MULTIPLE PRIMARY INTRACRANIAL TUMORS IN VON RECKLINGHAUSENS NEUROFIBROMATOSIS

MULTIPLE PRIMARY INTRACRANIAL TUMORS IN VON RECKLINGHAUSENS NEUROFIBROMATOSIS
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DOI:
10.1001/archneur.1966.00470110011002
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发表时间:
1966-01-01
影响因子:
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通讯作者:
BERTHRONG, M
BERTHRONG, M
中科院分区:
其他
文献类型:
--
作者:
RODRIGUEZ, HA;BERTHRONG, M

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一件20年前的案子。本文报告一位患有中央型雷克林豪森S病的老人,并伴有听神经瘤、多发性脑膜瘤、多发性室管膜瘤及脊髓空洞症。复习文献,分析48例雷克林豪森S病合并听神经瘤和多发性脑膜瘤的临床资料。患者的平均年龄为27岁。女性和男性受到的影响是一样的。听神经瘤为双侧占82%,单侧占18%。多发性脑膜瘤54%位于颅内,42%位于颅内和椎管内,仅4%位于椎管内。合并神经胶质瘤占45%,多发于颈髓和脊髓背侧,脊髓空洞症和S神经纤维瘤病家族史占20%。
The case of a 20 yr. old man with Recklinghausen''s disease of the central type, associated with an acoustic neurinoma, multiple meningiomas, multiple ependymomas, and syringomyelia, is presented. The medical literature was reviewed, and 48 cases of Recklinghausen''s disease with acoustic neurinomas and multiple meningiomas were analyzed. The average age of the patients was 27 yr. Females and males were equally affected. Acoustic neurinomas were bilateral in 82% and unilateral in 18% of the cases. Multiple meningiomas were intracranial in 54%, intracranial and intraspinal in 42%, and only intraspinal in 4% of the cases. Of the patients 45% had associated gliomas which were frequently multiple andlocalizedin the cervical and dorsal spinal cord, Syringomyelia and family history of Recklinghausen''s neurofibromatosis were found in 20% of the cases.