Childhood Pseudotumor Cerebri: Clinical and Intracranial Pressure Response to Acetazolamide and Furosemide Treatment in a Case Series

The purpose of this study was to investigate the efficacy of combined therapy with acetazolamide and furosemide in normalizing intracranial pressure in children with pseudotumor cerebri. The role of repeated lumbar cerebrospinal fluid pressure monitoring in evaluating the response to therapy is also demonstrated. Continuous 1-hour lumbar cerebrospinal fluid pressure monitoring was done in eight children with pseudotumor cerebri on admission and at weekly intervals until the baseline pressure had normalized. (One child had two episodes of pseudotumor cerebri). All patients were treated with oral acetazolamide and furosemide until papilledema had cleared. Raised intracranial pressure was present on admission in all nine episodes of pseudotumor cerebri. Six children had an increased baseline cerebrospinal fluid pressure, whereas raised intracranial pressure was diagnosed in three children on account of an abnormal pulse wave and/or pressure waves. The mean baseline pressure was significantly lower after the 1st week of treatment than on admission (P = .007) and normalized in all patients within 6 weeks of start of therapy. All children had a rapid clinical response. Combined therapy with acetazolamide and furosemide is an effective first-line method of treating raised intracranial pressure in children with pseudotumor cerebri. The good correlation found between the clinical response and normalization of baseline cerebrospinal fluid pressure suggests that clinical monitoring of treatment is adequate in most children with this condition.