A review of charts of 30 children with idiopathic intracranial hypertension, seen in a 30 year period between 1960 and 1990, is reported from the University of Iowa Hospital and Clinics. Common presenting symptoms included headache (63%), vomiting (43%), diplopia (36%), blurred vision (26%), and nausea (23%). The majority were heavier than the 50th percentile. All had bilateral papilledema, 30% had retinal hemorrhage, and 53% enlarged blind spots. CTs and MRIs were normal. Associated illnesses were URIs in 16%, otitis in 16%, head trauma in 6%; 46% were otherwise healthy. Six had taken antibiotics, and 4 were on vitamins, including vitamin A. Neurologic signs included VIth nerve palsy in 14, and ataxia in 4. Steroids were used in 9 and acetazolamide in 8. [1]

COMMENT. Abnormalities on neurologic examination are more common in children than in adults. The infrequent occurrence or recognition of the syndrome in infants and young children is noteworthy. A review of the literature before 1960 and prior to the period examined in the Iowa study showed that 84 (37%) of 224 patients were children and of these 75 (90%) were between 5 and 15 years of age [2]. Antecedent otitis media was reported in 65 (29%), and mild head injury or infection other than otitis in 66 (29%). The 93 (41%) cases classified as idiopathic occurred principally in adults in this earlier series whereas approximately one half the pediatric cases in the later Iowa report were unassociated with otitis or other infection, trauma, or predisposing illness. The advent of antibiotics and virtual abolition of mastoiditis and “otitic hydrocephalus” accounts for the change in frequency of predisposing causes and the increase in “idiopathic” cases. For a recent major review of pediatric pseudotumor cerebri, see Lessell S. Survey of Ophthalmology 1992;37:155-166).

Combined therapy with acetazolamide (37 - 100 mg/kg/daily) and furosemide (1 mg/kg/daily) was effective in treating raised intracranial pressure in 8 children with pseudotumor cerebri at the Department of Pediatrics, University of Stellenbosch and Tygerberg Hospital, Republic of South Africa [3]. Repeated lumbar cerebrospinal fluid pressure monitoring was used to evaluate response to therapy, but clinical monitoring correlated well and would be adequate in most children.