Department of Pediatrics, The Warren Alpert Medical School, Brown University, Providence, Rhode Island
School of Medicine, Duke University, Durham, North Carolina.
Hosp Pediatr. 2021 Jul;11(7):743-750. doi: 10.1542/hpeds.2020-005298. Epub 2021 Jun 8.
Patients with autoimmune encephalitis (AE) often present with symptoms that are broadly characterized as psychiatric or behavioral, yet little attention is given to the precise symptomatology observed. We sought to more fully define the psychiatric symptoms observed in patients with anti-N-methyl-D-aspartate receptor (NMDAR), anti-glutamic-acid-decarboxylase 65 (GAD65), and anti-voltage-gated-potassium-channel complex (VGKC) antibody-mediated AE using the nomenclature.
We present a case series ( = 25) using a retrospective chart review of 225 patients evaluated for AE in a tertiary care academic medical center between 2014 and 2018. The included patients were ≤18 years old with anti-NMDAR AE ( = 13), anti-GAD65 AE ( = 7), or anti-VGKC AE ( = 5). The frequency of neuropsychiatric symptoms present at the onset of illness and time to diagnosis were compared across groups.
Psychiatric symptoms were seen in 92% of patients in our cohort. Depressive features (72%), personality change (64%), psychosis (48%), and catatonia (32%) were the most common psychiatric symptoms exhibited. On average, patients experienced impairment in ≥4 of 7 symptom domains. No patients had isolated psychiatric symptoms. The average times to diagnosis were 1.7, 15.5, and 12.4 months for anti-NMDAR AE, anti-GAD65 AE, and anti-VGKC AE, respectively ( < .001).
The psychiatric phenotype of AE in children is highly heterogenous. Involving psychiatry consultation services can be helpful in differentiating features of psychosis and catatonia, which may otherwise be misidentified. Patients presenting with psychiatric symptoms along with impairments in other domains should prompt a workup for AE, including testing for all known antineuronal antibodies.
自身免疫性脑炎（AE）患者常表现出广泛特征为精神或行为的症状，但对观察到的精确症状学关注甚少。我们试图通过使用术语更全面地定义抗 N-甲基-D-天冬氨酸受体（NMDAR）、抗谷氨酸脱羧酶 65（GAD65）和抗电压门控钾通道复合物（VGKC）抗体介导的 AE 患者观察到的精神症状。
我们进行了病例系列研究（=25），对 2014 年至 2018 年间在一家三级保健学术医疗中心接受 AE 评估的 225 例患者进行了回顾性图表审查。纳入的患者年龄均≤18 岁，包括抗 NMDAR AE（=13）、抗 GAD65 AE（=7）或抗 VGKC AE（=5）。比较了各组患者疾病发作时出现的神经精神症状的频率和诊断时间。
在我们的队列中，92%的患者存在精神症状。最常见的精神症状为抑郁特征（72%）、人格改变（64%）、精神病（48%）和紧张症（32%）。平均而言，患者在≥7 个症状领域中均存在损伤。没有患者仅有孤立的精神症状。抗 NMDAR AE、抗 GAD65 AE 和抗 VGKC AE 的平均诊断时间分别为 1.7、15.5 和 12.4 个月（<0.001）。
儿童 AE 的精神病表型高度异质。精神科会诊服务的参与有助于区分精神病和紧张症的特征，否则这些特征可能会被误诊。伴有其他领域损伤的精神病症状患者应促使进行 AE 检查，包括对所有已知神经元抗体进行检测。
