Gregory Timothy A, Jones Gillian N, Loh Yince
Department of Medicine, Neurology - Madigan Army Medical Center, Tacoma, Washington, USA
Department of Neurology, Darnall Army Medical Center, Fort Hood, Texas, USA.
BMJ Case Rep. 2019 Nov 21;12(11):e230011. doi: 10.1136/bcr-2019-230011.
There are many examples in the literature of Hashimoto's encephalopathy (HE) presenting with heterogeneous manifestations to include stroke-like episodes, seizures, myoclonus and psychiatric symptoms. The pathogenesis is poorly understood but is thought to involve an autoimmune-mediated vasculitis. Here, we present a novel case showing hemispheric hyperaemia which created a diagnostic challenge and insinuated a vascular mechanism for the condition. The patient presented with left-sided stroke-like symptoms and had head CT angiography notable for asymmetric vasculature initially interpreted radiographically as decreased left middle cerebral artery (MCA) flow. An MRI brain demonstrated right-sided holohemispheric fluid-attenuatedinversion recovery (FLAIR) hyperintensity with right insula contrast enhancement. She was found to have elevated anti-thyroid peroxidase (TPO) antibodies with an otherwise negative encephalitis workup. The patient was diagnosed with HE and acutely progressed to have focal seizures during a prolonged intensive care unit stay. She ultimately required intravenous Ig and antiepileptic medications to gain control of her disease. This case appears to be the first described presentation of hemiencephalitis with local hyperaemia, and may represent local autoregulatory loss as a result of vasculitis. This supports the existing literature implicating inflammatory microvascular infiltration in the mechanism of the disease. HE must be considered in a broad range of unexplained neurological symptoms.
文献中有许多桥本脑病（HE）表现出异质性症状的例子，包括类中风发作、癫痫、肌阵挛和精神症状。其发病机制尚不清楚，但被认为涉及自身免疫介导的血管炎。在此，我们报告一例新病例，该病例显示半球充血，这带来了诊断挑战，并暗示了该病的血管机制。患者出现左侧类中风症状，头部CT血管造影显示血管不对称，最初影像学解释为左侧大脑中动脉（MCA）血流减少。脑部MRI显示右侧全半球液体衰减反转恢复（FLAIR）高信号，右侧岛叶有对比增强。发现患者抗甲状腺过氧化物酶（TPO）抗体升高，而其他脑炎检查结果为阴性。该患者被诊断为HE，在重症监护病房长时间住院期间急性进展为局灶性癫痫发作。她最终需要静脉注射免疫球蛋白和抗癫痫药物来控制病情。该病例似乎是首例描述的伴有局部充血的半侧脑炎，可能代表血管炎导致的局部自动调节丧失。这支持了现有文献中关于炎症性微血管浸润参与该病机制的观点。对于广泛的不明原因神经症状，必须考虑到HE。
