An Unusual Case of Central Nervous System Lymphoma Presenting With Ataxic Quadriparesis Showing ‘Wine glass’-Like Appearance

B. Ahn, H. Go, Kyum-Yil Kwon
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Abstract

The wine glass sign on brain magnetic resonance imaging (MRI) appears as symmetrical corticospinal tract hyperintensities on coronal sections in T2-weighted imaging. The differential diagnoses of the classical wine glass-like appearance include amyotrophic lateral scle-rosis and osmotic myelinolysis. 1,2 However, to the best of our knowledge, this sign has not been reported in other brain conditions, including tumors. Herein we describe an unusual case presenting with progressive ataxic quadriparesis over several months in a patient with a wine glass-like appearance in brain MRI without gadolinium enhancement. The patient was finally diagnosed as primary central nervous system (CNS) lymphoma. A 71-year-old female presented with recurrent falls accompanied by general weakness. Two months previously she had insidiously developed gait difficulty with postural instabil-ity that worsened progressively, resulting in her not being able to walk independently. A neurological examination revealed quadriparesis (4/5 muscle strength) of the motor system in all extremities, whereas sensory function showed no abnormalities. A mild-to-moderate de-gree of cerebellar ataxia was noted in all extremities. Her deep tendon reflexes were normo-active and her upper motor neuron signs were not remarkable. There were no symptoms of cognitive decline. Routine laboratory tests including of electrolytes produced normal findings. Serologic tests, including a test for vasculitis and an enzyme-linked immunosorbent assay for human immunodeficiency virus, were unremarkable. Brain MRI including axial fluid-attenuated inversion recovery (FLAIR) sequences showed slightly asymmetrical white-matter-dominant hyperintensities extending from the pons up to the corona radiata, al-though with some marginal lesions involving the thalamus and basal ganglia (Fig. 1A). Es-pecially notable was coronal T2-weighted imaging showing bilateral
罕见的中枢神经系统淋巴瘤,表现为共济失调性四肢瘫,呈“酒杯”样外观
脑磁共振成像(MRI)上的葡萄酒杯征表现为冠状面t2加权成像上对称的皮质脊髓束高信号。典型的葡萄酒杯样外观的鉴别诊断包括肌萎缩性侧索硬化和渗透性髓鞘溶解。然而,据我们所知,这一迹象还没有出现在其他脑部疾病中,包括肿瘤。在此,我们描述了一个不寻常的病例,在几个月的时间里,患者出现了进行性共济性四肢瘫,在大脑MRI中表现为葡萄酒杯样外观,没有钆增强。患者最终被诊断为原发性中枢神经系统淋巴瘤。一名71岁女性,表现为反复跌倒并伴有全身无力。两个月前,她出现了步态困难和姿势不稳,并逐渐恶化,导致她不能独立行走。神经学检查显示四肢瘫(4/5肌肉力量),而感觉功能未见异常。四肢均有轻度至中度小脑共济失调。她的深肌腱反射正常活跃,她的上运动神经元征象不显著。没有认知能力下降的症状。包括电解质在内的常规实验室检查结果正常。血清学测试,包括血管炎测试和人类免疫缺陷病毒的酶联免疫吸附试验,没有什么特别之处。包括轴向液体衰减反转恢复(FLAIR)序列在内的脑部MRI显示,从脑桥向上延伸至辐射冠的白质为主的轻度不对称高信号,尽管有一些边缘病变累及丘脑和基底节区(图1A)。特别值得注意的是冠状t2加权成像显示双侧
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