Neurological Management of Von Hippel-Lindau Disease

Neurologist. 2016 Sep;21(5):73-8. doi: 10.1097/NRL.0000000000000085.

Abstract

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.

Publication types

  • Review

MeSH terms

  • Angiogenesis Inhibitors / therapeutic use*
  • Central Nervous System Neoplasms / therapy*
  • Genetic Therapy
  • Hemangioblastoma / therapy*
  • Humans
  • Neurosurgical Procedures*
  • Radiosurgery*
  • von Hippel-Lindau Disease / therapy*

Substances

  • Angiogenesis Inhibitors