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胸腺瘤相关副肿瘤性脑炎(TAPE),一种边缘性脑炎的潜在病因。

Thymoma associated paraneoplastic encephalitis (TAPE), a potential cause of limbic encephalitis.

作者信息

Liu Henry, Edson Randall S

机构信息

California Pacific Medical Center, Internal Medicine, San Francisco, California, USA.

Division of Infectious Diseases, California Pacific Medical Center, San Francisco, California, USA.

出版信息

BMJ Case Rep. 2019 Aug 30;12(8):e230709. doi: 10.1136/bcr-2019-230709.

Abstract

A 59-year-old man presents with expressive aphasia and short term memory deficits. Shortly thereafter, he started developing staring spells and intermittent right hand spasms, preliminarily thought to be simple partial seizures. Subsequent MRI brain imaging was highly suggestive of herpes simplex virus (HSV) encephalitis; however, HSV PCR from cerebrospinal fluid was negative. On further testing, the patient was found to have an autoimmune encephalitis thought to be related to an incidentally found thymoma. His clinical presentation, in conjunction with imaging and response to therapy, was strongly suggestive of thymoma associated paraneoplastic encephalitis. Early recognition is the only way to ensure prompt initiation of appropriate treatment. Immunotherapy and cancer directed therapy (including tumour resection, if indicated) have been shown to have favourable outcomes, improved speed of neurological recovery and reduced risk of relapses. Without treatment, progressive neurologic deterioration can occur over months to years, eventually resulting in death.

摘要

一名59岁男性出现表达性失语和短期记忆缺陷。此后不久,他开始出现凝视发作和右手间歇性痉挛,初步认为是单纯部分性癫痫发作。随后的脑部MRI成像高度提示单纯疱疹病毒(HSV)脑炎;然而,脑脊液的HSV聚合酶链反应(PCR)为阴性。进一步检查发现,该患者患有自身免疫性脑炎,被认为与偶然发现的胸腺瘤有关。他的临床表现,结合影像学检查和治疗反应,强烈提示胸腺瘤相关副肿瘤性脑炎。早期识别是确保及时开始适当治疗的唯一方法。免疫疗法和针对癌症的治疗(包括必要时的肿瘤切除)已显示出良好的效果,可提高神经功能恢复速度并降低复发风险。若不进行治疗,数周至数年可能会出现进行性神经功能恶化,最终导致死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bedc/6720759/c1bb39f5e913/bcr-2019-230709f01.jpg

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