Background: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. Objectives: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. Methods: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. Results: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2–39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy ( p = 0.001), seizures ( p = 0.045), and leptomeningeal enhancement ( p = 0.045). Conclusion: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes.
RESUMEN El tumor fibroso solitario de pleura es una neoplasia poco frecuente. La asociación de hipoglicemia como manifestación paraneoplásica con este tipo de tumor, se conoce como Síndrome de Doege-Potter. La hipoglicemia es secundaria a la secreción tumoral de una forma aberrante de IGF-II, que estimula de manera permanente al receptor de insulina. La resección quirúrgica completa es el tratamiento de elección, que se asocia con la curación instantánea de la hipoglicemia y bajo riesgo de recurrencia. Se presenta el caso de una mujer de 55 años de edad, que acudió al servicio de emergencia por presentar episodios recurrentes de hipoglicemia. A su ingreso, los análisis mostraron hipoglicemia severa y niveles bajos de insulina y péptido C. La radiografía de tórax mostró una opacidad homogénea que ocupaba casi todo el hemitórax derecho, y la tomografía computada, una tumoración heterogénea con bordes bien definidos en el mismo hemitórax. La paciente fue sometida a una toracotomía abierta con resección completa del tumor. El estudio histológico fue compatible con tumor fibroso solitario pleural. La paciente no volvió a presentar otro episodio de hipoglicemia, siendo dada de alta. PALABRAS CLAVE: Hipoglucemia, tumor fibroso solitario pleural, síndromes paraneoplásicos (Fuente: DeCS BIREME). SUMMARY The solitary fibrous tumor of the pleura is an infrequent neoplasm. Hypoglycemia is a paraneoplastic manifestation of this tumor and it is named the Doege-Potter Syndrome. Hypoglycemia results from the aberrant tumoral secretion of IGF-II that permanently stimulates the insulin receptor. Surgical removal of the tumor is the treatment of choice and results in immediate resolution of hypoglycemia with low risk of recurrence. We present the case of a 55-year-old female patient who attended the emergency room with recurrent episodes of hypoglycemia. On admission, the laboratory results revealed severe hypoglycemia and low levels of insulin and C-peptide. Chest x-ray showed a homogenous opacity that affected almost the entire right hemithorax. The CT-scan showed a heterogeneous tumor with no well-defined borders. An open thoracotomy with complete resection of the tumor was performed. Histopathological findings were compatible with solitary fibrous tumor of the pleura. Hypoglycemic episodes resolved and the patient was discharged.
Billiary tract involvement in the course of disseminated histoplasmosis has been rarely reported. Here we present a severely immunosuppressed HIV-infected patient who presented with symptomatic acalculous cholecystitis caused by Histoplasma capsulatum.
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