Do infantile, prepubertal and pubertal pseudotumor cerebri share the same characteristics? A retrospective review of forty cases in southern part of Iran

M.J.Yavari, S.Inaloo, MD, F.Rastgoo

Shiraz University of medical science, Department of pediatric neurology, Namazi hospital, Shiraz, Fars province, Iran

Introduction: Pediatric pseudotumor cerebri (PTC) is a well-known syndrome in the pediatric population. It is characterized by symptoms and signs of increased intracranial pressure without evidence of an intracranial or obstructive process, mass lesion and hydrocephaly, and with normal cerebrospinal fluid (CSF) contents. It is rare in the pediatric age and presents with clinical features that differ from the clinical picture seen in adults. Our aim of study was to determine whether infants, prepubertal and pubertal children with pseudotumor cerebri differ from each other by characteristics emphasis on clinical presentation, etiological factors and outcomes.

Material and methods:The medical records of patients diagnosed with PTC in two major university hospitals (Namazi and Dastgheyb hospitals) in Shiraz, Fars province, southern part of Iran during an eight and a half years period (1999-June 2007) were obtained. A total of 37 patients (13 males and 27 females) fulfilled the Dandy diagnostic criteria for PTC. The patients were categorized in three age groups: infancy, prepuberty and puberty. The following data were reviewed for each group: age at diagnosis, sex, clinical features, etiological factors, presence of obesity, opening CSF pressure and CSF analysis, ophthalmologic examination, CT-scan and magnetic resonance imaging (MRI) results, treatment, and outcomes. Statistical analysis for each group was performed by statistical software SSPS version 9 and necessary information for further assessments was obtained.

Results:The diagnosis of PTC was confirmed in 13 infants, 13 prepubertal and 14 pubertal children. The most common clinical manifestations in infantile PTC were fontanel bulging in 13(%100) and vomiting in 10(%76.9) and in prepubertal PTC were vomiting and headache in 9(%69.2) each and papilledema in 8(%61.5) and in pubertal PTC were headache in 14(%100), papilledema in 12(%85.7) and vomiting in 10(%71.4).

The only etiological factor for infantile PTC was drugs of which nalidixic acid was the most common drug found in 10(%76.9). The most common etiological factor in prepubertal PTC was idiopathic seen in 5(%38.4) and head trauma and nalidixic acid consumption in 2(%15.3) each. The most common etiological factor in pubertal PTC were oral contraceptive pills for ovarian cyst and idiopathic in 5(%35.7) each. Outcome was favorable for all the cases, except for two cases with recurrence in prepubertal PTC and two cases, one with advanced depression of visual field and one with recurrence in pubertal PTC.

Discussion and Conclusion:Prepubertal and pubertal PTC didn’t differ significantly from each other in respect to clinical manifestations but infantile PTC didn’t present in the same fashion as in its counterparts prepubertal and pubertal PTC. Etiological factors were much different in these three groups. As nalidixic acid was the most common cause of PTC in infants, it should be administered carefully by physicians. Outcome was much more favorable in infantile PTC than prepubertal and pubertal in respect to recurrence and complications.

Key words:Infantile, prepubertal, pubertal pseudotumor cerebri, increased intracranial pressure, clinical presentation, etiological factors, outcomes