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[失语症和顶叶综合征作为伴有假瘤样病变的脱髓鞘疾病的首发症状]

[Aphasia and parietal syndrome as the presenting symptoms of a demyelinating disease with pseudotumoral lesions].

作者信息

Navarro S, Mondéjar-Marín B, Pedrosa-Guerrero A, Pérez-Molina I, Garrido-Robres J A, Alvarez-Tejerina A

机构信息

Servicio de Neurología, Hospital Virgen de la Salud. Complejo Hospitalario de Toledo, 45005 Toledo, Spain.

出版信息

Rev Neurol. 2005;41(10):601-3.

Abstract

INTRODUCTION

Multiple sclerosis (MS) often presents with sensory symptoms, which are usually due to spinothalamic or spinal cord disorders; parietal syndrome is, however, very rare as the initial symptom. Likewise, aphasia is also an infrequent symptom of MS; in the few cases that have been reported, it is usually linked to the existence of important pseudotumoral lesions.

CASE REPORT

We describe the case of a 31-year-old female with a 48-hour history of a progressive clinical picture consisting in nominal aphasia and a sensory parietal syndrome. Magnetic resonance imaging showed a lesion 3.6 cm in diameter that was hyperintense in T2 with perilesional edema and minimal gadolinium uptake, along with other images that revealed increased signal intensity in the periventricular subcortical white matter on the right-hand side and in the left-hand frontal subcortical region. A spectroscopic analysis of the largest lesion revealed that this lesion showed evidence of inflammation, with cell destruction and replacement, although it was not possible to distinguish between a demyelinating disease and a high grade glioma. Hence, a brain biopsy was required in order to reach the final diagnosis of demyelinating pseudotumoral lesion.

CONCLUSIONS

Giant pseudotumoral plaques are a rare form of presenting symptom in MS; use of the clinical features, simple images and spectroscopy is not a very reliable means of reaching a differential diagnosis with a tumour and this often makes it necessary to conduct a biopsy study of the lesion.

摘要

引言

多发性硬化症(MS)常伴有感觉症状,通常由脊髓丘脑束或脊髓疾病引起;然而,顶叶综合征作为初始症状非常罕见。同样,失语症也是MS不常见的症状;在已报道的少数病例中,它通常与重要的假瘤性病变的存在有关。

病例报告

我们描述了一名31岁女性的病例,其有48小时渐进性临床表现病史,包括命名性失语和感觉性顶叶综合征。磁共振成像显示一个直径3.6厘米的病变,在T2加权像上呈高信号,周围有水肿,钆摄取极少,同时其他图像显示右侧脑室周围皮质下白质和左侧额叶皮质下区域信号强度增加。对最大病变进行光谱分析显示,该病变有炎症迹象,伴有细胞破坏和替代,尽管无法区分脱髓鞘疾病和高级别胶质瘤。因此,需要进行脑活检以最终诊断为脱髓鞘假瘤性病变。

结论

巨大假瘤性斑块是MS一种罕见的症状表现形式;利用临床特征、简单图像和光谱分析并非可靠的鉴别肿瘤的方法,这往往使得有必要对病变进行活检研究。

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