Pseudotumour cerebri-neurosurgical considerations.

Pseudotumour was diagnosed in six patients aged 3-38 years during an 8 years period. The diagnosis was based on headache, papilloedema, normal CT scan and cerebrospinal fluid (CSF) composition. Additional clinical symptoms were nausea, VIth nerve palsy, ataxia, blurred vision and frank visual reduction over time. Sagittal sinus thrombosis was ruled out by angiography or magnetic resonance imaging. In five of the six patients lumbar steady state infusion tests were performed to evaluate intracranial hydrodynamics and CSF resorbtion. All patients demonstrated a markedly increased opening pressure (range 13 to 48 mm Hg). CSF outflow resistance ranged from upper normal to pathologically increased levels (8-19 mm Hg/ml/min). Combined epidural intracranial pressure/middle cerebral artery blood velocity monitoring in 3 patients revealed a great number of B waves and a labile cerebral vasomotor state. Pharmacological treatment was tried with digitoxin, acetazolamide, furosemide and/or corticosteroids. Two patients did well on long-term treatment with digitoxin and furosemide, respectively. In the other four patients the clinical development was unsatisfactory on medical treatment alone. They were subsequently operated with implantation of a lumboperitoneal, cisternoatrial or cisternoperitoneal shunt. Shunting rapidly reversed clinical signs and symptoms, except for a partial persistent visual loss in an 18 years old boy who had experienced symptoms for 3 years resistant to pharmacological treatment.