Pseudotumor cerebri under prednisolone treatment for infantile spasms

Pseudotumor cerebri (PC) is defined as raised intracranial pressure (ICP) causing headaches, visual impairment, and papilledema in the absence of a tumour or hydrocephalus occlusus. It is most prevalent in postpuberal girls; obesity is a risk factor. PC mostly is idiopathic or can be caused by cerebrovascular or endocrine dysfunctions, drugs, infections, refeeding after dehydration. Treatment options are directed at the underlying conditions, and if unknown, aim to decrease intracranial pressure via repeated lumbar punctures or diuretic medications like acetazolamide, furosemide and steroids.

Our patient is a 10 months old girl with a previous uneventful history. At four month infantile spasms and hypsarrhythmia in the EEG were seen. Pyridoxine was given without any effect on seizures and EEG pattern. Then prednisolone treatment 4×10mg/day (Lux, Lancet 2004) was started, seizures improved and EEG as well. At the same time the girl presented with high pitched screeming attacks and opistotonus. This symptoms became worse after prednisolone was increased to 3×20mg/day. No papilledema was present, however lumbar puncture revealed abnormal intracranial hypertension at 25cmH2O.

We decreased and finally stopped prednisolone and the girl improved dramatically. Seizure control was reached with sultiame (10mg/kg). Since the EEG remained pathological, we treated her with vigabatrin (100mg/kg) and the child became seizure free and the EEG was normalised.

We suspect that the pseudotumor cerebri symptoms were caused by the prednisolone treatment for infantile spasms. This case is unusual due to the young age, and the induction by steroids. Though prednisolone treatment is well established for infantile spasms, pseudotumor cerebri has never been on report.

Intracranial hypertension is rare in infancy, symptoms are atypical, and papilledema often is absent. To diagnose pseudotumor cerebri, lumbar puncture for intracranial pressure is essential.

pseudotumor cerebri - infantile spasms - prednisolone