Encefalitis de Von Economo / Von Economo Encephalitis

Introducción: Constantin von Economo reportó en 1917 múltiples casos de manifestaciones neurológicas secundarias a la pandemia de la gripe española, clasificándolos en tres grandes grupos: forma somnolienta-oftalmopléjica, mutismo y la hipercinética, con secuelas similares a la enfermedad de Parkinson. Objetivo: presentar un caso de reciente aparición de patología rara en Cali, Colombia con manejo adecuado en unidad de cuidados intensivos (UCI). Presentación del caso: paciente de 9 años con disminución de la fuerza en extremidades, disartria y somnolencia, que inició deterioro neurológico progresivo requiriendo manejo en UCI. El equipo multidisciplinario diagnosticó encefalitis letárgica e iniciaron manejo con plasmaféresis e inmunosupresión con mejoría significativa. Discusión y conclusiones: como la prevalencia es escasa, el diagnóstico exige un alto índice de sospecha como la ocurrencia de un cuadro infeccioso previo al inicio de los síntomas, ya que se considera una reacción autoinmune cruzada contra antígenos de la sustancia nigra. En algunos casos hay alteraciones en los estudios imagenológicos o en citoquímico de líquido cefalorraquídeo. El manejo con pulsos de metilprednisolona y filtración de plasma con plasmaféresis brinda mejoría significativa con disminución de las secuelas a futuro.

Introduction: In 1917, Constantin von Economo reported multiple cases of neurological manifestations secondary to the Spanish flu pandemic. He classified them into three main clinical forms: somnolent-ophthalmoplegic, mutism and hyperkinetic, causing sequelae resembling Parkinson ́s disease. Objective: to present a case of a recent appearance rare disease entity, in Cali Colombia, receiving appropriate management in the Intensive Care Unit (ICU). Case presentation: 9-year-old patient presenting with limb muscle weakness, dysarthria and somnolence, evidencing progressive neurological deterioration requiring admission to the ICU for management. A diagnosis of encephalitis lethargica (EL) was made by the attending multidisciplinary team and management with plasmapheresis and immunosuppression was started, obtaining significant improvement. Discussion and conclusions: as the prevalence is low, the diagnosis requires a high level of suspicion in cases presenting with infectious conditions prior to the development of symptoms, since it is considered an autoimmune cross-reaction against substantia nigra antigens. Alterations in brain imaging or in cerebrospinal fluid cytometry may be found in some cases. Management with methylprednisolone pulse therapy and filtration plasmapheresis provides significant improvement with a decrease in future sequelae.

Impact of pharmacokinetics to reduce bleeding in a cohort of patients with severe hemophilia A in a personalized comprehensive management program.

In recent decades, hemophilia A treatment has been focused on body weight, without taking pharmacokinetic parameters into account. Previous research has shown that the individual pharmacokinetic response is more effective in predicting the required dose of clotting factor. We want to evaluate the impact on reducing the frequency of bleeding in patients treated with recombinant factor VIII, based on a personalized comprehensive management program. Our aim was to compare the results of a standard comprehensive treatment program (stage I) vs. a personalized pharmacokinetic - based treatment program (stage II) in a cohort of 60 patients with severe hemophilia without inhibitors. The median age was 15.5 years (3-68). The annual bleeding rate (ABR) was 1.03 (62 episodes) in the first stage and 0.58 (35 episodes) in the second one, (p=0.004). By type of bleeding, the impact of the intervention differs significantly in spontaneous bleeding (p=0.007) and a 73% reduction in the first stage. There were no significant differences in traumatic bleeding. The use of pharmacokinetics (PK) for personalized dosing of patients with severe hemophilia A, significantly reduces ABR and spontaneous bleeding, improving the patient's quality of life and costs for the health system.