[Neuro-Behçet: clinicopathological findings in an autopsy case]. / Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia.

INTRODUCTION: Behçet's disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in this study. CASE REPORT: A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobule extended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammation in small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. CONCLUSIONS: The necropsy studies of patients with neuro-Behcet's disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem.

Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia / Neuro-behçet: Clinicopathological findings in an autopsy case

Introducción. La enfermedad de Behçet (EB) la describió por primera vez en 1937 Hulusi Behçet como la tríada deúlceras orales, úlceras genitales y uveítis. Involucra a casi todos los tejidos y órganos sin excepción, y la tasa de afectación del sistema nervioso central es del 5 al 30%. Los estudios histopatológicos de casos de necropsias son escasos, a pesar de que la causa de muerte se constata en este estudio. Caso clínico. Varón de 30 años que comenzó con EB con afectación neurológicade evolución progresiva y fatal. El estudio autópsico mostró en el examen externo úlceras orales y escrotales. El encéfalo presentaba marcado edema cerebral, meninges opacas y un infarto hemorrágico frontal izquierdo con extensión hacia el ventrículo lateral homólogo, y el tronco del encéfalo, infartos hemorrágicos confluentes y hemorragia del cuarto ventrículo. En el examen de los órganos restantes destacaban múltiples infartos pulmonares bilaterales. El estudio histológico del encéfalo mostró, de forma generalizada, predominante en el tronco del encéfalo, vasos de pequeño y mediano calibre rodeados por una corona de linfocitos y neutrófilos, con intensa extravasación eritrocitaria perivascular. Conclusiones. Los estudios denecropsias de pacientes con neurobehçet son escasos. Presentamos los hallazgos clínicos y patológicos de un paciente con EB con afectación neurológica, en el que destaca una afectación vascular cerebral predominante en el tronco del encéfalo

Introduction. Behçet’s disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in thisstudy. Case report. A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobuleextended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammationin small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. Conclusions. The necropsy studies of patients with neuro-Behçet’s disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem

Prevalencia de la patología fúngica en biopsias y autopsias realizadas en el Hospital Universitario Joan XXIII de Tarragona / Prevalence of fungal infections detected from biopsies and autopsies in the past 11 years at the University Hospital Joan XXIII in Tarragona, Spain

La patología infecciosa, favorecida por distintos factores, es cada vez más frecuente en nuestros pacientes. Dentro de ésta, la patología debida a hongos está en aumento. Se han descrito más de 300 especies de hongos patógenos para el hombre. Para conocer la prevalencia de la patología fúngica en nuestro hospital (de nivel C y con 330 camas), se ha realizado un estudio retrospectivo de la presencia de micosis en biopsias y autopsias en los últimos once años (1994-2004). De un total de 78.310 biopsias y 753 autopsias, un 0,24% mostraron la presencia de hongos (0,21% en biopsias y 4,25% en autopsias), siendo la localización más común la piel y las mucosas (66%). De los hongos observados, un 61% correspondía al género Candida, seguido de Aspergillus (10%) y zigomicetos (5%). La patología de base más importante fue la enfermedad pulmonar obstructiva crónica, seguida de diabetes y sida. Cabe destacar la observación de un aumento del número de casos con la edad del paciente, siendo la octava década de la vida la que presentaba mayor incidencia de infección fúngica. El tratamiento mejoraba la evolución de los pacientes, si bien las patologías de base son factores muy importantes en la evolución. Las micosis sistémicas presentaban el peor pronóstico con un 91% de mortalidad. En consecuencia, un diagnóstico rápido con intervención del patólogo es muy importante de cara al pronóstico(AU)

Infectious diseases caused by fungal pathogens have increased in the past 10 years. More than 300 pathogenic fungal species have been incriminated as the etiologic agents. We carried out a retrospective study (1994-2004) to evaluate the prevalence of mycoses at the University Hospital Joan XXIII (330 beds). This report found 0.24% of the studied cases (78,310 biopsies and 753 autopsies) were diagnosed as fungal infections (0.21% of the total studied biopsy and 4.25% of the whole autopsies). Skin and mucose were involved in 66% of cases, followed by other less affected anatomical areas. 61% of studied cases were caused by Candida spp (the most frequent in our environment), followed by Aspergillus spp (10%) and the Zygomycetes (5%). The most important underlying illness was obstructive chronic pulmonary disease followed by diabetes and AIDS. The incidence of mycoses increased with the patient's age, especially those patients in their 80s. Antifungal management improved the clinical outcome of the patient but predisposing factors are crucial for diagnosis. Systemic mycoses have poor prognosis with 91% of fatal outcome. Thus, it is important to perform a rapid diagnosis of the fungal infections a diagnostic area in which pathology could play a major role(AU)

Linfoma cardiaco primario. Presentación de dos casos en pacientes inmunocompetentes / Primary cardiac lymphoma: presentation of two cases in immunocompetent patients

Fundamento: El linfoma cardiaco primario (LCP) es muy infrecuente en pacientes sin alteraciones inmunitarias habiéndose descrito unos 50 casos en el mundo hasta 1996. Su patogenia no se conoce y algunos estudios que han investigado la presencia del virus de Epstein Barr en el tumor en general no lo han demostrado. Casos: Se describen los casos de dos pacientes con LCP. En ambos casos el diagnóstico fue realizado post mortem. Ambos pacientes presentaban un linfoma B de células grandes difuso. En los dos casos se ha identificado la presencia de focos inflamatorios a cierta distancia del tumor. No se ha podido demostrar la presencia de VEB en el tumor. Conclusiones: Los LCPs son tumores de rápido crecimiento con un pronóstico muy desfavorable. El virus de Epstein-Barr no parece desempeñar un papel en la etiopatogenia de estos tumores (AU)

