Idiopathic Intracranial Hypertension


KEY FACTS

Terminology

  • Idiopathic intracranial hypertension (IIH)

  • Pseudotumor cerebri

  • “Benign intracranial hypertension”

  • ↑ intracranial pressure (ICP) without identifiable cause

Imaging

  • Empty or partially empty sella

  • Posterior globe flattening

  • Intraocular protrusion of optic nerve head

  • Optic nerve sheath enlargement: Widened ring of cerebrospinal fluid around optic nerve

  • Optic nerve tortuosity

  • Slit-like ventricles, rare: Poor neuroimaging sign of IIH

  • MRV: Often shows transverse sinus stenosis and “flow gaps”

    • Whether this is cause or consequence of raised ICP is controversial

  • Best imaging tool: MR brain + T2 coronal fat-sat orbit + MRV

Top Differential Diagnoses

  • Secondary pseudotumor syndromes

    • Cerebral venous thrombosis, dural sinus stenosis

  • Idiopathic or postinflammatory (i.e., multiple sclerosis) optic nerve atrophy

  • Idiopathic empty sella (normal variant)

Clinical Issues

  • Obese woman age 20-44 years most common

    • Headache in 90-95%

    • Papilledema (bilateral optic nerve head swelling) virtually universal

  • Progressive visual loss ± CN6 paresis, diplopia

  • Chief hazard: Vision loss from chronic papilledema

  • Treatment: Medical or surgical (lLP and VP shunt, optic nerve sheath fenestration)

Funduscopy in a patient with IIH, severe papilledema shows swelling/blurred margins of the optic disc
, striking venous engorgement
, and focal hemorrhages
adjacent to the disc.

Axial T2WI MR shows dilated CSF spaces around the optic nerves
and protrusion of the optic nerve papilla into the posterior globes
. Opening CSF pressure in this 32-year-old woman was 45 cm of H₂O. Prominent CSF space in the suprasellar cistern represents an empty sella
. Note the tortuosity of the left optic nerve
.

Axial T1WI C+ MR in the same patient demonstrates enhancement, as well as protrusion of prelaminar optic nerves bilaterally
. Mild diffuse optic nerve sheath enhancement
is also present.

Coronal T1WI C+ FS MR in the same patient shows diffuse enhancement of the optic nerve sheaths
associated with prominent subarachnoid spaces along the optic nerves. Treatment for IIH includes weight loss and medications (i.e., diuretics), as well as lumbar punctures, shunt, and optic nerve fenestration.

TERMINOLOGY

Abbreviations

  • Idiopathic intracranial hypertension (IIH)

  • Pseudotumor cerebri (PTC)

Synonyms

  • Pseudotumor cerebri

  • Benign intracranial hypertension

Definitions

  • ↑ intracranial pressure (ICP) without identifiable cause

  • Association of any medication or condition with IIH better termed “secondary pseudotumor syndrome”

IMAGING

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