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[髓内海绵状血管瘤的自然史。法国多中心研究结果]

[Natural history of intramedullary cavernomas. Results of the French Multicentric Study].

作者信息

Parker F, Lejeune J-P, Bouly S, Lonjon M, Emery E, Proust F, Auque J, Loiseau H, Gallas S, Boetto S, Labauge P

机构信息

Service de neurochirurgie, CHU de Bicêtre, université Paris-XI, 78 rue du Général-Leclerc, 94275 Le-Kremlin-Bicêtre cedex, France.

出版信息

Neurochirurgie. 2007 Jun;53(2-3 Pt 2):208-16. doi: 10.1016/j.neuchi.2007.02.010.

Abstract

INTRODUCTION

The prevalence of cerebral cavernomas is about 0.5% in the general population. In contrast, spinal cord cavernomas are considered as rare. The objective of this study was to determine the natural history of spinal cord cavernomas in a multicentric study.

METHODS

Clinical and neuroradiological findings were retrospectively collected. Diagnosis was based on pathological criteria or magnetic resonance (MR) findings.

RESULTS

Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (range: 11-80). Initial symptoms were progressive (32) and acute myelopathy (20). One patient was asymptomatic. Clinical symptoms were related to spinal cord compression (24) and hematomyelia (19). Cavernoma location was dorsal (41) and cervical (12.). MR findings consisted of hyperintense signal on T1 and T2 sequences (19 cases), mixed hyperintense and hypointense signal (33 cases), and hypointense signal on T1 and T2 sequences in 1 case. Mean size was 16.3 mm (range: 3-54). Forty patients underwent surgical resection. Improvement was observed in 20 patients and worsening of neurological symptoms in 11. Length of follow up was 7.1 years. At the end of the study, 26 patients were autonomous, 18 handicapped and 1 bedridden.

CONCLUSION

This study provided precise data on the clinical and MR patterns of these lesions. The natural history is associated with a higher risk of hemorrhage recurrence, but is favorable in many operated patients. Microsurgery is the treatment of choice for most of these lesions.

摘要

引言

脑海绵状血管瘤在普通人群中的患病率约为0.5%。相比之下,脊髓海绵状血管瘤被认为较为罕见。本研究的目的是在一项多中心研究中确定脊髓海绵状血管瘤的自然病史。

方法

回顾性收集临床和神经放射学检查结果。诊断基于病理标准或磁共振成像(MR)表现。

结果

纳入53例患者(男性26例,女性27例)。症状出现时的平均年龄为40.2岁(范围:11 - 80岁)。初始症状为进行性(32例)和急性脊髓病(20例)。1例患者无症状。临床症状与脊髓压迫(24例)和脊髓出血(19例)有关。海绵状血管瘤位于背部(41例)和颈部(12例)。MR表现包括T1和T2序列上的高信号(19例)、高信号与低信号混合(33例)以及1例T1和T2序列上的低信号。平均大小为16.3 mm(范围:3 - 54 mm)。40例患者接受了手术切除。20例患者症状改善,11例神经症状恶化。随访时间为7.1年。研究结束时,26例患者生活自理,18例患者残疾,1例患者卧床。

结论

本研究提供了这些病变的临床和MR特征的精确数据。其自然病史与出血复发风险较高相关,但许多接受手术的患者预后良好。显微手术是大多数此类病变的首选治疗方法。

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