ABSTRACT
INTRODUCTION: Human T-lymphotropic virus 1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a prevalent disease in certain tropical regions endemic for HTLV-1, being a rare entity in areas such as Europe and North America. CASE REPORTS: We report two new cases of HAM/TSP in Caucasians, native from Galicia, Spain. Serum and cerebrospinal fluid (CSF) analysis, clinical neurophysiologic studies and brain and spinal cord MRI scans were performed. Both patients presented a progressive chronic myelopathy, evolving to spastic paraparesis; one of them presenting with uveitis, prior to the onset of neurological symptoms. CSF analysis revealed mild lymphocytic pleocytosis and increased protein concentration with positive anti-HTLV-1 antibodies. Polymerase chain reaction was positive for HTLV-1. Oligoclonal bands were not detected. In one of the patients, MRI scans did not reveal abnormalities whilst in the other there was an elongated high intensity lesion at the thoracic spinal cord level, which resolved after treatment. No evidence of peripheral neuropathy was found. Corticosteroids and interferon alpha therapy was started, with moderate functional improvement. A history of unprotected sexual relationships while travelling to HTLV-1 endemic areas was revealed. CONCLUSIONS: HTLV-1-associated uveitis may predict HAM/TSP. HAM/TSP is probably an underdiagnosed disease due to the high prevalence of asymptomatic carriers, insidious clinical presentation and low suspicion index in non-endemic regions for HTLV-1. In non-tropical countries, HAM/TSP should not only be suspected in migrants from endemic areas for HTLV-1, but also in patients from communities with a tradition of migration to tropical countries.
TITLE: Paraparesia espastica tropical en una region no tropical.Introduccion. El virus linfotropo humano de celulas T tipo 1 (HTLV-1) es el agente causal de la paraparesia espastica tropical. Su prevalencia, elevada en determinadas areas tropicales, es baja en Europa y Norteamerica. Casos clinicos. Se describen dos casos de paraparesia espastica tropical en varones naturales y residentes en Galicia. Se realizaron estudios analiticos en la sangre y el liquido cefalorraquideo (LCR), examenes neurofisiologicos y resonancia magnetica craneal y medular. En ambos pacientes, la presentacion clinica fue la de una mielopatia cronica, con cuadro torpido y progresivo que evoluciono a paraparesia espastica. Un paciente desarrollo uveitis antes de la clinica neurologica. En los dos casos, el estudio del LCR demostro leve pleocitosis linfoide, ligera hiperproteinorraquia, bandas oligoclonales negativas y anticuerpos anti-HTLV-1 positivos. La reaccion en cadena de la polimerasa para HTLV-1 resulto positiva en ambos casos. La resonancia magnetica raquidea resulto normal en un paciente y mostro en el otro hiperseñal medular dorsal, que desaparecio tras el tratamiento. No se demostraron datos de polineuropatia periferica. Recibieron corticoides e interferon alfa, con leve mejoria y estabilizacion del cuadro clinico. La anamnesis dirigida revelo antecedentes de contactos sexuales de riesgo en regiones endemicas de HTLV-1. Conclusiones. La uveitis asociada a HTLV-1 podria ser predictora de paraparesia espastica tropical. Esta es probablemente una entidad infradiagnosticada (alto porcentaje de portadores asintomaticos, clinica insidiosa y bajo indice de sospecha en areas no endemicas). Debe considerarse su diagnostico en zonas no tropicales que reciben inmigrantes de areas endemicas y tambien en regiones con una tradicional emigracion a regiones tropicales.
Subject(s)
Paraparesis, Tropical Spastic , Humans , Male , Middle Aged , Paraparesis, Tropical Spastic/diagnosis , Paraparesis, Tropical Spastic/drug therapy , SpainABSTRACT
El fallo hepático agudo neonatal es una entidad poco frecuente, con una mortalidad muy elevada. La sospecha de fallo hepático ante situaciones de mal estado general y disfunción hepática, hipoglucemia recurrente o persistente y clínica de sepsis es fundamental para establecer un tratamiento precoz y efectivo. Existen pruebas de primera y segunda línea para poder orientar un diagnóstico etiológico, a la vez que se instauran medidas generales que permitan su estabilización, antes de considerar otras medidas terapéuticas, como el trasplante hepático. Presentamos el inicio y la evolución de 2 casos de fallo hepático neonatal diagnosticados de hemocromatosis y linfohistiocitosis hemofagocítica familiar (AU)
Acute neonatal liver failure is a rare entity with a high mortality rate. In order to establish an early and effective treatment, a high index of suspicion in newborns with a poor general condition and hepatic dysfunction, recurrent or persistent hypoglycaemia and signs and symptoms of sepsis, is critical. There are first- and second-line test to establish the aetiology, parallel to the implementation of general stabilization measures, and prior to the consideration of other therapeutic options such as liver transplantation. We present the onset and the outcome of two patients affected from acute neonatal liver failure that were diagnosed of hemochromatosis and hemophagocytic lymphohistiocytosis, respectively (AU)
