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临床病例分析:一名19岁进行性神经功能衰退且伴有多处颅内病变的女性

Clinical Problem-Solving: A 19-Year-Old Woman With Progressive Neurological Decline and Multiple Intracranial Lesions.

作者信息

Ardakani Rumyar, Hatanpaa Kimmo, De Los Sanotos Yanel, Hardeman Paula, Tardo Lauren

机构信息

Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.

Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA.

出版信息

Neurohospitalist. 2024 Oct;14(4):419-422. doi: 10.1177/19418744241273283. Epub 2024 Aug 13.

Abstract

The differential diagnosis for multiple intracranial lesions in a young adult is broad and includes demyelinating, neoplastic, and infectious etiologies. In this report, we describe the case of a 19-year-old immunocompetent woman presenting with progressive headaches and aphasia. MRI of the brain revealed multiple, large supratentorial lesions with concentric bands of alternating T2 signal intensities and peripheral contrast enhancement. Cerebrospinal fluid (CSF) analysis was overall bland with negative oligoclonal bands. Serum antibody testing for neuromyelitis optica (NMO) and myelin-oligodendrocyte associated disease (MOGAD) were negative. A broad infectious work-up was also unrevealing. A definitive diagnosis was ultimately obtained after brain biopsy and the patient was started on appropriate therapy. This case highlights a diagnostic framework in evaluating immunocompetent patients presenting with multiple intracranial lesions and progressive neurological decline. The main differential diagnoses for this constellation of radiological and clinical findings are discussed and a literature review is performed on the revealed diagnosis. Lastly, both acute and long-term therapeutic approaches are reviewed.

摘要

年轻成年人颅内多发病变的鉴别诊断范围广泛,包括脱髓鞘、肿瘤性和感染性病因。在本报告中,我们描述了一名19岁免疫功能正常的女性,她出现进行性头痛和失语。脑部MRI显示多个大脑幕上大病灶,T2信号强度呈同心带状交替,周边有对比增强。脑脊液(CSF)分析总体无异常,寡克隆带阴性。视神经脊髓炎(NMO)和髓鞘少突胶质细胞相关疾病(MOGAD)的血清抗体检测均为阴性。广泛的感染性检查也未发现异常。最终通过脑活检获得了明确诊断,并开始对患者进行适当治疗。本病例突出了评估出现颅内多发病变和进行性神经功能衰退的免疫功能正常患者的诊断框架。讨论了这一系列影像学和临床发现的主要鉴别诊断,并对所揭示的诊断进行了文献综述。最后,回顾了急性和长期治疗方法。

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