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[脑内海绵状血管瘤的家族性发病:兄弟病例报告]

[Familial occurrence of intracerebral cavernous angioma: report of cases in brothers].

作者信息

Yamamura K, Kajikawa H, Wada M, Kajikawa M, Sumioka S, Suyama Y, Shimamoto F

机构信息

Section of Neurosurgery, Suiseikai Kajikawa Hospital.

出版信息

No Shinkei Geka. 1995 Dec;23(12):1105-9.

PMID:8927218
Abstract

Familial occurrence of intracerebral cavernous angioma has been rarely reported. We report two histologically verified cases of cavernous angioma among brothers and review relevant cases in the literature. Case 1 is that of a 3-year-old boy who suffered front acute onset of headache, vomiting, and tonic-clonic type seizure. CT revealed a well-demarcated tumor with partial hemorrhage in the left frontal lobe which was strongly enhanced with contrast Medium. Complete excision was carried out and the patient had a satisfactory clinical course and was able to be followed up for 13 years after the Surgery. Case 2 is that of a 17-year-old boy who was the elder brother of case 1 and presented with gradually increasing episodes of a psychomotor seizure which started at the age of 16. CT and MRI revealed a well-demarcated tumor in the left subcortical temporal lobe and an asymptomatic small calcified lesion in the left subcortical parietal lobe. The temporal tumor was totally excised and histologically diagnosed as cavernous angioma. The seizures gradually decreased and eventually disappeared one year after the surgery. This report reviews 13 previously reported cases, and surgical indication for asymptomatic cases.

摘要

脑内海绵状血管瘤的家族性发病鲜有报道。我们报告了两例经组织学证实的兄弟间海绵状血管瘤病例,并回顾了文献中的相关病例。病例1为一名3岁男孩,突发急性头痛、呕吐和强直阵挛性癫痫发作。CT显示左额叶有一个边界清晰的肿瘤,伴有部分出血,增强造影剂后有明显强化。进行了完整切除,患者临床过程良好,术后能够随访13年。病例2为一名17岁男孩,是病例1的哥哥,自16岁起出现逐渐增多的精神运动性癫痫发作。CT和MRI显示左颞叶皮质下有一个边界清晰的肿瘤,左顶叶皮质下有一个无症状的小钙化病灶。颞叶肿瘤被完全切除,组织学诊断为海绵状血管瘤。术后癫痫发作逐渐减少,最终在术后一年消失。本报告回顾了13例先前报道的病例以及无症状病例的手术指征。

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