Debut de sarcoidosis simulando una metástasis hepática única / Sarcoidosis onset simulating a unique hepatic metastasis

La sarcoidosis es una enfermedad granulomatosa sistémica de etiología incierta, caracterizada por la formación de granulomas no necrotizantes. La afectación más frecuente es la pulmonar y mediastínica, aunque puede afectar a cualquier órgano. La afectación hepática ocurre en el 50-65% de los casos, pero suele ser subclínica o descubierta en el estudio de una alteración de las enzimas hepáticas. El debut de una sarcoidosis como una tumoración hepática aislada en muy inusual. La biopsia hepática suele ser necesaria para confirmar el diagnóstico y el diagnóstico diferencial debe establecerse con cualquier enfermedad hepática granulomatosa de carácter infeccioso o autoinmune y la exclusión de malignidad. Presentamos un caso clínico de una paciente diagnosticada de sarcoidosis hepática aislada simulando una lesión única hepática metastásica. La biopsia hepática fue diagnóstica

Sarcoidosis is a systemic granulomatous disease with an uncertain etiology, characterized by the production of non-necrotizing granulomas. The most frequent presentation is pulmonary and mediastinal, although it might affect any other organ. Hepatic alterations occur in 50 to 65% of the cases. Nevertheless, it is commonly subclinical or detected during a study of the alteration of liver enzymes. It is very unusual that disease onset occurs as an isolated hepatic tumor. A hepatic biopsy is usually required to confirm the diagnosis. A differential diagnosis must be established via any hepatic granulomatous disease, infectious or autoimmune disease as well as the exclusion of malignancy. We present a clinical case of a female diagnosed with an isolated hepatic sarcoidosis that simulated a unique hepatic metastatic lesion. The hepatic biopsy was diagnostic

Sarcoidosis onset simulating a unique hepatic metastasis.

Sarcoidosis is a systemic granulomatous disease with an uncertain etiology, characterized by the production of non-necrotizing granulomas. The most frequent presentation is pulmonary and mediastinal, although it might affect any other organ. Hepatic alterations occur in 50 to 65% of the cases. Nevertheless, it is commonly subclinical or detected during a study of the alteration of liver enzymes. It is very unusual that disease onset occurs as an isolated hepatic tumor. A hepatic biopsy is usually required to confirm the diagnosis. A differential diagnosis must be established via any hepatic granulomatous disease, infectious or autoimmune disease as well as the exclusion of malignancy. We present a clinical case of a female diagnosed with an isolated hepatic sarcoidosis that simulated a unique hepatic metastatic lesion. The hepatic biopsy was diagnostic.

Definición clínico-patológica de los subtipos de epilepsia temporal medial con esclerosis del hipocampo / Clinical and pathological definition of temporal medium epilepsy subtypes with hypocampic sclerosis

Antecedentes y objetivo: La epilepsia temporal con esclerosis hipocampal es la causa más frecuente de epilepsia refractaria, y la indicación más común de cirugía. Aunque eficaz, la cirugía fracasa hasta en un 40% de los pacientes. El objetivo de nuestro trabajo es establecer una correlación entre los distintos subtipos histológicos de epilepsia temporal con esclerosis hipocampal y el pronóstico, control de crisis, efectos secundarios y retirada de fármacos anticomiciales en los pacientes con epilepsia resistente a fármacos intervenidos. Pacientes y método: Se analizaron de forma retrospectiva las historias clínicas y muestras anatomopatológicas de 228 pacientes con epilepsia temporal intervenidos en nuestro centro entre los años 1993 y 2014. Todos fueron sometidos a una evaluación prequirúrgica estándar e intervenidos mediante una resección temporal anterior (modificada de Spencer). El estudio anatomopatológico incluyó el protocolo de hematoxilina-eosina e inmunohistoquímico estándar, con especial interés en la valoración de pérdida neuronal con NeuN. El control de las crisis fue valorado de acuerdo a la escala de resultados de la ILAE y de Engel. El seguimiento medio fue de 8,6 años (2-19). Resultados: A los 10 años tras la intervención, un 67,9% de los pacientes se encontraban libres de crisis (ILAE 1). Un 77,5% de los pacientes estaba libre de crisis (Engel 1) al final del seguimiento. La probabilidad de quedar sin crisis (ILAE 1) tras la cirugía a los 2 (p = 0,042), 5 (p = 0,001) y 7 (p = 0,022) años fue mayor en las formas clásica y severa frente a las formas de esclerosis aislada CA1 y CA4. Una mayor pérdida neuronal medida con NeuN en CA1 se relacionó con un mejor resultado en el control de las crisis (análisis multivariante, p = 0,08). La presencia de antecedentes personales desencadenantes de epilepsia se relacionó con una mayor pérdida neuronal en CA1 (p = 0,028) y CA3 (p = 0,034), y la presencia de auras psíquicas con una mayor pérdida de neuronas en CA3 (p = 0,025). En nuestro caso, la probabilidad de dejar la medicación se relacionó con la presencia de antecedentes personales (p = 0,003) y, de forma inversa, con la pérdida neuronal en CA1 (p = 0,036) y CA3 (p = 0,038). El mayor deterioro de memoria verbal ocurrió en aquellos pacientes con menor pérdida neuronal en CA1 (p = 0,023), CA2 (p = 0,049) y CA3 (p = 0,035). Conclusiones: Los resultados señalan que los subtipos clásico y severo tienen un mejor pronóstico en el control de las crisis frente a las formas atípicas, validándose la utilidad clínica y pronóstica de la clasificación de consenso de los subtipos histológicos de esclerosis hipocampal de la ILAE. Se ha demostrado el valor de la inmunohistoquímica en la epilepsia temporal con esclerosis hipocampal como un elemento clave para determinar el pronóstico en el control de las crisis y neuropsicológico de los pacientes tras la cirugía

