The diagnosis and treatment of 32 patients diagnosed with idiopathic intracranial hypertension were analysed in a retrospective chart review at the Hospital for Sick Children, Toronto, ON, Canada, and Great Ormond Street Hospital for Children, Institute of Child Health, London, UK. Ages ranged from 2 to 17.5 years. Twenty three were female. The most common presenting symptom was headache (91%), followed by nausea and vomiting (56%), double vision (38%), and visual loss or blurring (25%). Signs at presentation included papilledema (97%), VIth cranial nerve palsy (31% unilateral, 9% bilateral), retinal hemorrhages (13%), constricted visual fields (12%), and decreased visual acuity (29%). Associated disorders in 59% included obesity in 48%, recent or recurrent otitis media (28%), upper respiratory tract infection (16%), sinusitis (13%), Addison’s disease and thyroiditis in 1 patient, and sudden withdrawal of cyproterone acetate for precocious puberty in 1. CT or MRI excluded hydrocephalus or space-occupying lesion, and none had sinus venous thrombosis. All but one had a CSF pressure of >20 cm (a normal opening pressure does not negate the diagnosis, since pressure is known to rise intermittently or infrequently). None developed tonsillar herniation. Treatments intended to alleviate symptoms and prevent visual loss included acetazolamide as first line treatment (44%), corticosteroids (short course) used for deteriorating visual loss or persistent headache (34%), lumboperitoneal shunt (25%), optic nerve fenestration (16%), and repeat lumbar puncture (25%). Combination treatments were used in 40%. Four (13%) received no treatment. Four recovered after the first lumbar puncture and never required further therapy. None of the therapies has been proven effective by randomized controlled trial. The terms “benign” and “pseudotumor” should be discarded in favor of “idiopathic intracranial hypertension.” [1]

COMMENT. Idiopathic intracranial hypertension is not “benign” and is associated with significant morbidity and short- or long-term symptoms, often resistant to therapy. None of the proposed therapies is of proven efficacy, and this extensive case series and review emphasizes the need for prospective studies.