primary immunodeficiencies

autoimmune encephalitis

World Allergy Organization Journal

This article was originally published online on 8 April 2015 Encephalitis is defined by the presence of brain inflammation associated with clinical evidence of neurological dysfunction. It can be due to infection (most common cause is viruses) or not (like post vaccine or auto immune). Primary immunodeficiency is defined as a genetic basis that leads any alteration in immune system (innate or adaptative) predisposing to infections, auto immunity and malignancies. Combined immunodefiency,alteration in T and B cell function, are potentially fatal disease. Review of clinical data from eletronic records.The objective of this paper is relate a case of a patient with encephalitis and our difficulty in establishing the etiology L.S.N., 25 years old female patient followed by combined immunodeficiency presented with acute arthritis, nodosum erythema and acute seizures. The liquor analysis evidenced lymphocitic pleocitosis with elevated protein levels. No alterations were evidenciated in brain CT. The patient underwent an uncontrolled epileptic status that required intubation,sedation, broad-spectrum antibiotics, anti-fungal, anti-viral, tuberculostatic agents and corticosteroids. Despite all clinical investiment the patient had a fatal cardiac arrest. Establish specific sorological etiology of clinical manifestations in primary immunodeficiencies is essential but a chalenge in clinical practice. New techniques in the diagnosis of infections and autoimmune conditions are necessary.

Undefined and fatal meningoenchephalitis in a patient with combined immune deficiency: a case report