Idiopathic Intracranial Hypertension

Diseases

Synonyms:

a.k.a. Benign intracranial hypertesion a.k.a. Pseudotumor cerebri

Diagnosis:

Clinical:

  • Headache
  • +Visual disturbances e.g. peripheral visual field loss or diplopia, “transient visual obscurations”.
  • +Papilloedema, +/-VI palsy

+Imaing MRI, MRV or CTV:

  • No ventricular dilation
  • No venous sinus thrombosis
  • Transependymal oedema may occur

+lumbar puncture opening pressure: 25—40 cm H2O
Visual fields, by perimetry: nasal step defect, arcuate scotoma,

Findings on other investigations:

MRI:

  • Increased CSF space around optic nerve, empty sella turcica, protrusion of optic nerve head (into the globe), Posterior flattening of the globe,

MRV:

  • Venous stenosis

Lars Frisen grades of papilloedema:

Grade 0: normal disc, mild nasal disc blurring
Grade I: C-shaped halo of oedema (elevation) around the optic disc; nasal, superior & inferior aspect
Grade II: circumferential halo of oedema (elevation) around the optic disc
Grade III: above +involvement of vessels leaving the disc
Grade IV: above +involvement of vessels on the disc +/-hemorrhages

Monitor:

Visual fields by Perimetry

Treatment:

  • Acetazlamide orally TID

If refractory:

  • Repeated lumbar punctures
  • Lumboperitoneal shunting

Consider:

  • Transorbital optic nerve sheath fenestration
  • Venous sinus stenting [case series]

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