Department of Pediatrics, The Warren Alpert Medical School of Brown University, Providence, Rhode Island.
Department of Pediatrics, Duke University, Durham, North Carolina; Department of Psychiatry, Duke University, Durham, North Carolina.
Pediatr Neurol. 2020 Jun;107:41-47. doi: 10.1016/j.pediatrneurol.2019.12.011. Epub 2020 Feb 8.
The recently proposed adult diagnostic criteria for the Hashimoto encephalopathy (HE) include a requirement of subclinical or mild thyroid disease. However, most reports indicate that most children treated for HE do not have evidence of thyroid disease. We aim to evaluate the impact of applying the current adult diagnostic criteria to pediatric patients.
Pediatric patients with HE were evaluated at time of symptom onset and follow-up at least 1 year after initiation of immunomodulatory treatment for degree of impairment within the neuropsychiatric domains of cognition, language, psychiatric disturbance, seizure, movement disorder, sleep disruption, and overall functionality. We compared the response to treatment among patients stratified by the presence or absence of subclinical or mild thyroid disease using the Modified Rankin Scale, the Liverpool Outcome Score, and a novel multidomain scale designed for the population with pediatric autoimmune brain disorders.
Of 17 pediatric patients treated for HE, 6 met full adult diagnostic criteria, whereas 11 patients did not meet criteria solely owing to the absence of thyroid disease. Using our novel scale, the 6 patients meeting full criteria had statistically significant improvement from time of onset of disease to follow-up in the domain of cognition. The 11 patients who did not meet full criteria based on their absence of thyroid disease exhibited statistically significant improvement from time of onset of disease to follow-up in the domains of cognition, language, psychiatric disturbance, movement, and sleep.
Rigidly applying the current diagnostic criteria to pediatric patients with suspected HE may result in the failure to treat potential responders. We propose a set of diagnostic criteria for HE in children, which does not require thyroid disease but include abrupt onset cognitive regression with deficits in one or more other neuropsychiatric domains in the setting of antithyroid antibodies.
最近提出的桥本脑病 (HE) 成人诊断标准包括亚临床或轻度甲状腺疾病的要求。然而，大多数报告表明，大多数接受 HE 治疗的儿童没有甲状腺疾病的证据。我们旨在评估将当前成人诊断标准应用于儿科患者的影响。
在开始免疫调节治疗后至少 1 年，对出现症状时和随访时的 HE 儿科患者进行评估，评估认知、语言、精神障碍、癫痫发作、运动障碍、睡眠障碍和整体功能等神经精神领域的损伤程度。我们比较了根据亚临床或轻度甲状腺疾病的存在与否分层的患者在使用改良 Rankin 量表、利物浦结局量表和为儿科自身免疫性脑疾病人群设计的新型多领域量表的治疗反应。
在 17 名接受 HE 治疗的儿科患者中，有 6 名符合成人诊断标准，而 11 名患者仅因甲状腺疾病缺失而不符合标准。使用我们的新型量表，符合完整标准的 6 名患者在疾病发作到随访期间的认知域中具有统计学显著改善。基于缺乏甲状腺疾病而不符合完整标准的 11 名患者在认知、语言、精神障碍、运动和睡眠等领域的疾病发作到随访期间均有统计学显著改善。
严格将当前诊断标准应用于疑似 HE 的儿科患者可能会导致潜在反应者得不到治疗。我们提出了一组儿童 HE 的诊断标准，该标准不要求存在甲状腺疾病，但包括抗甲状腺抗体背景下急性认知性回归和一个或多个其他神经精神领域的缺陷。
