[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

[Discrepancies between clinical and pathological diagnosis in a polyvalent intensive care service]. / Discrepancias entre el diagnóstico clínico y el anatomopatológico en un Servicio de Cuidados Intensivos Polivalente.

OBJECTIVES: Analyze the frequency and spectrum of the most relevant diseases found in the necropsic study. Assess the association between stay in Intensive Care Unit (ICU) less than 24 hours and rate of diagnostic errors. MATERIAL AND METHODS: Retrospective, observational study during a 46 month period in a polyvalent ICU. The differences between the clinical and pathological diagnoses were established based on Goldman's classification. RESULTS: A total of 85 autopsies out of 520 exitus (16.3%) were done. Five patients were excluded due to incomplete information. Of the 80 cases, we found 30 patients with major errors, 21 with therapeutic and prognostic repercussion, 9 in which the therapeutic strategy had not been modified. The most frequently found diagnosis in type I error was bacterial infection followed by cardiovascular disease. Major error rate with therapeutic repercussion was superior in patients with a stay in the ICU less than 24 hours (40% vs 21%; p < 0.05). CONCLUSIONS: Autopsy continues to be a useful tool to assess quality of clinical diagnosis. The diagnostic errors with therapeutic repercussion are bacterial infections and cardiovascular disease. Patients with a stay less than 24 hours have a higher rate of type I diagnostic errors.

Discrepancias entre el diagnóstico clínico y el anatomopatológico en un Servicio de Cuidados Intensivos Polivalente / Discrepancies between clinical and pathological diagnosis in a Polyvalent Intensive Care Service

Objetivos. Analizar la frecuencia y espectro de las patologías más relevantes encontradas en el estudio necrópsico. Valorar la asociación entre estancia en la Unidad de Cuidados Intensivos (UCI) inferior a 24 horas y la tasa de errores diagnósticos. Material y métodos. Estudio retrospectivo observacional durante un período de 46 meses en una UCI polivalente. Las diferencias entre el diagnóstico clínico y anatomopatológico se establecieron en función de la clasificación de Goldman. Resultados. Se realizaron 85 autopsias de un total de 520 exitus (16,3%). Cinco pacientes fueron excluidos por información incompleta. De los 80 casos, encontramos 30 pacientes con errores mayores, 21 con repercusión terapéutica y pronóstica, y 9 en los que la estrategia terapéutica no se hubiera modificado. El diagnóstico más frecuentemente encontrado en el error tipo I fue la infección bacteriana seguida de la patología cardiovascular. La tasa de errores mayores con repercusión terapéutica fue superior en los pacientes con una estancia en UCI inferior a 24 horas (40% frente a 21%; p < 0,05). Conclusiones. La autopsia continúa siendo una herramienta útil para evaluar la calidad del diagnóstico clínico. Los errores diagnósticos con repercusión terapéutica son las infecciones bacterianas y la patología cardiovascular. Los pacientes con una estancia en la UCI inferior a 24 horas presentan una tasa mayor de errores diagnósticos tipo I

Objectives. Analyze the frequency and spectrum of the most relevant diseases found in the necropsic study. Assess the association between stay in Intensive Care Unit (ICU) less than 24 hours and rate of diagnostic errors. Material and methods. Retrospective, observational study during a 46 month period in a polyvalent ICU. The differences between the clinical and pathological diagnoses were established based on Goldman's classification. Results. A total of 85 autopsies out of 520 exitus (16.3%) were done. Five patients were excluded due to incomplete information. Of the 80 cases, we found 30 patients with major errors, 21 with therapeutic and prognostic repercussion, 9 in which the therapeutic strategy had not been modified. The most frequently found diagnosis in type I error was bacterial infection followed by cardiovascular disease. Major error rate with therapeutic repercussion was superior in patients with a stay in the ICU less than 24 hours (40% vs 21%; p < 0.05). Conclusions. Autopsy continues to be a useful tool to assess quality of clinical diagnosis. The diagnostic errors with therapeutic repercussion are bacterial infections and cardiovascular disease. Patients with a stay less than 24 hours have a higher rate of type I diagnostic errors