differences in short-term orlong-term survival between treatment and placebo groups. Thegroup on pentoxiphylline increased systemic vascular resistanceand decreased cardiac indices (from 1,721 ± 567 to 2,082 ± 622dyn.sec-1 cm-5 m-2 and from 4.17 ± 1.4 to 3.4 ± 0.9 l.m-2, p =0.05).Conclusions: although pentoxiphylline seems to providesome short-term aemodynamic benefits in patients with advancedalcoholic cirrhosis, this drug has no effect on survival or portalpressure in these patients

Hippocampal sclerosis: histopathology substrate and magnetic resonance imaging.

The term hippocampal sclerosis was originally used to describe a shrunken and hardened hippocampus, which histologically displayed neuronal loss and glial proliferation. These alterations are mainly located in the hilus of the dentate gyrus and in the CA1 and CA3 pyramidal cell layers but all hippocampal regions may show neuronal cell loss to varying degrees. A number of morphologic and cytochemical findings are associated with mesial temporal sclerosis, especially within the dentate gyrus. These changes include selective loss of inhibitory interneurons, abnormal sprouting of axons, reorganization of neural transmitter receptors, alterations in second messenger systems, and hyperexcitability of the granule cells. Extrahippocampal pathology is also found at other temporal lobe structures. Frequent extrahippocampal pathology affects the amygdala, first seen with neuronal cell loss and gliosis in the laterobasal complex. Surgical removal of this epileptogenic area can be curative or provide significant reduction in seizure frequency in the majority of individuals. Magnetic resonance imaging (MRI) is highly sensitive in detecting and locating mesial temporal sclerosis when a correct MRI temporal lobe protocol is used. The most important MRI findings, atrophy and abnormal T2 signal, allow us to detect mesial temporal sclerosis in the majority of the cases. Secondary MRI findings help in the diagnosis and lateralization of mesial temporal sclerosis in patients with subtle primary findings and in cases of bilateral hippocampal abnormalities. The development of advanced magnetic resonance (MR) techniques, such as functional MR, diffusion, or transference of magnetization, will lead to greater understanding of this pathology and will improve our diagnostic capacity.

Florid reactive periostitis ossificans of the distal ulna.

Florid reactive periostitis is the term used to describe a benign disease characterized by aggressive periosteal reaction and benign soft-tissue inflammation. Although it is considered rare, there are numerous reports in the literature that refer to this disease with different names such as parosteal fasciitis, fasciitis ossificans, benign fibro-osseous pseudotumor, pseudomalignant osseous tumor of soft tissue, and bizarre parosteal osteochondromatous proliferation. As a result, the nomenclature is confusing, and some authors have placed florid reactive periostitis ossificans into the heterogeneous group of pseudomalignant osseous tumors of soft tissue or proliferative periosteal processes, whereas other authors place this entity in the myositis ossificans group. In the same manner, florid reactive periostitis has been considered to be a previous stage of bizarre parosteal osteochondromatous proliferations. This article presents a case of florid reactive periostitis ossificans of the distal ulna in a 13-year-old boy. The patient presented with a painful lesion in the distal ulna, and plain radiographs suggested the presence of a quickly growing periosteal lesion with associated calcification and soft tissue mass. Histologically, the appearance was that of reactive periostitis. The clinical, radiological, and histologic features of florid reactive periostitis are described.

Macroanatomy and microanatomy of the temporal lobe.

Because of its different functions and organization, the temporal lobe may be divided into lateral and medial parts. This separation may be useful for teaching purposes, since the medial temporal lobe needs a separated and a more precise study because of its complex structure and because it is the substrate where some specific types of epilepsy originate. The use of certain magnetic resonance imaging (MRI) sequences and protocols has improved the diagnosis of some particular epilepsies, but this technical benefit must be accompanied by the accurate knowledge of the anatomy of the temporal lobe. With this purpose we have prepared this article, which highlights the ultastructural and macroanatomy of the temporal lobe seen on MRI.