Background and objective: Mesial temporal lobe epilepsy with hippocampal sclerosis is the most common cause of refractory epilepsy, and the most common indication for surgery. Although effective, surgery fails in up to 40% of patients. The objective of our study was to establish a correlation between the different histological subtypes of mesial temporal lobe epilepsy with hippocampal sclerosis and the prognosis, seizures control, side effects and anticonvulsivant drug withdrawal in patients with refractory epilepsy. Patients and method: Clinical histories and anatomopathological specimens of 228 patients with temporal epilepsy surgically obtained at our hospital between 1993 and 2014 were retrospectively analysed. All patients underwent a standard preoperative evaluation and anterior temporal resection (modified from Spencer). The anatomopathological study included the standard hematoxylin-eosin and immunohistochemical protocol, with special interest in the assessment of neuronal loss with NeuN. Seizure control was assessed according to the scale of results of the ILAE and Engel. The mean follow-up was 8.6 years (2-19). Results: At 10 years after the intervention, 67.9% of patients were seizure-free (ILAE 1) and as many as 77.5% of the patients were seizure-free (Engel 1) at the end of the follow-up. The probability of not having a seizure (ILAE 1) after surgery at 2 (p=.042), 5 (p=.001) and 7 years (p=.22) was higher in classic and severe forms compared to isolated sclerosis CA1 and CA4 forms. Higher neuronal loss measured with the NeuN immunostain in CA1 was associated with better outcome in seizure management (multivariate analysis, p=.08). The presence of a personal history of epilepsy was associated with greater neuronal loss in CA1 (p=.028) and CA3 (p=.034), and the presence of psychic auras was related with greater neuronal loss in CA3 (p=.025). In our case, the probability of medication withdrawal was related to the presence of personal history (p=.003) and, inversely, to neuronal loss in CA1 (p=.036) and CA3 (p=.038). The greatest impairment of verbal memory occurred in those patients with a lower neuronal loss in CA1 (p=.023), CA2 (p=.049) and CA3 (p=.035). Conclusions: The results indicate that the classical and severe subtypes have a better prognosis in the control of seizures against the atypical forms, validating the clinical and prognostic utility of the classification of histological subtypes of hippocampal sclerosis from the ILAE. The value of the immunohistochemistry in the mesial temporal lobe epilepsy with hippocampal sclerosis has been demonstrated as a key element to determine the neuropsychological prognosis and seizure management of the patients after surgery

[Clinical and pathological definition of temporal medium epilepsy subtypes with hypocampic sclerosis]. / Definición clínico-patológica de los subtipos de epilepsia temporal medial con esclerosis del hipocampo.

BACKGROUND AND OBJECTIVE: Mesial temporal lobe epilepsy with hippocampal sclerosis is the most common cause of refractory epilepsy, and the most common indication for surgery. Although effective, surgery fails in up to 40% of patients. The objective of our study was to establish a correlation between the different histological subtypes of mesial temporal lobe epilepsy with hippocampal sclerosis and the prognosis, seizures control, side effects and anticonvulsivant drug withdrawal in patients with refractory epilepsy. PATIENTS AND METHOD: Clinical histories and anatomopathological specimens of 228 patients with temporal epilepsy surgically obtained at our hospital between 1993 and 2014 were retrospectively analysed. All patients underwent a standard preoperative evaluation and anterior temporal resection (modified from Spencer). The anatomopathological study included the standard hematoxylin-eosin and immunohistochemical protocol, with special interest in the assessment of neuronal loss with NeuN. Seizure control was assessed according to the scale of results of the ILAE and Engel. The mean follow-up was 8.6 years (2-19). RESULTS: At 10 years after the intervention, 67.9% of patients were seizure-free (ILAE 1) and as many as 77.5% of the patients were seizure-free (Engel 1) at the end of the follow-up. The probability of not having a seizure (ILAE 1) after surgery at 2 (p=.042), 5 (p=.001) and 7 years (p=.22) was higher in classic and severe forms compared to isolated sclerosis CA1 and CA4 forms. Higher neuronal loss measured with the NeuN immunostain in CA1 was associated with better outcome in seizure management (multivariate analysis, p=.08). The presence of a personal history of epilepsy was associated with greater neuronal loss in CA1 (p=.028) and CA3 (p=.034), and the presence of psychic auras was related with greater neuronal loss in CA3 (p=.025). In our case, the probability of medication withdrawal was related to the presence of personal history (p=.003) and, inversely, to neuronal loss in CA1 (p=.036) and CA3 (p=.038). The greatest impairment of verbal memory occurred in those patients with a lower neuronal loss in CA1 (p=.023), CA2 (p=.049) and CA3 (p=.035). CONCLUSIONS: The results indicate that the classical and severe subtypes have a better prognosis in the control of seizures against the atypical forms, validating the clinical and prognostic utility of the classification of histological subtypes of hippocampal sclerosis from the ILAE. The value of the immunohistochemistry in the mesial temporal lobe epilepsy with hippocampal sclerosis has been demonstrated as a key element to determine the neuropsychological prognosis and seizure management of the patients after surgery.