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多发性骨髓瘤侵犯中枢神经系统。

Multiple myeloma invasion of the central nervous system.

作者信息

Schluterman Keith O, Fassas Athanasios B-T, Van Hemert Rudy L, Harik Sami I

机构信息

Department of Neurology, University of Arkansas for Medical Sciences, 4301 W. Markham Street, Little Rock, AR 72205, USA.

出版信息

Arch Neurol. 2004 Sep;61(9):1423-9. doi: 10.1001/archneur.61.9.1423.

Abstract

BACKGROUND

Although neurologic manifestations often complicate the course of patients with multiple myeloma (MM), direct central nervous system invasion is rare.

OBJECTIVE

To describe the neurologic symptoms and signs, imaging, cerebrospinal fluid findings, and the clinical course of patients with central nervous system myeloma invasion, all of whom had leptomeningeal myelomatosis.

DESIGN AND PARTICIPANTS

Review of 23 patients with MM and leptomeningeal myelomatosis proven by malignant plasma cells in their cerebrospinal fluid.

SETTING

Tertiary-care university medical center.

RESULTS

Twenty-one patients had advanced-stage MM. Leptomeningeal myelomatosis was diagnosed up to 29 months (median, 13 months) after diagnosis of MM. Symptoms precipitating neurologic evaluation included manifestations of diffuse cerebral dysfunction, cranial nerve palsies, and spinal radiculopathies. Cerebrospinal fluid was abnormal in all patients, usually exhibiting pleocytosis and elevated protein content, plus positive cytologic findings. Specific magnetic resonance imaging findings suggestive of central nervous system invasion were found in 70% of the patients. These included leptomeningeal contrast enhancement and evidence of meningeal-based lesions sometimes masquerading as intraparenchymal lesions. Despite aggressive systemic and local treatment, the outcome was poor, reflecting the aggressiveness of the underlying MM.

CONCLUSION

Leptomeningeal myelomatosis, although rare, should be considered in patients with MM and symptoms suggestive of widespread nervous system involvement.

摘要

背景

尽管神经系统表现常使多发性骨髓瘤(MM)患者的病程复杂化,但中枢神经系统直接侵犯很少见。

目的

描述中枢神经系统骨髓瘤侵犯患者的神经系统症状和体征、影像学表现、脑脊液检查结果及临床病程,所有患者均患有柔脑膜骨髓瘤病。

设计与参与者

回顾23例脑脊液中发现恶性浆细胞而确诊为MM和柔脑膜骨髓瘤病的患者。

地点

三级医疗大学医学中心。

结果

21例患者为晚期MM。柔脑膜骨髓瘤病在MM诊断后29个月(中位时间13个月)内被诊断出来。促使进行神经学评估的症状包括弥漫性脑功能障碍、颅神经麻痹和脊髓神经根病的表现。所有患者的脑脊液均异常,通常表现为细胞增多、蛋白含量升高以及细胞学检查结果呈阳性。70%的患者发现了提示中枢神经系统侵犯的特定磁共振成像表现。这些表现包括柔脑膜对比增强以及有时伪装成脑实质内病变的脑膜性病变证据。尽管进行了积极的全身和局部治疗,但结果仍很差,这反映了潜在MM的侵袭性。

结论

柔脑膜骨髓瘤病虽然罕见,但对于有MM且有提示广泛神经系统受累症状的患者应予以考虑。

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