非压缩性脊髓病和球蛋白异常。偶然的联想?3例新病例讨论]。

M Boyer, M Barat, J M Mazaux, X Debelleix, L Arné
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引用次数: 0

摘要

三例新的脊髓病无压迫与副蛋白血症相关的报告。神经学表现为脊髓性肌萎缩1例,慢性脊髓联合硬化2例。讨论了副蛋白血症的性质:所有三例患者似乎都有良性单克隆球蛋白异常血症。一名患者在发病5年后死于心血管疾病,另一名患者在发病7年后死于与卧位相关的并发症。这两起案件都无法进行尸检。脊髓性肌萎缩在某些癌(肺癌、胃癌、乳腺癌)中有报道,在巨球蛋白血症中较少出现。良性球蛋白异常血症常见于60岁以后,故不能排除偶然性。然而,来自文献的数据和两名患者对癌症化疗的反应表明了一种原始的病理关联。最近与良性副蛋白血症相关的脱髓鞘神经病变的研究为支持这种关联提供了进一步的证据。
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[Noncompressive myelopathy and dysglobulinemia. A fortuitous association? Discussion of 3 new cases].

Three new cases of myelopathy without compression associated with paraproteinemia are reported. The neurologic picture was that of spinal muscular atrophy in one case and chronic spinal combined sclerosis in two. The nature of the paraproteinemia is discussed: all three patients seem to have had benign monoclonal dysglobulinemia. One patient died from cardiovascular disease five years after onset, and another from complications related to decubitus seven years after onset. Postmortem examination was not possible in either case. Spinal muscular atrophy has been reported in certain carcinomas (lung, stomach, breast) and, less frequently, in macroglobulinemia. As benign dysglobulinemia is common after sixty, coincidental association cannot be outruled. However, data from the literature and the response to cancer chemotherapy in two patients suggest an original pathologic association. Recent demonstration of demyelinating neuropathies associated with benign paraproteinemia provide further evidence in support of such an association.

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