Abstract
Background. Diagnostics of idiopathic intracranial hypertension (pseudotumor cerebri, PTC) in childhood is often difficult because the clinical picture differs from that in the adult population.
Considering the significant variability of the disease course and prognosis, it is difficult to standardize therapeutic procedures. Our study presents experiences in a relatively large group of pediatric patients with pseudotumor cerebri.
Methods. We have retrospectively analyzed clinical data of children hospitalized for pseudotumor cerebri in the Pediatric Neurology Clinic, 2nd Medical Faculty, Charles University and Faculty Hospital Motol in the years 1998-2002.
Patients were comprehensively examined using imaging (CT, MRI), electrophysiological (EEG, VEP) and ophthalmological (ophthalmoscopy, visual acuity, perimeter, US of papillae, ophthalmoscopic photography) methods, all had lumbar puncture with CSF pressure measurement. Results.
Pseudotumor cerebri was diagnosed in 14 children, age 4-17. Habitus typical of adult patients with PTC (obese girl) was not found in pre-adolescent children.
Some clinical features were different firom the adult population (e.g., the absence of tinnitus or, in contrast, a frequent occurrence of meningeal syndrome). In three patients, we have not demonstrated increased CSF pressure, most likely because of its temporal fluctuation.
MRI played a central role in the diagnostics: it excluded or demonstrated cerebral venous thrombosis (found in 2 patients with clinical presentation of PTC) and it further revealed indirect signs of intracranial hypertension in 9/15 patients. In 13 out of 16 patients, therapy with p.o. acetazolamide and/or i.v. methylprednisolone was successful.
In the 3 remaining refractory cases, lumboperitoneal shunt and/or decompressive surgery of optic nerve sheaths was indicated. In two patients, irreversible vision impairment has developed.
Discussion. We propose a diagnostic protocol for patients with suspected PTC, including speciál MRI studies focusing on optic nerves.
Central role in follow-up is played by careful examination of visual function with perimetry, VEP, US of papillae and photo documentation of fundus. Finally, we discuss indication criteria for surgery in patients at risk for vision loss.