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[与白质脑病相关的快速进展性痴呆:脑淋巴瘤病一例报告]

[Rapidly progressive dementia associated with leukoencephalopathy: A case report of lymphomatosis cerebri].

作者信息

Vences Miguel A, Saravia-Ayala Adriana F, Barreto-Acevedo Elliot

机构信息

Hospital Nacional Edgardo Rebagliati Martins, Essalud, Lima, Perú. Dirección: Departamento de Neurología, Hospital Nacional Edgardo Rebagliati Martins, Av. Rebagliati 490, Jesús María, Lima, Perú. Email:

Hospital Nacional Edgardo Rebagliati Martins, Essalud, Lima, Perú. ORCID: 0000-0001-6570-5640.

出版信息

Medwave. 2021 Mar 26;21(2):e8138. doi: 10.5867/medwave.2021.02.8138.

Abstract

INTRODUCTION

Lymphomatosis cerebri is a rare form of primary central nervous system lymphoma characterized by an atypical clinical presentation and neuroimaging, with a poor short-term prognosis.

CASE REPORT

A 51-year-old woman began with clinical manifestations characterized by rapidly progressing cognitive impairment associated with a behavioral disorder, myoclonus, and gait disturbance. The brain magnetic resonance image showed extensive signaling in the bilateral periventricular white matter. The 18F-FDG PET-CT showed severe dorsolateral neocortical hypometabolism in the absence of focal hypermetabolic lesion, a metabolic pattern indicative of cerebri lymphomatosis. A brain biopsy confirmed the diagnosis. The patient started chemotherapy achieving complete remission. Eighteen months after diagnosis, the patient had improved clinically and neuroimaging.

CONCLUSION

This is the first report in Peru of an entity that should be considered in rapidly progressive dementia and leukoencephalopathy cases. Timely diagnosis and appropriate chemotherapy management can increase patient survival.

摘要

引言

脑淋巴瘤病是原发性中枢神经系统淋巴瘤的一种罕见形式,其临床表现和神经影像学表现不典型,短期预后较差。

病例报告

一名51岁女性开始出现以快速进展的认知障碍为特征的临床表现,伴有行为障碍、肌阵挛和步态障碍。脑部磁共振成像显示双侧脑室周围白质广泛信号改变。18F-FDG PET-CT显示在无局灶性高代谢病变的情况下,背外侧新皮质严重代谢减低,这种代谢模式提示脑淋巴瘤病。脑活检确诊了该诊断。患者开始化疗并实现完全缓解。诊断后18个月,患者的临床症状和神经影像学表现均有所改善。

结论

这是秘鲁首例关于在快速进展性痴呆和白质脑病病例中应考虑的一种疾病的报告。及时诊断和适当的化疗管理可提高患者生存率。

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