背景:桥本脑病有多种临床表现,除了甲状腺抗体的存在,实验室和影像学检查都是非特异性的.泰国桥本脑病患者的临床表现和治疗结果的具体数据仍然很少。
目的:回顾性调查Siriraj医院桥本脑病患者的临床表现和治疗结果。
方法:对2012年7月至2017年3月在我们中心就诊的急性脑病患者进行资格评估。纳入标准为血清抗甲状腺过氧化物酶(抗TPO)或抗甲状腺球蛋白(抗Tg)阳性,血清或脑脊液(CSF)神经元抗体阴性。临床表现,症状持续时间,甲状腺状态和甲状腺自身抗体的实验室结果,CSF研究,并收集临床结果.
结果:在204例出现脑病的患者中,31(15.2%)对于抗TPO或抗Tg抗体是阳性的。其中,13例符合桥本脑病诊断标准。临床表现包括认知障碍(76.9%),意识模糊(46.2%),和行为改变(30.8%)。神经精神表现为视觉幻觉(30.8%),幻听(15.4%),妄想(7.7%),和情绪障碍(23.1%)。其他临床表现包括癫痫发作(38.5%),异常运动(23.1%),睡眠障碍(38.5%),共济失调(46.2%),中风样发作(15.4%),和发烧(15.4%)。大多数患者(76.9%)在<3个月内发病。关于结果,1例未接受皮质类固醇的患者死于癫痫持续状态和感染性休克。在接受皮质类固醇治疗的12例患者中,9人(75%)有显著改善,1(8.3%)略有改善,2例(16.6%)无临床改善。7例(53.9%)甲状腺功能正常,4例患者(30.8%)有亚临床甲状腺功能减退症,2例患者(15.4%)有亚临床甲状腺功能亢进。
结论:这项研究的结果揭示了认知障碍,神经精神症状,癫痫发作,共济失调,睡眠障碍是桥本脑病的常见表现。在亚急性发作的无法解释的认知障碍或小脑共济失调的个体中,应始终考虑这种情况。实验室和神经影像学检查均发现桥本脑病无特异性。大多数患者对治疗反应良好,因此,临床怀疑和早期诊断和治疗将导致改善患者的结果。
BACKGROUND: Hashimoto encephalopathy has multiple clinical presentations, and other than the presence of thyroid antibody, laboratory and imaging investigations are all non-specific. Data specific to the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy in Thailand remain scarce.
OBJECTIVE: To retrospectively investigate the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy at Siriraj Hospital.
METHODS: Patients who presented with acute encephalopathy at our center during July 2012-March 2017 were evaluated for eligibility. The inclusion criteria were positive anti-thyroperoxidase (anti-TPO) or anti-thyroglobulin (anti-Tg) in serum with negative neuronal antibody in serum or cerebral spinal fluid (CSF). Clinical presentations, symptom duration, laboratory results of thyroid status and thyroid autoantibody, CSF study, and clinical outcomes were collected.
RESULTS: Of the 204 patients who presented with encephalopathy, 31 (15.2%) were positive for the anti-TPO or anti-Tg antibody. Of those, 13 patients met the diagnostic criteria for Hashimoto encephalopathy. Clinical presentations included cognitive impairment (76.9%), clouding of consciousness (46.2%), and behavior change (30.8%). The neuropsychiatric presentations were visual hallucination (30.8%), auditory hallucination (15.4%), delusion (7.7%), and mood disturbance (23.1%). Other clinical presentations included seizure (38.5%), abnormal movement (23.1%), sleep disturbance (38.5%), ataxia (46.2%), stroke-like episode (15.4%), and fever (15.4%). Most patients (76.9%) had onset within < 3 months. Regarding outcomes, 1 patient who did not receive corticosteroid died from status epilepticus and septic shock. Among the 12 patients who received corticosteroid, 9 (75%) had marked improvement, 1 (8.3%) had slight improvement, and 2 (16.6%) had no clinical improvement. Seven patients (53.9%) had normal thyroid function, 4 patients (30.8%) had subclinical hypothyroidism, and 2 patients (15.4%) had subclinical hyperthyroidism.
CONCLUSIONS: The results of this study revealed cognitive impairment, neuropsychiatric symptoms, seizure, ataxia, and sleep disturbance to be common manifestations of Hashimoto encephalopathy. This condition should always be considered in individuals with subacute onset of unexplained cognitive impairment or cerebellar ataxia. Laboratory and neuroimaging investigations were all found to be nonspecific in Hashimoto encephalopathy. Most patients responded well to treatment, so clinical suspicion and early diagnosis and treatment will lead to improved patient outcomes.