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伴有进行性认知衰退的桥本脑病:中美洲一种罕见的自身免疫性疾病。

Hashimoto's Encephalopathy With Progressive Cognitive Decline: A Rare Autoimmune Disorder in Central America.

作者信息

Hong Anthony, Santos Ana B, Moya Porras Luis Fernando, Piedra Pacheco Ana Lucía, Hong Isaac, Esquivel Jose E, Villegas José D

机构信息

Faculty of Medicine, University of Costa Rica, San José, CRI.

Department of Endocrinology, Hospital San Juan de Dios, San José, CRI.

出版信息

Cureus. 2025 Apr 30;17(4):e83235. doi: 10.7759/cureus.83235. eCollection 2025 Apr.

DOI:10.7759/cureus.83235
PMID:40453306
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12123474/
Abstract

Hashimoto's encephalopathy (HE) is a rare autoimmune disorder, typically associated with Hashimoto's thyroiditis, and often presents with rapidly progressive cognitive impairment, psychiatric symptoms, and neurological deficits. Since the first case reported, there have been relatively few reports of HE cases, and it remains rare in Central America. A 59-year-old male patient with a past medical history of hypertension (HTN) and type 2 diabetes mellitus (DM2) was hospitalized due to a one-month history of rapidly progressive cognitive impairment associated with behavioral changes and episodes of amnesia. It was not associated with motor or sensory deficits, seizures, or movement disorders. Given this presentation, alongside normal head CT findings and mild age-related brain atrophy on MRI, the initial diagnosis posed a challenge. Further investigations, including cerebrospinal fluid (CSF) analysis, autoimmune panels, and viral encephalitis testing, helped exclude other potential causes of encephalopathy. Notably, elevated anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies were detected, strongly suggesting HE. The patient responded favorably to immunosuppressive treatment with intravenous methylprednisolone and subsequent oral prednisone, leading to significant cognitive improvement and recovery of function. This case highlights the importance of considering HE in the differential diagnosis of rapidly progressive cognitive decline, especially when routine standard investigations do not reveal an alternative cause.

摘要

桥本脑病(HE)是一种罕见的自身免疫性疾病,通常与桥本甲状腺炎相关,常表现为快速进展的认知障碍、精神症状和神经功能缺损。自首例病例报告以来,HE病例的报道相对较少,在中美洲地区仍然罕见。一名59岁男性患者,有高血压(HTN)和2型糖尿病(DM2)病史,因1个月来快速进展的认知障碍伴行为改变和失忆发作而住院。该症状与运动或感觉功能缺损、癫痫发作或运动障碍无关。鉴于此临床表现,以及头颅CT检查结果正常和MRI显示轻度与年龄相关的脑萎缩,初步诊断颇具挑战性。进一步检查,包括脑脊液(CSF)分析、自身免疫指标检测和病毒性脑炎检测,有助于排除其他潜在的脑病病因。值得注意的是,检测到抗甲状腺过氧化物酶(抗-TPO)和抗甲状腺球蛋白(抗-TG)抗体升高,强烈提示为HE。该患者对静脉注射甲泼尼龙及随后口服泼尼松的免疫抑制治疗反应良好,认知功能显著改善,功能得以恢复。本病例强调了在快速进展性认知衰退的鉴别诊断中考虑HE的重要性,尤其是在常规标准检查未发现其他病因时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d09b/12123474/cb6f3a2c377f/cureus-0017-00000083235-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d09b/12123474/cec3a896dba4/cureus-0017-00000083235-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d09b/12123474/cb6f3a2c377f/cureus-0017-00000083235-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d09b/12123474/cec3a896dba4/cureus-0017-00000083235-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d09b/12123474/cb6f3a2c377f/cureus-0017-00000083235-i02.jpg

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