Muñoz-Osores Elizabeth, Sánchez Ortiz Natali, Andresen Vásquez Max, Ávila-Smirnow Daniela, Valle Muñoz Patricio, Barriga Gonzaga José
Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Pontificia Universidad Católica de Chile, Santiago, Chile.
Rev Chil Pediatr. 2020 Dec;91(6):941-946. doi: 10.32641/rchped.vi91i6.1665. Epub 2020 Sep 24.
Acute necrotizing encephalopathy of childhood (ANEC) is a rare disease characterized by alteration of consciousness and multiple symmetric brain lesions mainly involving the thalamus. It presents a high mortality rate and severe sequelae.
To describe a school-age patient with influenza A-related ANEC with favorable evolution.
Six-year-old boy with 3 days history of upper respiratory symptoms and fever (39 °C). One day previous to admission, he presented altered state of consciousness. A lumbar puncture was performed, showing a mild increase of protein level in CSF. MRI showed bilateral foci of symmetric restricted signal in the thalamus, mammillary bodies, periaqueductal gray, ventral tegmentum, hippocampus, and in both external capsules, which was compatible with ANEC. The patient received empirical treatment with methylprednisolone and oseltamivir. Subsequently, a positive result was received for influenza. Considering diagnosis and severity of illness, it was decided to administer immunoglobulin. The patient got better slowly but favorably. At discharge, he still was mildly bradypsychic with decreased visual acuity, spontaneous speech and walking with assistance. At 6 months of follow-up, the patient presented normal speech and gait, with persistent visual impairment in the right eye.
Our patient presented ANEC, whose timely diagnosis and management were associated with a favorable neurological evolution in the long term. Although ANEC is an infrequent pathology, it has very high morbidity and mortality rates, so it is very important to have a high degree of suspicion in order to request a targeted imaging study, search for related infectious causes, and start proper treatment.
儿童急性坏死性脑病(ANEC)是一种罕见疾病,其特征为意识改变和主要累及丘脑的多发性对称性脑损伤。它具有高死亡率和严重后遗症。
描述一名患甲型流感相关ANEC且病情转归良好的学龄期患者。
一名6岁男孩,有3天上呼吸道症状和发热(39℃)病史。入院前一天,他出现意识状态改变。进行了腰椎穿刺,脑脊液蛋白水平轻度升高。磁共振成像(MRI)显示丘脑、乳头体、导水管周围灰质、腹侧被盖区、海马以及双侧外囊有双侧对称的信号受限灶,符合ANEC表现。患者接受了甲泼尼龙和奥司他韦的经验性治疗。随后,流感检测结果呈阳性。考虑到诊断和病情严重程度,决定给予免疫球蛋白治疗。患者恢复缓慢但情况良好。出院时,他仍有轻度精神迟缓,视力下降,能自发讲话,需借助辅助行走。在随访6个月时,患者语言和步态正常,但右眼仍有持续性视力损害。
我们的患者患有ANEC,其及时诊断和治疗与长期良好的神经功能转归相关。尽管ANEC是一种罕见疾病,但其发病率和死亡率非常高,因此高度怀疑以进行针对性影像学检查、寻找相关感染病因并开始适当治疗非常重要。