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
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- 33 EALES RETINOPATHY
- 34 ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY
- 35 MICROANGIOPATHY OF THE RETINA, INNER EAR, AND BRAIN: SUSAC’S SYNDROME
- 36 HEREDITARY ENDOTHELIOPATHY WITH RETINOPATHY, NEPHROPATHY, AND STROKE (HERNS)
- 37 COGAN’S SYNDROME
- 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
36 - HEREDITARY ENDOTHELIOPATHY WITH RETINOPATHY, NEPHROPATHY, AND STROKE (HERNS)
from PART III: - VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
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
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- 33 EALES RETINOPATHY
- 34 ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY
- 35 MICROANGIOPATHY OF THE RETINA, INNER EAR, AND BRAIN: SUSAC’S SYNDROME
- 36 HEREDITARY ENDOTHELIOPATHY WITH RETINOPATHY, NEPHROPATHY, AND STROKE (HERNS)
- 37 COGAN’S SYNDROME
- 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
Hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS) typically begins with progressive visual loss in the third or fourth decade of life followed by focal neurological deficits within 4-10 years. The clinical features of HERNS include strokes, retinopathy, nephropathy, migraine, mood disorders, and dementia. The underlying mechanism of HERNS appears to be a generalized vasculopathy with disruption of the integrity of capillaries and arterioles. Fluorescein angiograms clearly show retinal vasculopathic changes. That the intracerebral lesions show contrast enhancement on magnetic resonance imaging (MRI) indicates break down in the blood-brain barrier. The surrounding edema in a vasogenic pattern also suggests increased capillary permeability. At the present time there is no known treatment that is effective in patients with HERNS. However, corticosteroids have been useful to decrease cerebral edema and may even be life-saving in patients with large edematous lesions.
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- Uncommon Causes of Stroke , pp. 255 - 258Publisher: Cambridge University PressPrint publication year: 2008