Background: Primary cardiac lymphomas (PCL) are extremely rare in immunocompetent patients. Their pathogenesis seems obscure and a few studies which have sought Epstein Barr virus (EBV) agree that this is generally not demonstrable. Cases: Two cases of cardiac lymphoma have been described. In both cases, as usually occurs, the histological diagnosis was performed after post-mortem examination. Histological examinations revealed a B large cell lymphoma in both patients. Autopsy material from both cases showed an imflamatory focus distant from the tumoral mass. EBERs and LMP for EBV were negative. Conclusions: PCLs are often fast-growing tumours with a highly unfavorable prognosis. Epstein-Barr virus appears to play no role in the development of this malignant condition (AU)

[Primary cardiac lymphoma: presentation of two cases in immunocompetent patients]. / Linfoma cardiaco primario. Presentación de dos casos en pacientes inmunocompetentes.

BACKGROUND: Primary cardiac lymphomas (PCL) are extremely rare in immunocompetent patients. Their pathogenesis seems obscure and a few studies which have sought Epstein Barr virus (EBV) agree that this is generally not demonstrable. CASES: Two cases of cardiac lymphoma have been described. In both cases, as usually occurs, the histological diagnosis was performed after post-mortem examination. Histological examinations revealed a B large cell lymphoma in both patients. Autopsy material from both cases showed an inflammatory focus distant from the tumoral mass. EBERs and LMP for EBV were negative. CONCLUSIONS: PCLs are often fast-growing tumours with a highly unfavorable prognosis. Epstein-Barr virus appears to play no role in the development of this malignant condition.

Síndromes paraneoplásicos con afectación nerviosa periférica / Paraneoplastic syndromes involving the peripheral nerves

Objetivo. Realizar la revisión de las complicaciones paraneoplásicas descritas que afectan al sistema nervioso periférico.Desarrollo. Diferentes tipos de tumores, especialmente los carcinomas secretores y algunos linfomas, pueden producir afectación de neurona periférica, que en muchas ocasiones precede a la manifestación del tumor. Según su manifestación clínica se puede hablar de neuronopatías sensitivas, neuronopatías motoras, neuropatías sensitivomotoras, neuropatía autonómica, mononeuritis y neuromiotonía. Pueden presentarse de forma aislada o dentro de una afectación más amplia del sistema nervioso. Conclusiones. La presencia de determinados anticuerpos anti-Hu, que actúan contra las neuronas o anticuerpos que atacan los canales de potasio voltajedependientes en una afectación del sistema nervioso periférico, hacen pensar en un origen paraneoplásico (AU)

[Paraneoplastic syndromes with peripheral nerve involvement]. / Síndromes paraneoplásicos con afectación nerviosa periférica.

OBJECTIVE: To review the paraneoplastic complications described affecting the peripheral nervous system. DEVELOPMENT: Different types of tumors, especially secretory carcinomas and some lymphomas may affect the peripheral nerves, and this may often precede clinical signs of the tumor. Depending on the clinical features we may consider sensory neuropathies, motor neuropathies, senso-motor neuropathies, autonomic neuropathies, mononeuritis, and neuromyotonia. They may present in an isolated form or as part of a more generalized involvement of the nervous system. CONCLUSIONS: The presence of certain anti-Hu antibodies, which act against nerves or antibodies which attack the voltage-dependent potassium channels in a peripheral nervous system disorder make one suspect a paraneoplastic origin.

[Intraperitoneal hemorrhage caused by cavernous hemangioma of the small intestine]. / Hemorragia intraperitoneal por hemangioma cavernoso de intestino delgado.

We report a case of hemorrhage into the abdominal cavity due to the rupture of an intestinal cavernous hemangioma. Out of the 30 published cases from 1976 to 1993 dealing with hemangioma of the small intestine, none presented with intraperitoneal bleeding.

[Perforation of small intestine diverticulum in Ehlers-Danlos syndrome]. / Perforación de divertículo de intestino delgado en el síndrome de Ehlers-Danlos.

We report a case of acute abdomen in a patient previously diagnosed of Ehlers-Danlos syndrome. The pneumoperitoneum on the chest x-ray demanded urgent operative intervention. Laparotomy revealed two diverticula perforations in the small bowel. Perforation in the colon is common; however only two cases of small bowel perforation have been reported so far.

[Schistosomiasis (bilharziosis). Importance of cytology. Report of 2 cases]. / Esquistosomiasis (Bilharziosis). Importancia de la citología. A propósito de dos casos.

We report two cases of bilharziasis of the bladder in immigrants who presented with spontaneous hematuria. This condition was diagnosed by urine cytology. The current tide of immigration and tourism have led to an increased number of diseases acquired abroad. A simple, economic and rapid method for diagnosing schistosomiasis in patients at risk with hematuria is described.

Hepatic infarction: an unusual complication of nephrotic syndrome in a patient with diabetes mellitus.

A 67 year old woman with widespread atherosclerosis and diabetic nephropathy manifested by nephrotic syndrome and moderate renal failure developed multiple hepatic infarctions. The infarctions were documented by computed tomographic scan and needle aspiration biopsy of the liver. Except for the nephrotic syndrome and the atherosclerosis no other cause of hepatic infarction was found. We suggest that hepatic infarction should be considered in the thrombotic complications of the nephrotic syndrome secondary to diabetic nephropathy.