Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

Abstract

Neurology| October 01 2001 Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) AAP Grand Rounds (2001) 6 (4): 45. https://doi.org/10.1542/gr.6-4-45 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Idiopathic Intracranial Hypertension (Pseudotumor Cerebri). AAP Grand Rounds October 2001; 6 (4): 45. https://doi.org/10.1542/gr.6-4-45 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: pseudotumor cerebri Source: Salman MS, Kirkham FJ, MacGregor DL. Idiopathic “benign” intracranial hypertension: case series and review. J Child Neurol. 2001;16:465–470. Chronic, recurring headache is a common symptom in children, with an increasing prevalence in recent years variously estimated at 5 to 11%,1 which is most often due to migraine or tension. Idiopathic intracranial hypertension (IIH) is an uncommon cause which requires prompt diagnosis and appropriate management to prevent vision loss. The authors retrospectively reviewed the diagnosis and treatment of 32 patients with IIH from Toronto, Canada and London, UK aged 2 to 17.5 years. Twenty-three (72%) were female. The most common presenting symptoms were headache (91%), 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), decreased visual acuity (29%), retinal hemorrhages (13%), and constricted visual fields (12%). Associated disorders in 19/32 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. In all cases, CT or MRI excluded hydrocephalus, space-occupying lesion, and sinus venous thrombosis. All but one had an opening CSF pressure of >20 cm. None developed tonsillar herniation after lumbar puncture (LP). 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 LP (25%). Combination treatments were used in 40%. Four (13%) received no treatment. Four recovered after the first LP and never required further therapy. None of these therapies has been proven to be effective in randomized controlled trials. Idiopathic benign intracranial hypertension or pseudotumor cerebri is defined as an increase in intracranial pressure in the absence of a space-occupying lesion. In children, 90% of cases occur in the 5- to 15-year age range, and only 10% in infants and those under 5 years.2 In adults, the mean age at diagnosis is 28 years (range, 15–40).3 Neuroimaging to exclude a mass lesion, hydrocephalus, or venous thrombosis usually shows a normal or small ventricular system or, in young children, separated skull sutures. After excluding a surgical lesion, the diagnosis is confirmed by demonstrating elevated CSF pressure on LP. In some cases, CSF pressure may rise intermittently so a normal, single reading does not exclude the diagnosis. CSF analysis is normal or shows a mild pleocytosis. Because of the risk of tonsillar herniation during LP, patients with definite papilledema and/or retinal hemorrhages are probably best referred to a neurosurgeon or neurologist for LP. Known causes of IIH include recent or recurrent otitis media, venous sinus thrombosis, mastoiditis, hypervitaminosis A, recent administration of tetracycline or other antibiotic, and sudden withdrawal or prolonged use of steroids. To alleviate headache and prevent visual impairment, a normal CSF pressure must be maintained and confirmed by repeated... You do not currently have access to this content.