Hodgson Trent S, Nielsen Sarah M, Lesniak Maciej S, Lukas Rimas V
*Department of Neurology, Cornell University, New York, NY †Department of Medicine-Section of Hematology & Oncology, University of Chicago ‡Department of Neurosurgery §Department of Neurology, University of Chicago, Chicago.
Neurologist. 2016 Sep;21(5):73-8. doi: 10.1097/NRL.0000000000000085.
Von Hippel-Lindau disease is a genetic condition due to mutation of the Von Hippel-Lindau gene, which leads to an increased risk in the development of hemangioblastomas of the brain and spinal cord. The pathophysiology of disease and its clinical manifestations, as they pertain to the general neurologist, are discussed. Therapeutic management of central nervous system hemangioblastomas ranging from neurosurgical resection, radiation therapy, and systemic therapies is reviewed.
冯·希佩尔-林道病是一种由冯·希佩尔-林道基因发生突变引起的遗传性疾病，它会增加脑和脊髓血管母细胞瘤发生的风险。本文讨论了该疾病的病理生理学及其与普通神经科医生相关的临床表现。对中枢神经系统血管母细胞瘤从神经外科手术切除、放射治疗到全身治疗的治疗管理进行了综述。
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