Department of Pediatrics, "Pugliese-Ciaccio" Hospital, Catanzaro, Italy.
Unit of Pediatrics, Magna Graecia University of Catanzaro.
Acta Biomed. 2020 Sep 7;91(3):e2020087. doi: 10.23750/abm.v91i3.10157.
Hashimoto encephalopathy (HE) is a rare but controversial entity encompassing a variety of neuropsychological presentations in the setting of autoimmune thyroid disease. HE, mostly described in adults, with a female‑to‑male ratio of 4:1, is a relatively rare entity in the pediatric population and probably under recognized as a cause of acute encephalopathy in children and adolescents. A number of pathogenetic mechanisms have been suggested. Female prevalence, presence of autoantibodies, fluctuating course, and response to immunomodulatory therapy suggest the autoimmune nature of the disease. Existing diagnostic criteria for adults require modification to be applied to children and adolescents, who differ from adults in their clinical presentations, clinical findings, autoantibody profiles, treatment response, and long-term outcomes. A combination of neurological findings, positive antithyroid autoantibodies, and responsiveness to steroids is diagnostic of HE. We add a new case of HE in an adolescent girl and review the current HE literature.
桥本脑病(HE)是一种罕见但有争议的疾病实体,在自身免疫性甲状腺疾病的背景下表现出多种神经心理表现。HE 主要见于成人,男女比例为 4:1,在儿科人群中相对较少见,可能未被认识为儿童和青少年急性脑病的病因。已经提出了许多发病机制。女性患病率、自身抗体存在、波动性病程和对免疫调节治疗的反应提示疾病的自身免疫性质。现有的成人诊断标准需要修改以适用于儿童和青少年,他们在临床表现、临床发现、自身抗体谱、治疗反应和长期结局方面与成人不同。结合神经系统表现、抗甲状腺自身抗体阳性和对类固醇的反应可诊断为 HE。我们增加了一例青少年女性 HE 的新病例,并回顾了目前的 HE 文献。