Abstract
Objective
The goal of this study was to better understand pediatric Pseudotumor Cerebri syndrome, and its relationship to age, obesity, and other medical conditions; and to evaluate response to conventional treatments.
Methods
A retrospective chart review was performed on consecutive patients who were diagnosed with PTCS between January 1, 2007, and July 31, 2014. A total of 78 patients were included in this study: 54 female (69.3%) and 24 male (30.7%). Variables including age, sex, body mass index, concomitant medical conditions, secondary causes, associated symptoms, physical exam findings, imaging results, recurrence of symptoms, and treatment modalities were analyzed. Patients were grouped into “pre-kindergarten,” “elementary,” and “adolescent” based on their age; and weight categories of underweight, normal weight, overweight, moderately, and severely obese.
Results
Mean age of symptom onset was 11.92 ± 4.09 years. Elementary and adolescent age patients were more likely to be overweight, moderately obese, and severely obese, while this finding was not found for patients in pre-kindergarten group. Headache (83.3%) and visual disturbances (48.7%) were the most common presenting complaints. Asthma (16.6%) was the most common associated concomitant medical condition. Medical management resulted in resolution in 84% of population, 15% required surgical interventions, and the recurrence rate was found to be 20.5%. There was a statistically significant trend in success with medical management in younger patients (p = 0.04), while medically refractory PTCS was seen in adolescent females. Recurrence of PTCS had a linear trend with increased occurrence in adolescent age group with higher BMI. Asthma was observed to be frequently associated with PTCS in our cohort. Obesity is strongly associated with PTCS, not only in the adolescent group but also in the younger elementary age group. Treatment remains similar to management in the adults with a good response (84%) to medical management and a low relapse rate.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00381-019-04150-y/MediaObjects/381_2019_4150_Fig1_HTML.png)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs00381-019-04150-y/MediaObjects/381_2019_4150_Fig2_HTML.png)
Similar content being viewed by others
Abbreviations
- IIH:
-
Idiopathic intracranial hypertension
- PTCS:
-
Pseudotumor Cerebri syndrome
- ICP:
-
Intracranial pressure
- CSF:
-
Cerebrospinal fluid
- BMI:
-
Body mass index
- MRI:
-
Magnetic resonance imaging
- MRV:
-
Magnetic resonance venography
- LPS:
-
Lumboperitoneal shunt
- VPS:
-
Ventriculoperitoneal shunt
References
Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159–1165
Per H, Canpolat M, Gumus H et al (2013) Clinical spectrum of the pseudotumor cerebri in children: etiological, clinical features, treatment and prognosis. Brain and Development 35(6):561–568
Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC (2017) Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol 73:42–47
Mallery RM, Friedman DI, Liu GT (2014) Headache and the pseudotumor cerebri syndrome. Curr Pain Headache Rep 18(9):446
Rook BS, Phillips PH (2016) Pediatric pseudotumor cerebri. Curr Opin Ophthalmol 27(5):416–419
Aylward SC, Waslo CS, Au JN, Tanne E (2016) Manifestations of pediatric intracranial hypertension from the intracranial hypertension registry. Pediatr Neurol 61:76–82
Aylward SC, Way AL (2018) Pediatric intracranial hypertension: a current literature review. Curr Pain Headache Rep 22(2):14
Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 127(2):178–182
Smith SV, Friedman DI (2017) The idiopathic intracranial hypertension treatment trial: a review of the outcomes. Headache 57(8):1303–1310
Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS (2010) Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol 34(Suppl 2):217–221
Aylward SC, Aronowitz C, Roach ES (2016) Intracranial hypertension without papilledema in children. J Child Neurol 31(2):177–183
Faz G, Butler IJ, Koenig MK (2010) Incidence of papilledema and obesity in children diagnosed with idiopathic “benign” intracranial hypertension: case series and review. J Child Neurol 25(11):1389–1392
Reid JE, Reem RE, Aylward SC, Rogers DL (2016) Sixth nerve palsy in Paediatric intracranial hypertension. Neuroophthalmology 40(1):23–27
Phillips PH, Sheldon CA (2017) Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol 37(Suppl 1):S33–S40
Markey KA, Mollan SP, Jensen RH, Sinclair AJ (2016) Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 15(1):78–91
Genizi J, Lahat E, Zelnik N, Mahajnah M, Ravid S, Shahar E (2007) Childhood-onset idiopathic intracranial hypertension: relation of sex and obesity. Pediatr Neurol 36(4):247–249
Tibussek D, Schneider DT, Vandemeulebroecke N, Turowski B, Messing-Juenger M, Willems PHGM, Mayatepek E, Distelmaier F (2010) Clinical spectrum of the pseudotumor cerebri complex in children. Childs Nerv Syst 26(3):313–321
Balcer LJ, Liu GT, Forman S, Pun K, Volpe NJ, Galetta SL, Maguire MG (1999) Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology. 52(4):870–872
Sheldon CA, Paley GL, Xiao R, Kesler A, Eyal O, Ko MW, Boisvert CJ, Avery RA, Salpietro V, Phillips PH, Heidary G, McCormack SE, Liu GT (2016) Pediatric idiopathic intracranial hypertension: age, gender, and anthropometric features at diagnosis in a large, retrospective, multisite cohort. Ophthalmology 123(11):2424–2431
Degerliyurt A, Teber S, Karakaya G et al (2014) Pseudotumor cerebri/idiopathic intracranial hypertension in children: an experience of a tertiary care hospital. Brain and Development 36(8):690–699
Brara SM, Koebnick C, Porter AH, Langer-Gould A (2012) Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr 161(4):602–607
Peters U, Dixon AE, Forno E (2018) Obesity and asthma. J Allergy Clin Immunol 141(4):1169–1179
Ahmadizar F, Vijverberg SJ, Arets HG et al (2016) Childhood obesity in relation to poor asthma control and exacerbation: a meta-analysis. Eur Respir J 48(4):1063–1073
Forno E, Lescher R, Strunk R, Weiss S, Fuhlbrigge A, Celedón JC (2011) Decreased response to inhaled steroids in overweight and obese asthmatic children. J Allergy Clin Immunol 127(3):741–749
Balasubramanian S, Shivbalan S, Ganesh R (2005) Pseudotumor cerebri following oral betamethasone for common cold. Indian J Pediatr 72(11):994
Dees SC, Mc KH Jr (1959) Occurrence of pseudotumor cerebri (benign intracranial hypertension) during treatment of children with asthma by adrenal steroids; report of three cases. Pediatrics 23(6):1143–1151
Dhungana S, Sharrack B, Woodroofe N (2009) Cytokines and chemokines in idiopathic intracranial hypertension. Headache. 49(2):282–285
Edwards L, Constantinescu C (2010) Cytokines in idiopathic intracranial hypertension CSF. Headache. 50(2):323–325
Brodsky MC, Vaphiades M (1998) Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 105(9):1686–1693
Biousse V, Bruce BB, Newman NJ (2012) Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 83(5):488–494
Acknowledgements
Statistical support for this research was underwritten by the University of Louisville Office of Graduate Medical Education.
Funding
No financial supports were used to perform this study.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Bhalla, S., Nickel, N.E., Mutchnick, I. et al. Demographics, clinical features, and response to conventional treatments in pediatric Pseudotumor Cerebri syndrome: a single-center experience. Childs Nerv Syst 35, 991–998 (2019). https://doi.org/10.1007/s00381-019-04150-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-019-04150-y