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Published in: Child's Nervous System 6/2019

01-06-2019 | Obesity | Original Article

Demographics, clinical features, and response to conventional treatments in pediatric Pseudotumor Cerebri syndrome: a single-center experience

Published in: Child's Nervous System | Issue 6/2019

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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.
Literature
1.
go back to reference Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159–1165CrossRefPubMed Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159–1165CrossRefPubMed
2.
go back to reference 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):561–568CrossRefPubMed 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):561–568CrossRefPubMed
3.
go back to reference Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC (2017) Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol 73:42–47CrossRefPubMed Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC (2017) Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol 73:42–47CrossRefPubMed
4.
go back to reference Mallery RM, Friedman DI, Liu GT (2014) Headache and the pseudotumor cerebri syndrome. Curr Pain Headache Rep 18(9):446CrossRefPubMed Mallery RM, Friedman DI, Liu GT (2014) Headache and the pseudotumor cerebri syndrome. Curr Pain Headache Rep 18(9):446CrossRefPubMed
5.
6.
go back to reference Aylward SC, Waslo CS, Au JN, Tanne E (2016) Manifestations of pediatric intracranial hypertension from the intracranial hypertension registry. Pediatr Neurol 61:76–82CrossRefPubMed Aylward SC, Waslo CS, Au JN, Tanne E (2016) Manifestations of pediatric intracranial hypertension from the intracranial hypertension registry. Pediatr Neurol 61:76–82CrossRefPubMed
7.
go back to reference Aylward SC, Way AL (2018) Pediatric intracranial hypertension: a current literature review. Curr Pain Headache Rep 22(2):14CrossRefPubMed Aylward SC, Way AL (2018) Pediatric intracranial hypertension: a current literature review. Curr Pain Headache Rep 22(2):14CrossRefPubMed
8.
go back to reference Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 127(2):178–182CrossRefPubMed Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 127(2):178–182CrossRefPubMed
9.
go back to reference Smith SV, Friedman DI (2017) The idiopathic intracranial hypertension treatment trial: a review of the outcomes. Headache 57(8):1303–1310CrossRefPubMed Smith SV, Friedman DI (2017) The idiopathic intracranial hypertension treatment trial: a review of the outcomes. Headache 57(8):1303–1310CrossRefPubMed
10.
go back to reference Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS (2010) Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol 34(Suppl 2):217–221PubMed Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS (2010) Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol 34(Suppl 2):217–221PubMed
11.
go back to reference Aylward SC, Aronowitz C, Roach ES (2016) Intracranial hypertension without papilledema in children. J Child Neurol 31(2):177–183CrossRefPubMed Aylward SC, Aronowitz C, Roach ES (2016) Intracranial hypertension without papilledema in children. J Child Neurol 31(2):177–183CrossRefPubMed
12.
go back to reference 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):1389–1392CrossRefPubMedPubMedCentral 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):1389–1392CrossRefPubMedPubMedCentral
14.
go back to reference Phillips PH, Sheldon CA (2017) Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol 37(Suppl 1):S33–S40CrossRefPubMed Phillips PH, Sheldon CA (2017) Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol 37(Suppl 1):S33–S40CrossRefPubMed
15.
go back to reference Markey KA, Mollan SP, Jensen RH, Sinclair AJ (2016) Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 15(1):78–91CrossRef Markey KA, Mollan SP, Jensen RH, Sinclair AJ (2016) Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 15(1):78–91CrossRef
16.
go back to reference 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):247–249CrossRefPubMed 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):247–249CrossRefPubMed
17.
go back to reference 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):313–321CrossRefPubMed 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):313–321CrossRefPubMed
18.
go back to reference 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):870–872CrossRefPubMed 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):870–872CrossRefPubMed
19.
go back to reference 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):2424–2431CrossRefPubMedPubMedCentral 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):2424–2431CrossRefPubMedPubMedCentral
20.
go back to reference 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):690–699CrossRefPubMed 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):690–699CrossRefPubMed
21.
go back to reference Brara SM, Koebnick C, Porter AH, Langer-Gould A (2012) Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr 161(4):602–607CrossRefPubMedPubMedCentral Brara SM, Koebnick C, Porter AH, Langer-Gould A (2012) Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr 161(4):602–607CrossRefPubMedPubMedCentral
23.
go back to reference 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):1063–1073CrossRefPubMed 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):1063–1073CrossRefPubMed
24.
go back to reference 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):741–749CrossRefPubMedPubMedCentral 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):741–749CrossRefPubMedPubMedCentral
25.
go back to reference Balasubramanian S, Shivbalan S, Ganesh R (2005) Pseudotumor cerebri following oral betamethasone for common cold. Indian J Pediatr 72(11):994CrossRefPubMed Balasubramanian S, Shivbalan S, Ganesh R (2005) Pseudotumor cerebri following oral betamethasone for common cold. Indian J Pediatr 72(11):994CrossRefPubMed
26.
go back to reference 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):1143–1151PubMed 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):1143–1151PubMed
27.
go back to reference Dhungana S, Sharrack B, Woodroofe N (2009) Cytokines and chemokines in idiopathic intracranial hypertension. Headache. 49(2):282–285CrossRefPubMed Dhungana S, Sharrack B, Woodroofe N (2009) Cytokines and chemokines in idiopathic intracranial hypertension. Headache. 49(2):282–285CrossRefPubMed
28.
go back to reference Edwards L, Constantinescu C (2010) Cytokines in idiopathic intracranial hypertension CSF. Headache. 50(2):323–325PubMed Edwards L, Constantinescu C (2010) Cytokines in idiopathic intracranial hypertension CSF. Headache. 50(2):323–325PubMed
29.
go back to reference Brodsky MC, Vaphiades M (1998) Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 105(9):1686–1693CrossRefPubMed Brodsky MC, Vaphiades M (1998) Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 105(9):1686–1693CrossRefPubMed
30.
go back to reference Biousse V, Bruce BB, Newman NJ (2012) Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 83(5):488–494CrossRefPubMedPubMedCentral Biousse V, Bruce BB, Newman NJ (2012) Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 83(5):488–494CrossRefPubMedPubMedCentral
Metadata
Title
Demographics, clinical features, and response to conventional treatments in pediatric Pseudotumor Cerebri syndrome: a single-center experience
Publication date
01-06-2019
Published in
Child's Nervous System / Issue 6/2019
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04150-y

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