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Idiopathic Intracranial
Hypertension
By J Beatty
Introduction
 Common cause of disc swelling.
 Most common cause of papillodema.
 NB because papilloedema can = mass lesion.
 NB because IIH can lead to significant visual
loss.
Modified Dandy Criteria
 Signs and symptoms of raised intracranial
pressure
 Normal neuro exam, except 6th
nerve palsy
 Elevated CSF pressure with normal
constituents
 Normal neuroimaging
Demographics and General Info
 Typical = early adult, women, overweight or
recent weight gain.
 Cause and mechanism unclear (? decreased
CSF absorption from dysfunctional arachnoid
villi). Numerous other postulations.
Clinical Presentation
 Headache.
– 90% of patients (most common).
– 60% pulsatile intracranial noise.
 Visual symptoms.
– Transient visual obscurations (TVOs), 72%.
– Blurred vision.
– Enlarged blind spot or other visual field loss.
– Diplopia.
Associated Conditions
 Before examination, think!?
 Very long list of conditions to consider in the
differential diagnosis.
 Need to exclude cranial venous conditions,
mass lesions and specific known causes of
elevated intracranial pressure.
 Venous thrombosis or obstruction.
– Pseudotumor like appearance, difficult to exclude.
– Cerebral dural venous sinus thrombosis > increased
venous pressure >decreased CSF absorbtion.
– Seriously effected patients develop cortical vein
thrombosis and cerebral infarction.
– Also thrombosis of transverse and sigmoid sinus.
– Cause of clot formation:
 Dural Arteriovenous malformations
 Apnea
Neuro-ophthalmic Examination
– Insidious visual or field loss.
– Sever loss (chronic papilloedema, RD, Hx, macular
exudate).
– Peripheral field defects, enlarged blind spot.
– Colour and pupil normal.
– 50% abnormal contrast sensitivity.
– 6th
nerve palsy.
 Papilloedema
– Usually bilatereal
– Pantons lines
– Vascular changes (2nd
to compression)
– Loss of spontaneous venous pulsation
– Acute vs chronic
 Pseudopapilloedema
– Congenital, harmatoma, mylinated nerve fibers,
drusen
– Serial examinations
– Optic disc drusen
 Defect in axonal metabolism
 1-2% of population, often bilat, inherited
 Examination, u/s, CT
– Other causes
Diagnostic Evaluation
 MRI better than CT
– Empty sella, dilation optic nerve sheath, flat post
globe, elevation of optic disc, slit like ventricles
 LP
– >25mm H20
Management
 No visual loss
– Weight reduction
– Acetazolamide (500-2000 mg/day)
 Mild to moderate visual loss
– Acetazolamide (up to 2-3 gms/day)
– Or furosemide (40-80mg daily)
– Weight reduction
 Sever or progressive visual loss.
– Optic nerve sheath fenestration.
– High-dose IV steroids and acetazolamide.
– Lumboperitoneal shunt for failed ONSF or
intractable headache.
Outcome
 Mild and moderate do well
 Sever can have decreased vision and field
defects. Devestating 5%

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Idiopathic Intracranial Hypertension

  • 2. Introduction  Common cause of disc swelling.  Most common cause of papillodema.  NB because papilloedema can = mass lesion.  NB because IIH can lead to significant visual loss.
  • 3. Modified Dandy Criteria  Signs and symptoms of raised intracranial pressure  Normal neuro exam, except 6th nerve palsy  Elevated CSF pressure with normal constituents  Normal neuroimaging
  • 4. Demographics and General Info  Typical = early adult, women, overweight or recent weight gain.  Cause and mechanism unclear (? decreased CSF absorption from dysfunctional arachnoid villi). Numerous other postulations.
  • 5. Clinical Presentation  Headache. – 90% of patients (most common). – 60% pulsatile intracranial noise.  Visual symptoms. – Transient visual obscurations (TVOs), 72%. – Blurred vision. – Enlarged blind spot or other visual field loss. – Diplopia.
  • 6. Associated Conditions  Before examination, think!?  Very long list of conditions to consider in the differential diagnosis.  Need to exclude cranial venous conditions, mass lesions and specific known causes of elevated intracranial pressure.
  • 7.  Venous thrombosis or obstruction. – Pseudotumor like appearance, difficult to exclude. – Cerebral dural venous sinus thrombosis > increased venous pressure >decreased CSF absorbtion. – Seriously effected patients develop cortical vein thrombosis and cerebral infarction. – Also thrombosis of transverse and sigmoid sinus. – Cause of clot formation:
  • 8.  Dural Arteriovenous malformations  Apnea
  • 9. Neuro-ophthalmic Examination – Insidious visual or field loss. – Sever loss (chronic papilloedema, RD, Hx, macular exudate). – Peripheral field defects, enlarged blind spot. – Colour and pupil normal. – 50% abnormal contrast sensitivity. – 6th nerve palsy.
  • 10.  Papilloedema – Usually bilatereal – Pantons lines – Vascular changes (2nd to compression) – Loss of spontaneous venous pulsation – Acute vs chronic
  • 11.  Pseudopapilloedema – Congenital, harmatoma, mylinated nerve fibers, drusen – Serial examinations – Optic disc drusen  Defect in axonal metabolism  1-2% of population, often bilat, inherited  Examination, u/s, CT – Other causes
  • 12. Diagnostic Evaluation  MRI better than CT – Empty sella, dilation optic nerve sheath, flat post globe, elevation of optic disc, slit like ventricles  LP – >25mm H20
  • 13. Management  No visual loss – Weight reduction – Acetazolamide (500-2000 mg/day)  Mild to moderate visual loss – Acetazolamide (up to 2-3 gms/day) – Or furosemide (40-80mg daily) – Weight reduction
  • 14.  Sever or progressive visual loss. – Optic nerve sheath fenestration. – High-dose IV steroids and acetazolamide. – Lumboperitoneal shunt for failed ONSF or intractable headache.
  • 15. Outcome  Mild and moderate do well  Sever can have decreased vision and field defects. Devestating 5%