Critical Cases - Idiopathic Intracranial Hypertension!

HPI

  • 50 year old woman with a history of RA comes to the ED for headache
  • Intermittent headaches for 3 months, worsening over 10 days
  • She now has occasional spots in her vision
  • HA worse with movement, especially bending forward

 

Physical Examination

Vitals: 139/84 HR 78 T 97.8F. RR 16 SpO2 100%

  • CN are intact including visual fields
  • Strength and sensation are intact
  • Normal finger to nose and rapid/alternating movements
  • Fundscopic exam inconclusive
  • Patient ambulates with a slow but steady gait 

Differential Diagnosis

  • Primary headache (tension, migraine, cluster): These account for the vast majority of cases encountered in the ED
  • Space occupying lesion: tumor
  • Subarachnoid hemorrahge: Possible given severity and atypical nature of headache, though no classic "thunderclap" onset
  • Idiopathic intercranial hypertension
  • Cerebral venous sinus thrombosis

Management 

  • CT head without contrast prior to LP
  • LP in the lateral decubitus to measure opening pressure-this patient had an elevated opening pressure of 26 cm H20
  • Neurology consult: reccomoend MRI brain with and without contrast

 

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Radiology read this as: no acute intracranial abnormalities with partially empty sella and slight prominence of the subarachnoid space that can be seen in the setting of IIH.

Diagnostic Criteria for Idiopathic Intracranial Hypertension

  • Papilledema
  • Normal neuro examination except 6th nerve palsy
  • Normal neuroimaging with partial or completely empty sella
  • Normal CSF
  • Elevated opening pressure >25cm CSF

Take Home Points

  • IIH occurs more frequently in women of childbearing age with elevated BMI 
  • Patients present complaining of headache with visual symptoms and sometimes "pulsatile tinnitus"
  • Long term treatment with weight loss reduction has been shown to be effective in improving vision and headache symptoms
  • Initial medical therapy = acetazolamide to decrease CSF production
  • All patients require ophthalmology follow-up to screen for papilledema and for visual field testing
  • Rapidly progressive vision loss may require VP shunting
  • Undiagnosed IIH may lead to permanent vision loss

 

Sources

Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89(10):1088-1100. doi:10.1136/jnnp-2017-317440