Updated: 10/14/2020

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

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Snapshot
  • A 28-year-old woman is referred to a headache neurologist for throbbing left-sided headaches that are refractory to numerous analgesics. Her headache is associated with episodes of double vision and "vision blurriness." She also endorses worsening headache with a cough and has experienced nausea. Her BMI is 32 kg/m2. Neurological examination is notable for a left-sided sixth nerve palsy, decreased visual fields, and papilledema on fundoscopy. She undergoes an MRI brain with MR venography, which is unremarkable. A lumbar puncture demonstrates a significant opening pressure with normal protein, glucose, and cells. She is started on acetazolamide and is referred to an Ophthalmologist for possible optic nerve sheath fenestration.
Introduction
  • Definition
    • signs and symptoms consistent with increased intracranial pressure without an identifiable cause
      • a diagnosis of exclusion
  • Epidemiology
    • risk factors
      • female gender in childbearing age
      • obesity
      • oral contraceptive pills 
      • vitamin A
  • Pathogenesis
    • believed to be due to reduced cerebrospinal fluid absorption
Presentation
  • Symptoms
    • headache
      • typically lateralized and pulsatile in nature
    • nausea and vomiting
    • pulsatile tinnitus
    • diplopia
  • Physical exam
    • papilledema (hallmark) 
    • sixth nerve palsy
    • visual field loss
Imaging
  • MR brain with MR venography
    • indication
      • preferred initial imaging choice to exclude secondary causes of increased intracranial pressure
    • findings
      • typically normal; however, there are findings suggestive of idiopathic intracranial hypertension
        • posterior sclera flattening 
        • vertical tortuosity of the orbital optic nerve
        • perioptic subarachnoid space distension
Studies
  • Lumbar puncture  
    • most accurate diagnostic test for IIH 
    • indication
      • performed after secondary causes of increased intracranial pressure has been excluded on neuroimaging
    • findings
      • elevated opening pressure
      • otherwise normal CSF profile (protein, cells, and glucose)
    • method
      • perform in the lateral decubitus position with the legs extended
  • Ophthalmic examination
    • indication
      • to determine the extent of optic nerve damage from the increased intracranial pressure
Differential
  • Migraine
    • differentiating factor
      • no papilledema on fundoscopy
  • Secondary causes of increased intracranial pressure
    • differentiating factor
      • the presence of a malignancy, cerebral sinus thrombosis, or other space occupying lesions on neuroimaging
Treatment
  • Medical
    • carbonic anhydrase inhibitors
      • indication
        • first-line treatment for idiopathic intracranial hypertension
      • medication
        • acetazolamide 
          • a loop diuretic can also be added as adjunctive therapy
      • mechanism
        • decreases the rate of CSF production
  • Operative
    • optic nerve sheath fenestration
      • indication
        • in patients who fail medical management
Complications
  • Blindness
 

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(M2.GN.12.16) A 24-year-old woman presents to the emergency department with blurry vision. She describes having a headache that is painful. She has bilateral blurry vision that has been worsening over the past month. She denies any focal deficits or fevers but does endorse occasional nausea. The patient recently started walking to improve her health and is taking vitamin K, oral contraceptive pills, whey protein, and fish oil. She is not currently sexually active, does not smoke, and eats a diet mostly of pastries and baked goods. Her temperature is 98.0°F (36.7°C), blood pressure is 154/89 mmHg, pulse is 87/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam is notable for an obese woman who is closing her eyes. Neurological exam reveals 20/200 vision which is a decline from the patient's baseline and the finding in Figure A. Her gait is stable and no cranial nerve abnormalities are noted. The patient endorses some numbness in her ring finger and little finger bilaterally when she lays down and notes it currently. Which of the following is appropriate management of this patient's most likely diagnosis? Tested Concept

QID: 103379
FIGURES:
1

Discontinue oral contraceptive pills

44%

(7/16)

2

Discontinue vitamin K

6%

(1/16)

3

Initiate diltiazem

6%

(1/16)

4

Initiate glatiramer

6%

(1/16)

5

Initiate sumatriptan

38%

(6/16)

M 7 E

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