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[脱髓鞘疾病的假瘤样形式。三例报告及文献复习]

[Pseudotumor forms of demyelinating diseases. Report of three cases and review of the literature].

作者信息

de Lacour A, Guisado F, Zambrano A, Argente J, Acosta J, Ramos C

机构信息

Sección de Neurología, Hospital Universitario Puerta del Mar, Cádiz, España.

出版信息

Rev Neurol. 1998 Dec;27(160):966-70.

PMID:9951014
Abstract

INTRODUCTION AND CLINICAL CASES

We present three patients with unusual clinical findings studied in our hospital, after a period of follow-up of at least two years from the time of diagnosis of their demyelinating condition. We discuss the clinical onset, CT and magnetic resonance findings, neuropathological studies and posterior clinical course. Anatomopathological studies were done in two of the cases, since the diagnoses were not clear and the other paraclinical investigations did not clarify things.

DISCUSSION

The presence of large space-occupying lesions or ring-like outlines in myelinating disorders may make it difficult to make a differential diagnosis from other conditions such as neoplasias and abscesses. This may lead to an erroneous initial diagnosis and even to the use of unsuitable, aggressive treatments.

CONCLUSIONS

In young patients in whom radiological imaging suggests space-occupying lesions (single or multiple) one should consider the possibility of a primary demyelinating disease of the central nervous system with the appearance of a pseudo-tumour. In certain cases, stereotaxic biopsy should be considered if the diagnosis remains in doubt, rather than begin unsuitable treatment. The fundamental reason for the presentation of our cases is to emphasize that these pseudo-tumoral forms of demyelinating diseases should be considered in diagnosis.

摘要

引言与临床病例

我们呈现了三名在我院接受研究的患者,自其脱髓鞘疾病诊断之时起,经过了至少两年的随访。我们讨论了临床起病情况、CT及磁共振成像结果、神经病理学研究以及后续临床病程。对其中两例进行了解剖病理学研究,因为诊断不明确且其他辅助检查未能阐明情况。

讨论

在髓鞘形成障碍中出现大的占位性病变或环状轮廓可能难以与诸如肿瘤和脓肿等其他病症进行鉴别诊断。这可能导致初始诊断错误,甚至使用不适当的积极治疗方法。

结论

对于影像学检查提示有占位性病变(单发或多发)的年轻患者,应考虑中枢神经系统原发性脱髓鞘疾病伴假肿瘤表现的可能性。在某些情况下,如果诊断仍存疑问,应考虑进行立体定向活检,而不是开始不适当的治疗。我们展示这些病例的根本原因是强调在诊断中应考虑这些脱髓鞘疾病的假肿瘤形式。

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