Brennan Declan, Murphy Olwen C, Fearon Conor, Brett Francesca, Murray Brian, Lynch Tim
Centre for Brain Health Dublin Neurological Institute at the Mater Misericordiae University Hospital Dublin Ireland.
Division of Neuroimmunology Johns Hopkins Hospital Baltimore Maryland USA.
Mov Disord Clin Pract. 2020 Jan 7;7(2):223-227. doi: 10.1002/mdc3.12883. eCollection 2020 Feb.
We describe the clinical and neuropathological features of a patient with T-cell-mediated paraneoplastic limbic encephalitis, parkinsonism, hypothermia, and narcolepsy-like presentation associated with endometrial carcinoma.
This patient with prominent parkinsonism and narcolepsy broadens the phenotype of known paraneoplastic syndromes and demonstrates the importance of investigation for occult malignancy even in the absence of paraneoplastic antibodies.
This is a case report with diagnosis confirmed at postmortem.
Paraneoplastic antibodies were not detected. The initial improvement with immunosuppression was short lived, and postmortem neuropathological examination demonstrated encephalitis with predominant T-cell infiltration affecting the hypothalamus and extending to the brainstem, suggestive of a paraneoplastic syndrome.
Although the possibility of a novel antibody cannot be ruled out, consideration must also be given to recent demonstration of purely T-cell-mediated neuronal destruction in the context of paraneoplastic syndromes.
我们描述了一名患有T细胞介导的副肿瘤性边缘叶脑炎、帕金森综合征、体温过低和发作性睡病样表现且与子宫内膜癌相关的患者的临床和神经病理学特征。
这名具有显著帕金森综合征和发作性睡病的患者拓宽了已知副肿瘤综合征的表型，并证明了即使在没有副肿瘤抗体的情况下对隐匿性恶性肿瘤进行检查的重要性。
这是一份尸检确诊的病例报告。
未检测到副肿瘤抗体。免疫抑制治疗初期的改善是短暂的，尸检神经病理学检查显示为脑炎，主要是T细胞浸润，累及下丘脑并延伸至脑干，提示为副肿瘤综合征。
虽然不能排除存在新型抗体的可能性，但在副肿瘤综合征的背景下，也必须考虑到最近关于纯粹T细胞介导的神经元破坏的研究结果。
