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Imaging of Idiopathic Intracranial Hypertension

  • Anna ZimnyEmail author
  • Marek J. Sąsiadek
Living reference work entry

Abstract

Idiopathic intracranial hypertension (IIH) is a syndrome due to increased intracranial pressure without any evident cause, previously known as pseudotumor cerebri. We discuss the etiology, epidemiology, clinical symptoms, treatment options, and radiological findings of IIH, a rare condition with a predominance in obese women of reproductive age but also seen in men and children. The pathophysiology of IIH is not well understood, and several mechanisms are suggested to play a role such as excessive cerebrospinal fluid (CSF) production, impaired CSF absorption, increased intravascular volume, or increased intracranial venous pressure. Known risk factors are certain medications, hormonal alterations, infections, as well as autoimmune disorders. Main clinical symptoms of IIH are headache and vision loss, less frequently pulsatile tinnitus, diplopia, photopsia, eye pain, or cranial nerve palsies. Ophthalmological evaluation usually reveals papilledema. Lumbar puncture demonstrates elevated opening CSF pressure. The role of clinical neuroradiology is firstly to exclude causes of secondary intracranial hypertension due to space-occupying masses, hydrocephalus, infection, or vascular pathologies including venous sinus thrombosis. Secondly, imaging can show findings that support the diagnosis of IIH such as enlargement of the perioptic CSF spaces, tortuosity of the optic nerves, flattening of the posterior aspect of sclera, intraocular protrusions of optic nerve heads and their contrast enhancement, partially empty sella, enlarged Meckel’s cave, small meningoceles within the skull base, slit-like ventricles, acquired tonsillar ectopia, and transverse sinus narrowing. Recommended radiological protocol includes contrast-enhanced MR examination of the brain and orbits followed by MR venography.

Keywords

Intracranial hypertension Pseudotumor cerebri Thunderclap headache Papilledema Empty sella Cerebrospinal fluid 

Abbreviations

CISS

Constructive interference in steady state

CN

Cranial nerve

CSF

Cerebrospinal fluid

CT

Computed tomography

DRIVE

Driven equilibrium

DWI

Diffusion-weighted imaging

FIESTA

Fast imaging employing steady-state acquisition

IIH

Idiopathic intracranial hypertension

MR

Magnetic resonance

MRI

Magnetic resonance imaging

MRV

Magnetic resonance venography

ONS

Optic nerve sheath

SWI

Susceptibility-weighted imaging

TOF

Time of flight

References

  1. Aiken AH, Hoots JA, Saindane AM, Hudgins PA. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari 1 malformation. Am J Neuroradiol. 2012;33:1901–6.  https://doi.org/10.3174/ajnr.A3068.CrossRefPubMedGoogle Scholar
  2. Bialer OY, Rueda MP, Bruce BB, Newman NJ, Biousse V, Saindane AM. Meningoceles in idiopathic intracranial hypertension. Am J Roentgenol. 2014;202(3):608–13.  https://doi.org/10.2214/AJR.13.10874.CrossRefGoogle Scholar
  3. Bidot S, Saindane AM, Peragallo JH, Bruce BB, Newman NJ, Biousse V. Brain imaging in idiopathic intracranial hypertension. J Neuroophthalmol. 2015;35(4):400–11.  https://doi.org/10.1097/WNO.0000000000000303.CrossRefPubMedGoogle Scholar
  4. Chen J, Wall M. Epidemiology and risk factors for idiopathic intracranial hypertension. Int Ophthalmol Clin. 2014;54(1):1–11.  https://doi.org/10.1097/IIO.0b013e3182aabf11.CrossRefPubMedGoogle Scholar
  5. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. Am J Neuroradiol. 2011;D32:1986–93.  https://doi.org/10.3174/ajnr.A2404.CrossRefGoogle Scholar
  6. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81:1159–65.CrossRefGoogle Scholar
  7. Satti SR. Meta-analysis of CSF diversion procedure and dural venous sinus stenting in the setting of medically refractory IIH. Am J Neuroradiol. 2015;36:1899–904.CrossRefGoogle Scholar
  8. Suzuki H, Takanashi J, Kobayashi K, Nagasawa K, Tashima K, Kohno Y. MR imaging of idiopathic intracranial hypertension. Am J Neuroradiol. 2001;22:196–9.PubMedGoogle Scholar
  9. Walker RW. Idiopathic intracranial hypertension: any light on the mechanism of the pressure? J Neurol Neurosurg Psychiatry. 2001;71:1–7.CrossRefGoogle Scholar

Suggested Readings

  1. Chaudhry S, Bryant T, Peeler CE. Venous sinus stenting in idiopathic intracranial hypertension: a safer surgical approach? Curr Opin Ophthalmol. 2016;27:481–5.  https://doi.org/10.1097/ICU.0000000000000315.CrossRefPubMedGoogle Scholar
  2. Hingwala DR, Kesavadas C, Thomas B, Kapilamoorthy TR, Sarma PS. Imaging signs in idiopathic intracranial hypertension: are these signs seen in secondary intracranial hypertension too? Ann Indian Acad Neurol. 2013;16(2):229–33.  https://doi.org/10.4103/0972-2327.112476.CrossRefPubMedPubMedCentralGoogle Scholar
  3. Holbrook J, Saindane AM. Imaging of intracranial pressure disorders. Neurosurgery. 2017;80:341–54.  https://doi.org/10.1227/NEU.0000000000001362.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of General Radiology, Interventional Radiology and NeuroradiologyWroclaw Medical UniversityWroclawPoland

Section editors and affiliations

  • Charles Anthony Józef Romanowski
    • 1
  1. 1.Department of NeuroradiologySheffield Teaching Hospitals NHS Foundation TrustSheffieldUK

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