Endres Dominique, Vry Magnus S, Dykierek Petra, Riering Anne N, Lüngen Eva, Stich Oliver, Dersch Rick, Venhoff Nils, Erny Daniel, Mader Irina, Meyer Philipp T, Tebartz van Elst Ludger
Section for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Department of Neurology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Front Psychiatry. 2017 Oct 26;8:212. doi: 10.3389/fpsyt.2017.00212. eCollection 2017.
Hashimoto's encephalopathy (HE) is a rare immunological neuropsychiatric disorder characterized by increased antithyroid antibodies and mixed neurological and psychiatric symptoms. HE has been previously discussed as a differential diagnosis for rapid progressive dementia. However, most of these patients suffered from additional neurological symptoms, like ataxia or seizures.
Here, we present the case of a 59-year-old female patient suffering rapid onset dementia with salient frontal executive dysfunction. She developed rapid onset symptoms, including apathy, verbal depletion up to a stuporous state, severe working memory deficits, evidence of primitive reflexes, disturbed Luria's three-step test, and micturition disorder. Analysis of her cerebrospinal fluid was normal. The serum analyses showed increased antithyroid (antithyroid peroxidase and antithyroglobulin) antibodies. In the cerebral magnetic resonance imaging, supratentorial deep and peripheral white matter lesions were found; the electroencephalography showed intermittent slowing, and the [F]fluorodeoxyglucose positron emission tomography (FDG-PET) depicted medial and superior dorsolateral frontal hypometabolism. Several different psychopharmacological therapeutic approaches with various neuroleptics, antidepressants, and high doses of lorazepam were unsuccessful. Due to the organic alterations, including increased antithyroid antibodies, HE was suspected. Against expectations, treatment with high-dose corticosteroids proved to be ineffective and was associated with worsening symptoms. However, escalated treatment with plasmapheresis over 5 days led to significant improvement in all reported symptoms and in psychometric testing. The neuropsychological improvement was stable over a 6-month follow-up period, and the FDG-PET normalized.
This case report reveals that (1) HE can mimic rapid onset dementia with predominantly frontal dysfunction; (2) this syndrome can be successfully treated in the context of HE; and (3) plasmapheresis can be effective in such a disease constellation. The detection of the immunological causes of rapid onset dementia and other psychiatric syndromes is important because it opens opportunities for new, innovative immunosuppressive treatment options.
桥本脑病（HE）是一种罕见的免疫性神经精神疾病，其特征为抗甲状腺抗体升高以及混合性神经和精神症状。HE此前一直被作为快速进展性痴呆的鉴别诊断进行讨论。然而，这些患者中的大多数还伴有其他神经症状，如共济失调或癫痫发作。
在此，我们报告一例59岁女性患者，其患有快速起病的痴呆，并伴有明显的额叶执行功能障碍。她出现了快速起病的症状，包括淡漠、言语减少直至昏迷状态、严重的工作记忆缺陷、原始反射、卢里亚三步试验异常以及排尿障碍。她的脑脊液分析结果正常。血清分析显示抗甲状腺（抗甲状腺过氧化物酶和抗甲状腺球蛋白）抗体升高。在脑部磁共振成像中，发现幕上深部和外周白质病变；脑电图显示间歇性减慢，而[F]氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）显示内侧和背外侧额叶上部代谢减低。使用多种抗精神病药物、抗抑郁药物以及高剂量劳拉西泮的几种不同心理药物治疗方法均未成功。由于存在包括抗甲状腺抗体升高在内的器质性改变，怀疑为HE。出乎意料的是，高剂量皮质类固醇治疗无效且症状加重。然而，连续5天进行血浆置换强化治疗后，所有报告症状及心理测试均有显著改善。神经心理学改善在6个月的随访期内保持稳定，且FDG-PET恢复正常。
本病例报告表明：（1）HE可模拟以额叶功能障碍为主的快速起病痴呆；（2）该综合征在HE背景下可成功治疗；（3）血浆置换在这种疾病组合中可能有效。检测快速起病痴呆和其他精神综合征的免疫病因很重要，因为这为新的、创新的免疫抑制治疗选择提供了机会。
