科布综合征1例新病例报告。脑膜-脊髓皮肤血管瘤病]。

Acta neurologica latinoamericana Pub Date : 1981-01-01
S Gordon-Firing, J A Purriel, D Pereyra, I Brodbek
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引用次数: 0

摘要

本文报告一位47岁的女性患者,患有胸椎部位的皮脑脊膜血管瘤病或Cobb综合征。这种实体,包括主要的中胚层淋巴瘤,是极其罕见的,只有17例在文献中被充分记录。在这个病人中出现了罕见的与椎体血管瘤具有相同特异功能水平的关联。本文讨论了诊断标准、可能的关联、病理生理学和分类学情况。强调了毛细血管扩张类型的血管瘤和主要的硬膜外位置。作者总结了临床怀疑这种诊断的重要性,这种诊断是基于脊髓综合征的皮肤血管瘤的存在相同的代谢水平。一个完整的临床旁研究的必要性,其中包括选择性脊髓血管造影和脊柱的线性断层扫描也强调。有了这种方法,这种综合征的真实发病率将被揭示,允许有计划的手术治疗,这将减少到目前为止公布的病例的高死亡率和发病率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Report of a new case of Cobb syndrome. Meningo-spinal cutaneous angiomatosis].

The authors present a 47 years old female patient with cutaneomeningospinal angiomatosis or Cobb's syndrome of thoracic location. This entity, included in the primarily mesodermic phacomatose, is extremely rare, and only 17 cases have been sufficiently documented in the literature on the subject. The rare association with vertebral haemangioma of the same metameric level was present in this patient. The diagnostic criteria, possible associations, pathophysiology and nosological situation are discussed. The telangiectasic type of angioma and the principally extradural location are stressed. The authors concluded on the importance of clinical suspicion of this diagnosis based on a spinal cord syndrome in the presence of a cutaneous angioma of the same metameric level. The necessity of a complete paraclinical studies which includes selective spinal angiography and linear tomography of the spine are also stressed. With this methodology the real incidence of this syndrome will be revealed, permitting a planned surgical treatment which will diminish the high mortality and morbidity of the cases published up to the present.

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