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[表现为可能初发的多发性硬化的假瘤性延髓综合征。临床、放射学及神经病理学相关性]

[Pseudotumoral medullary syndrome manifesting a probably beginning multiple sclerosis. Clinical, radiological and neuropathological correlations].

作者信息

Reznik M, Franck G, Flandroy P, Lenelle J

机构信息

Service de Neuropathologie, Université de Liège.

出版信息

Acta Neurol Belg. 1994;94(1):8-16.

PMID:8140889
Abstract

A 19-y old sportsman was admitted because he complained of ascending paresthesia since two weeks, muscular strength diminution in both arms, then respiratory difficulties without fever. Clinical examination confirmed an asymmetric bilateral pyramidal syndrome affecting the four limbs, a hyposensitive level up to C5, without meningeal symptoms. MRI detected a widening of the cervical (C2-C5) spinal cord, with gadolinium enhancement. CSF demonstrated lymphocytic pleocytosis and total protein of 530 mg/l with an oligoclonal IgG pattern. With a diagnosis of multiple sclerosis, a corticoid therapy was started but without clinical improvement. Six weeks later, a new MRI revealed an increased enlargement of the cervical spinal cord suggesting a tumoral process. No tumor could be detected at neurosurgery. Biopsy specimen (from C3-C4) demonstrated an inflammatory demyelinating disease with astrocytic hypertrophy. Electron microscopy confirmed the active demyelinating disorder with persistently denuded axons. Several macrophages were observed, but no lymphocytic invasion. Astrocytes were prominent in some areas with "en plaque" gliosis. MRI failed to discover any other demyelinating lesions. Further clinical evaluation was inconspicuous, the patient regaining full activities after 6 months. MRI after 9 months demonstrated no spinal cord lesion. This observation contributes to suggest the influence of the blood-brain barrier breakdown on the occurrence of clinical and radiological signs of new lesions in suspected multiple sclerosis.

摘要

一名19岁的运动员因两周来出现上行性感觉异常、双臂肌力减弱,随后出现呼吸困难且无发热症状而入院。临床检查证实为不对称性双侧锥体束综合征,累及四肢,感觉减退平面达C5,无脑膜刺激症状。磁共振成像(MRI)检测到颈段(C2 - C5)脊髓增宽,钆增强。脑脊液显示淋巴细胞增多,总蛋白为530mg/l,呈寡克隆IgG模式。诊断为多发性硬化后,开始使用皮质类固醇治疗,但临床症状无改善。六周后,新的MRI显示颈段脊髓增宽加剧,提示肿瘤性病变。神经外科手术未发现肿瘤。活检标本(取自C3 - C4)显示为伴有星形细胞肥大的炎性脱髓鞘疾病。电子显微镜证实为活动性脱髓鞘疾病,轴突持续裸露。观察到多个巨噬细胞,但无淋巴细胞浸润。星形细胞在某些区域突出,伴有“斑块状”胶质增生。MRI未发现任何其他脱髓鞘病变。进一步的临床评估无明显异常,患者6个月后恢复了全部活动能力。9个月后的MRI显示脊髓无病变。该观察结果提示血脑屏障破坏对疑似多发性硬化新病变的临床和影像学表现的发生有影响。

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