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罕见的混合性痴呆:一例报告。

Rare mixed dementia: A case report.

作者信息

Chen Xu-Hui, Xia Wen, Ma Jia-Bin, Chen Jiao, Hu Jun, Shi Xin, Yu Jing-Jing, Gong Jia, Liu Lu, Sun Yong-An, Liu Zhi-Gang

机构信息

Department of Neurology, Peking University First Hospital, Beijing 100034, China.

Department of Neurology, Peking University Shenzhen Hospital, Shenzhen 518000, Guangdong Province, China.

出版信息

World J Radiol. 2025 Jan 28;17(1):102579. doi: 10.4329/wjr.v17.i1.102579.

Abstract

BACKGROUND

Autoimmune encephalitis (AE) is a rare and recently described neuroinflammatory disease associated with specific autoantibodies. Anti-leucine-rich glioma inactivated 1 (anti-LGI1) encephalitis is a rare but treatable type of AE discovered in recent years. Alzheimer's disease (AD) is a degenerative brain disease and the most common cause of dementia. AD may undergo a series of pathological physiological changes in brain tissue 20 years before the onset of typical symptoms. The stage of mild cognitive impairment (MCI) that occurs during this process, known as MCI due to AD, is the earliest stage with clinical symptoms. MCI is typically categorized into two subtypes: Amnestic MCI (aMCI) and non-aMCI.

CASE SUMMARY

This report describes a patient with rapid cognitive impairment, diagnosed with anti-LGI1 antibody-mediated AE and aMCI, and treated at Peking University Shenzhen Hospital in March 2023. The patient was hospitalized with acute memory decline for more than 3 months. Both the cerebrospinal fluid and serum were positive for anti-LGI1 antibodies, biomarkers of AD coexisting in the patient's cerebrospinal fluid. Following combination treatment with immunoglobulin therapy and glucocorticoid, plus inhibition of acetylcholinesterase, the patient's cognitive function significantly improved. Throughout the 3-month follow-up period, a sustained improvement in cognitive function was observed. The results of serum anti-LGI1 antibody were negative.

CONCLUSION

This case has raised awareness of the possible interaction between AE and early AD (including MCI due to AD), and alerted clinicians to the possibility of concurrent rare and common diseases in patients presenting with cognitive impairment.

摘要

背景

自身免疫性脑炎(AE)是一种罕见的、最近才被描述的与特定自身抗体相关的神经炎症性疾病。抗富含亮氨酸胶质瘤失活1(anti-LGI1)脑炎是近年来发现的一种罕见但可治疗的AE类型。阿尔茨海默病(AD)是一种退行性脑疾病,也是痴呆最常见的病因。AD在典型症状出现前20年,脑组织可能会经历一系列病理生理变化。在此过程中出现的轻度认知障碍(MCI)阶段,即因AD导致的MCI,是最早出现临床症状的阶段。MCI通常分为两种亚型:遗忘型MCI(aMCI)和非遗忘型MCI。

病例摘要

本报告描述了一名认知功能快速减退的患者,于2023年3月在北京大学深圳医院确诊为anti-LGI1抗体介导的AE和aMCI并接受治疗。该患者因急性记忆减退住院3个多月。脑脊液和血清中anti-LGI1抗体均为阳性,患者脑脊液中还存在AD的生物标志物。经过免疫球蛋白治疗、糖皮质激素联合乙酰胆碱酯酶抑制治疗后,患者认知功能显著改善。在3个月的随访期内,认知功能持续改善。血清anti-LGI1抗体结果转为阴性。

结论

该病例提高了对AE与早期AD(包括因AD导致的MCI)之间可能存在相互作用的认识,并提醒临床医生,认知功能受损患者可能同时患有罕见病和常见疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e0b/11755906/2a47bf79fae4/102579-g001.jpg

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