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老年患者的意识混乱和幻觉:罕见鉴别诊断的陷阱:抗 LGI1 脑炎病例报告。

Confusion and Hallucination in a Geriatric Patient. Pitfalls of a Rare Differential: Case Report of an Anti-LGI1-Encephalitis.

机构信息

Internal Medicine, Bürgerspital Solothurn, Solothurn, Switzerland.

Internal Medicine, Salemspital Hirslanden Bern, Bern, Switzerland.

出版信息

Clin Interv Aging. 2022 Sep 26;17:1423-1432. doi: 10.2147/CIA.S380316. eCollection 2022.

Abstract

BACKGROUND

Confusion and hallucinations in geriatric patients are frequent symptoms and typically associated with delirium, late-life psychosis or dementia syndromes. A far rarer but well-established differential in patients with rapid cognitive deterioration, acute psychosis, abnormal movements and seizures is autoimmune encephalitis. Exemplified by our case we highlight clinical and economic problems arising in management of geriatric patients with cognitive decline and psychotic symptoms.

CASE PRESENTATION

A 77-year-old female caucasian patient with an unremarkable medical history was hospitalized after a fall in association with diarrhea and hyponatremia. Upon adequate therapy, disorientation and troubled short-term memory persisted. Within a week the patient developed visual hallucinations. Basic blood and urine samples and imaging (cranial computed tomography and magnetic resonance imaging) were unremarkable. With progressive cognitive decline, amnestic impairment, word finding difficulty and general apathy, psychiatric and neurologic expertise was introduced. Advanced diagnostics did not resolve a final diagnosis; an electroencephalogram showed unspecific generalized slowing. Extended clinical observation revealed visual hallucinations and faciobrachial dystonic seizures. A treatment with anticonvulsants was initiated. Cerebrospinal fluid ultimately tested positive for voltage-gated potassium channel LGl1 (leucine-rich-inactivated-1) antibodies confirming diagnosis of autoimmune anti-LGI1 encephalitis. Immediate immunotherapy (high-dose glucocorticoids and administration of intravenous immunoglobulin G) led to a rapid improvement of the patient's condition. After immunotherapy was tapered, the patient had one relapse and completely recovered with reintroduction of glucocorticoids and initiation of therapy with rituximab.

CONCLUSION

Rapidly progressive dementia in geriatric patients demands a structured and multidisciplinary diagnostic approach. Accurate management and financially supportable care is a major issue in rare diseases such as anti-LGI1-encephalitis. Education and awareness about autoimmune encephalitis of all physicians treating a geriatric population is important in order to involve expertise and establish treatment within reasonable time.

摘要

背景

在老年患者中,意识混乱和幻觉是常见症状,通常与谵妄、老年期精神病或痴呆综合征有关。但在认知迅速恶化、急性精神病、异常运动和癫痫发作的患者中,一种更为罕见但已被充分证实的鉴别诊断是自身免疫性脑炎。通过我们的病例,我们强调了在管理认知能力下降和精神病症状的老年患者时出现的临床和经济问题。

病例介绍

一名 77 岁的白人女性,既往病史无特殊,因腹泻和低钠血症伴跌倒后住院。在适当治疗后,定向障碍和短期记忆障碍持续存在。一周内,患者出现了视幻觉。基本的血液和尿液样本以及影像学(头颅计算机断层扫描和磁共振成像)无明显异常。随着认知能力的逐渐下降、记忆障碍、找词困难和普遍的冷漠,引入了精神科和神经科专家的意见。高级诊断未明确最终诊断;脑电图显示非特异性广泛减速。进一步的临床观察显示出视觉幻觉和面肩肱型张力障碍性癫痫发作。开始使用抗惊厥药物治疗。最终脑脊液检测出电压门控钾通道 LGl1(富含亮氨酸失活 1)抗体阳性,证实诊断为自身免疫性抗 LGI1 脑炎。立即免疫治疗(大剂量糖皮质激素和静脉注射免疫球蛋白 G)使患者病情迅速改善。免疫治疗逐渐减少后,患者复发一次,重新开始使用糖皮质激素和利妥昔单抗治疗后完全恢复。

结论

老年患者迅速进展的痴呆需要进行结构化和多学科的诊断方法。在罕见疾病(如抗 LGI1 脑炎)中,准确的管理和经济上可负担的护理是一个主要问题。治疗老年患者的所有医生都需要了解自身免疫性脑炎,以便在合理的时间内获得专家意见并开始治疗。

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