Myofibroblastic tumor (MIT) is characterized by the infiltration of different organs, most commonly the lungs, with nodular lesions composed of myofibroblasts and inflammatory cells, which can be identified by specific patterns in the immunohistochemical studies. When it involves the peritoneum it is difficult to eradicate, tends to relapse and it has an invasive behavior, requiring its differentiation from peritoneal carcinomatosis. Treatment may be surgical excision, the use of non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids. We report a 30 years old female with an unremarkable medical history, presenting with abdominal pain and progressive abdominal distention. A CT scan revealed multiple peritoneal nodular lesions. A surgical biopsy was reported as a myofibroblast and inflammatory cell infiltrate. Immunohistochemical staining was consistent with MIT. Given the extensive involvement of the peritoneum surgical therapy was not considered appropriate and treatment with NSAID and glucocorticoids was started. No response was observed after 6 months, therefore infliximab therapy was started. After 10 months of follow-up the patient is well, returned to normal life, ascites improved and resolved and CT scan showed partial regression or stabilization of the lesions.
Los síndromes de falsas identificaciones delirantes consisten en percepciones erróneas de estímulos externos con una creencia o elaboración asociada que se sostiene con una intensidad delirante. Dentro de ellos el signo de la TV consiste en que el paciente imagina que los eventos televisivos observados están ocurriendo en un espacio tridimensional real, es una manifestación poco frecuente en pacientes con demencia. Presentamos una serie de 7 casos clínicos de pacientes con demencia que han manifestado el signo de la TV durante la pandemia COVID-19. La edad promedio fue de 77 años. Dos pacientes tuvieron diagnóstico de demencia tipo Alzheimer, dos de demencia tipo Alzheimer atípico, uno de demencia vascular y tres de causa mixta (2 con demencia tipo Alzheimer + vascular y 1 demencia con cuerpos de Lewy + vascular). Tres presentaron otros síntomas psicóticos y uno se asoció también con la presencia de síndrome de Capgras. Esta serie de casos plantea la posibilidad de un aumento de la incidencia del signo de la TV en pacientes con demencia durante la pandemia precipitado por el aumento de la exposición a dispositivos con pantallas y el aislamiento social durante este período.
Background Rapidly progressive dementia (RPD) is a syndrome caused by different disease entities. Accurate diagnosis is crucial as numerous proportions of these diseases are highly treatable. There are few RPD series published in South America. Knowing regional data may be useful to establish specific diagnostic guidelines based on local information. The main study objectives are to describe the etiological diagnosis of patients with RPD in Argentina and to compare the clinical characteristics and data obtained from complementary studies among patients with Autoimmune Encephalitis (AE), Prion Diseases (PD), Neurodegenerative Diseases (ND) and Other Causes (OC). Method A multicenter observational cross‐sectional study was performed with the information obtained from patients referred to the neurology department of the participant centers. Patients with RPD criteria (developing dementia in less than 24 months) were retrospectively included between Dec‐2001 and Jun‐2019. Based on International diagnostic criteria, patients were divided into: AE, PD, ND and OC. We compared baseline clinical data, complementary exams, MRI and serologic characteristics between groups. Parametric or non‐parametric statistic and multivariate analysis was used according to the sample distribution of the available data. Result On a sample of 328 patients with a diagnosis of RPD, 54.87% were men (n=180) with a mean age of 60.78±5.57 years at admission. The diagnosis time for dementia was 4.06 (0.83–23.6) months. The three most frequent causes were AE 23.17% (n=76), ND 22.86% (n=75) and PD 15.85% (n = 52). In 15.85% of the patients the diagnosis remained unknown. When comparing AE, ND, PD and OC, statistically significant differences were observed in age (younger age for AE, p=0.0001), higher mortality for PD (p=0.0001) and larger time of evolution in ND (p=0.0001). In the PD group more behavioral symptoms (p = 0.002) and pathological electroencephalogram (p=0.001) were found. Conclusion In accordance with similar series, autoimmune etiology was the more frequent cause. This is the first South American RPD multicentric study. These results might supply valuable data for the healthcare system and future epidemiological investigations more focused diagnostic guidelines.
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