Keywords: idiopathic intracranial hypertension, papilledema, intracranial pressure, cranial nerve palsy (PubMed Search)
Idiopathic intracranial hypertension (IIH) is a vision-threatening illness with significant morbidity and needs to be considered as a possible headache diagnosis in the ED. Most often, this occurs in women of childbearing age with a BMI >30, but atypical varieties exist.
Symptoms: Headache (90%), visual disturbance, pulsatile tinnitus, horizotal diplopia.
Signs: Papilledema, 6th cranial nerve (abducens) palsy.
Evaluation: Neuroimaging including CTV or MRV to identify alternate cause including cerebral venous outflow obstruction, lumbar puncture with opening pressure >30 cmH2O (25-30 cmH2O is gray zone), blood work per clinical presentation, CSF analysis.
Treatment: No clear consensus, but typically acetazolamide. Severe or refractory symptoms may require surgical intervention such as optic nerve sheath fenestration, VP shunt, venous sinus stenting.
Hoffmann J, Mollan SP, Paemeleire K, et al. European Headache Federation guideline on idiopathic intracranial hypertension. J Headache Pain. 2018;19(1):93.
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