Crisis Convulsivas Asociadas a Síndrome Poliglandular Autoinmune

Abstract

Polyglandular autoimmune syndrome (SPA) is a rare disorder characterized by the coexistence of at least two glandular insufficiencies mediated immune mechanisms, together with other diseases not endocrinological it. The objective of this research is to describe the clinical presentation and the flaws in the health system that impede the timely treatment of these patients, this case is a teenage female patient 17 years old with a history of primary adrenal insufficiency and hypothyroidism primary diagnosed since age 7, which required several hospitalizations due to decompensation of the underlying pathology; as family history has consanguinity of parents (first cousins), maternal grandmother with diabetes; twin sister diagnosed with adrenal insufficiency and epilepsy, who dies at age 7 by their condition; gynecological history menarche at age 11 with irregular menstrual cycles every 3-5 months, amenorrhea over 16 years. As usual medication consume prednisone 5 mg QD, levothyroxine 100 ug QD, income family relates my having tingling in the arms, lower limb pain and seizure generalized 24 hours earlier, involuntary movements of upper and lower limbs is observed deviation glance the right, relaxation of sphincters, recover spontaneously, and left with paresthesias in upper limbs, lower limbs muscle pain with spasmodic muscular contraction that prevents full extension; hospitalization tonic clonic seizure new box about 5 minutes, without relaxation of sphincters with spontaneous recovery is observed. In addition to the physical examination Chevostek positive sign, upper limbs, Trossseau positive sign, lower limb muscle strength IV / V, negative Babinsky was found; with these symptoms the patient goes emergency service to Internal Medicine Hospital IESS of Latacunga, where several complementary tests showing change in values electrolyte is conducted, several imagenlógicos studies performed after which the group of diseases presenting is cataloged from childhood to today as autoimmune polyglandular syndrome type I, restoring itself compensator treatment of their endocrinological disorders.