Neuropediatrics 2012; 43 - PS14_02
DOI: 10.1055/s-0032-1307100

Pseudotumor cerebri in an 8 month old infant

F Brueckner 1, B Kohl 1, C Muehlhausen 2, B Püst 1
  • 1Kinderkrankenhaus Wilhelmstift, Hamburg, Germany
  • 2Uniklinikum Eppendorf, Zentrum für Kinder- und Jugendmedizin, Hamburg, Germany

Aims: Pseudotumor cerebri (benign intracerebral hypertension) is a rare disease in childhood, especially in young infants. It is caused by disturbed circulation of cerebrospinal fluid (csf) without intracerebral tumor or obstruction of the csf pathways.

Methods: We present a 8-month female infant brought to our hospital because of vomiting and a pre-bulged fontanelle. There was no fever and no diarrhea. Sonography of the brain and status of csf were normal, so the infant was discharged after three days with the diagnosis of a viral infection.

One week later, vomiting, intermittent apathy and a pre-bulged fontanelle reoccured again. A papilledema in the left eye was found in funduscopy and orbitasonography. Csf diagnostic showed a pleocytosis of 183/3 Leucozytes and an csf opening pressure of 32.5 mmH2O. Five days of treatment with cefotaxim followed until csf-culture was sterile.

There were normal findings in brain-MRI (incl. angio-MRI), spinal MRI, blood results (red and white blood cells, C-reactive protein, liver enzyms, retention values, electrolytes, TSH, fT4, ACTH, Vit. B12), PCR of Enterovirus, Parechovirus, Adenovirus, Borrelia, Tuberculosis, Herpes-simplex-Virus, Mycoplasma and immunologic values of pANCA, cANCA, MPO-AB and PR3-AB.

We diagnosed an idiopathic pseudotumor cerebri.

Within a few days recurrent signs of elevated intracerebral pressure occurred inspite of stepwise (10–20–30–50mg/kg/d) raising dosage of Azetazolamid (AZA). So nine further lumbar punctures were necessary within 3 weeks.

Results: Csf opening pressure was in mean 52.6mmH2O (range19–100). After outflow of in mean 8.2ml csf (5–12ml) the csf-pressure was normalized (<28mmH2O). Finally under treatment with a combination of AZA (50mg/kg/d) and Furosemid (2mg/kg/d) the symptoms vanished one month after begin of disease.

Conclusion: This case report shows an exceptionally early and progressive process of pseudotumor cerebri according to the young age.

Pseudotumor cerebri - papilledema - azetazolamid