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脑淋巴瘤样肉芽肿病:一例报告

Lymphomatoid granulomatosis of the brain: A case report.

作者信息

Quinones Edgardo, Potes Laura I, Silva Nhora, Lobato-Polo Javier

机构信息

Physician, Intensive Care Unit, Fundación Valle del Lili, Cali, Colombia.

Physician, CIDEIM, International Biomedical Research Center, Cali, Colombia.

出版信息

Surg Neurol Int. 2016 Sep 1;7(Suppl 23):S612-6. doi: 10.4103/2152-7806.189732. eCollection 2016.

Abstract

BACKGROUND

Lymphomatoid granulomatosis is a rare disorder of the central nervous system (CNS) with few cases being reported in literature. We present the case of an adult with an unusual lesion of the CNS who presented with motor seizures and was diagnosed with lymphomatoid granulomatosis, followed by a discussion of the process of evaluation and management.

CASE DESCRIPTION

A 42-year-old male presented with motor seizures and loss of consciousness for 10 minutes along with dysarthria and left hemiplegia. Neurological examination and imaging with magnetic resonance imaging (MRI) of the brain revealed a mass in the right striatum. The patient was hospitalized and underwent an image-guided right frontal craniotomy using the Leksell Stereotactic G-Frame. Pathology reported a lymphomatoid granulomatosis. Being immunocompetent, the patient received medical treatment with prednisone and rituximab. Two years after his diagnosis, the patient had no active disease and his brain MRI did not show contrast enhancement. After almost 3 years of follow-up, the patient has a mild weakness in the left-side of his body (4/5), is seizure-free, and can walk and perform daily activities.

CONCLUSIONS

This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.

摘要

背景

淋巴瘤样肉芽肿病是一种罕见的中枢神经系统疾病,文献报道的病例较少。我们报告一例患有中枢神经系统异常病变的成年患者,该患者表现为运动性癫痫发作,被诊断为淋巴瘤样肉芽肿病,随后讨论评估和管理过程。

病例描述

一名42岁男性出现运动性癫痫发作和意识丧失10分钟,伴有构音障碍和左侧偏瘫。神经系统检查及脑部磁共振成像(MRI)显示右侧纹状体有一个肿块。患者住院并使用Leksell立体定向G型框架进行了影像引导下的右额开颅手术。病理报告为淋巴瘤样肉芽肿病。由于患者免疫功能正常,接受了泼尼松和利妥昔单抗的药物治疗。诊断两年后,患者无活动性疾病,脑部MRI未显示对比增强。经过近3年的随访,患者左侧身体轻度无力(4/5级),无癫痫发作,能够行走并进行日常活动。

结论

在一名免疫功能正常的成年患者中,这种以运动性癫痫发作为首发症状的罕见病变在诊断和治疗方面具有挑战性。经过手术和药物治疗,患者恢复良好。文中讨论了临床特征、影像学表现、鉴别诊断和病理学情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd15/5025951/151ce6df139c/SNI-7-612-g001.jpg

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