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Idiopathic Intracranial Hypertension: A Venous Disease?

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Cerebral Venous System in Acute and Chronic Brain Injuries

Part of the book series: Springer Series in Translational Stroke Research ((SSTSR))

Abstract

Idiopathic intracranial hypertension (IIH) is a condition characterized by a constellation of symptoms including headache, visual disturbance, tinnitus, and papilledema that affect primarily obese/young female. The pathophysiologic hallmark of the disease is a chronic elevation of intracranial pressure (opening pressure on lumbar puncture >250 cm H2O). Despite the significant morbidity associated with this condition (vision loss and debilitating headaches), the exact mechanisms leading high intracranial pressure is not known. It has been purported that cerebral venous stenosis (CVS) may play a role in the pathogenesis of idiopathic intracranial hypertension. In this chapter, we examine the evidence linking cerebral venous stenosis to IIH. From an epidemiological perspective, CVS is up to 9 times more prevalent among patients with IIH compared to their counterparts, and the cerebral venous pressure gradient across the stenotic segment by venographic manometry is higher among patients with IIH. Pathophysiological evidence for an association between CVS and IIH suggest that at least in some cases, variations in the caliber of the cerebral vein is associated with a change in cerebrospinal fluid pressure; an observation that is supported by mathematical models. In the absence of randomized clinical trials, the best evidence supporting CVS stenting are primarily provided by meta-analyses of individual studies that report a clinical benefit of the procedure albeit several shortfalls. In light of the suggested association between cerebral transverse sinus stenosis/hypoplasia and certain IIH cases, future research would need to focus on defining the clinical and radiological profile of individuals who will best benefit from CVS stenting. It is also expected that ongoing clinical trials shed more light on the association between CVS and IIH and refine the therapeutic potentials of stenting.

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Acknowledgments

We would like to thank Drs. Samir Belagaje and Saher Khalid for their generous comments on this manuscript. Dr. Feng would like to acknowledge the grant supports from National Institute of Health (P20GM109040 and HD086844), American Heart Association (14SDG1829003) and South Carolina Clinical & Translational Research Institute (UL1 TR001450).

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Authors and Affiliations

  1. Department of Neurology, Medical University of South Carolina, Charleston, SC, USA

    Alain Lekoubou & Wuwei Feng

Authors
  1. Alain Lekoubou
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  2. Wuwei Feng
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Corresponding author

Correspondence to Wuwei Feng .

Editor information

Editors and Affiliations

  1. Department of Neurology, The Second Affiliated Hospital of School of Medicine, Zhejiang University, Hangzhou, China

    Min Lou

  2. Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China

    Jianmin Zhang

  3. Beijing Tiantan Hospital, Capital Medical University, Beijing, China

    Yilong Wang

  4. Department of Neurosurgery Tangdu Hospital, PLA Air Force Medical University, Xian, USA

    Yan Qu

  5. Department of Neurology, Medical University of South Carolina, Charleston, SC, USA

    Wuwei Feng

  6. Xuanwu Hospital Neurosurgery, Capital Medical University, Beijing, China

    Xunming Ji

  7. Department of Anesthesiology and Physiology, Loma Linda University, Loma Linda, CA, USA

    John H. Zhang

Appendix

Appendix

1.1 Search Terms

#1 “pseudotumor cerebri” OR “idiopathic intracranial hypertension” OR “benign intracranial hypertension”

#2 “venous stenosis” OR “sinus stenosis” OR “stenosis of cerebral vein”

#3 1 AND 2

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Lekoubou, A., Feng, W. (2019). Idiopathic Intracranial Hypertension: A Venous Disease?. In: Lou, M., et al. Cerebral Venous System in Acute and Chronic Brain Injuries. Springer Series in Translational Stroke Research. Springer, Cham. https://doi.org/10.1007/978-3-319-96053-1_9

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  • DOI: https://doi.org/10.1007/978-3-319-96053-1_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-96052-4

  • Online ISBN: 978-3-319-96053-1

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