Book contents
- Uncommon Causes of Stroke
- Uncommon Causes of Stroke
- Copyright page
- Contents
- List of Contributors
- Preface
- PART I: INFECTIOUS AND INFLAMMATORY CONDITIONS
- PART II: HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
- 16 PULMONARY ARTERIOVENOUS MALFORMATIONS
- 17 HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER–WEBER–RENDU DISEASE)
- 18 CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL)
- 19 CEREBROVASCULAR COMPLICATIONS OF FABRY’S DISEASE
- 20 MARFAN’S SYNDROME
- 21 PSEUDOXANTHOMA ELASTICUM
- 22 EHLERS-DANLOS SYNDROME
- 23 PROGERIA
- 24 MELAS AND OTHER MITOCHONDRIAL DISORDERS
- 25 STURGE-WEBER SYNDROME
- 26 VON HIPPEL-LINDAU DISEASE
- 27 ANEURYSMS
- 28 ARTERIOVENOUS MALFORMATIONS OF THE BRAIN
- 29 CEREBRAL CAVERNOUS MALFORMATIONS AND DEVELOPMENTAL VENOUS ANOMALIES
- 30 CEREBROVASCULAR MANIFESTATIONS OF NEUROFIBROMATOSIS
- 31 MENKES DISEASE (KINKY HAIR DISEASE)
- 32 WYBURN-MASON SYNDROME
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- PART IV: DISORDERS INVOLVING ABNORMAL COAGULATION
- PART V: SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
- PART VI: NONINFLAMMATORY DISORDERS OF THE ARTERIAL WALL
- PART VII: VENOUS OCCLUSIVE CONDITIONS
- PART VIII: VASOSPASTIC CONDITIONS AND OTHER MISCELLANEOUS VASCULOPATHIES
- PART IX: OTHER MISCELLANEOUS CONDITIONS
- Index
26 - VON HIPPEL-LINDAU DISEASE
from PART II: - HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
Published online by Cambridge University Press: 06 January 2010
Book contents
- Uncommon Causes of Stroke
- Uncommon Causes of Stroke
- Copyright page
- Contents
- List of Contributors
- Preface
- PART I: INFECTIOUS AND INFLAMMATORY CONDITIONS
- PART II: HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
- 16 PULMONARY ARTERIOVENOUS MALFORMATIONS
- 17 HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER–WEBER–RENDU DISEASE)
- 18 CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL)
- 19 CEREBROVASCULAR COMPLICATIONS OF FABRY’S DISEASE
- 20 MARFAN’S SYNDROME
- 21 PSEUDOXANTHOMA ELASTICUM
- 22 EHLERS-DANLOS SYNDROME
- 23 PROGERIA
- 24 MELAS AND OTHER MITOCHONDRIAL DISORDERS
- 25 STURGE-WEBER SYNDROME
- 26 VON HIPPEL-LINDAU DISEASE
- 27 ANEURYSMS
- 28 ARTERIOVENOUS MALFORMATIONS OF THE BRAIN
- 29 CEREBRAL CAVERNOUS MALFORMATIONS AND DEVELOPMENTAL VENOUS ANOMALIES
- 30 CEREBROVASCULAR MANIFESTATIONS OF NEUROFIBROMATOSIS
- 31 MENKES DISEASE (KINKY HAIR DISEASE)
- 32 WYBURN-MASON SYNDROME
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- PART IV: DISORDERS INVOLVING ABNORMAL COAGULATION
- PART V: SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
- PART VI: NONINFLAMMATORY DISORDERS OF THE ARTERIAL WALL
- PART VII: VENOUS OCCLUSIVE CONDITIONS
- PART VIII: VASOSPASTIC CONDITIONS AND OTHER MISCELLANEOUS VASCULOPATHIES
- PART IX: OTHER MISCELLANEOUS CONDITIONS
- Index
Summary
Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder with a 95% penetrance at age 60 and incomplete expression that is characterized by the development of various benign and malignant tumors and cysts. Hemangioblastoma (HB), the most characteristic central nervous system (CNS) lesions, are highly vascular tumors comprising approximately 3% of all tumors of the CNS. HBs are diagnosed by magnetic resonance imaging (MRI) of the brain and the spinal cord. Presentation of HBs with a stroke syndrome is rare despite their highly vascular nature, and the risk of hemorrhage associated with them is not yet known. Screening for VHL disease following the diagnosis of a cerebral HB is indispensable given the high risk of associated lesions with hereditary forms. The best treatment for this tumor is surgical resection. If the tumor is difficult to remove from its primary site, it can sometimes be treated with radiosurgery.
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- Uncommon Causes of Stroke , pp. 163 - 170Publisher: Cambridge University PressPrint publication year: 2008