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Published in: Child's Nervous System 3/2010

01-03-2010 | Original Paper

Clinical spectrum of the pseudotumor cerebri complex in children

Authors: Daniel Tibussek, Dominik T. Schneider, Nicola Vandemeulebroecke, Bernd Turowski, Martina Messing-Juenger, Peter H. G. M. Willems, Ertan Mayatepek, Felix Distelmaier

Published in: Child's Nervous System | Issue 3/2010

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Abstract

Purpose

Our aim was to improve diagnosis and management of pseudotumor cerebri (PTC; also known as idiopathic intracranial hypertension) in children.

Methods

We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 53 consecutive patients.

Results

We identified several important aspects to be considered in the management of these children. First, patients may present without obvious symptoms at diagnosis. Second, bilateral papilledema might not or not yet be present in symptomatic patients. Third, measurement of cerebrospinal fluid (CSF) opening pressure may not always be reliable due to drugs used for sedation, which may alter intracranial pressure. Fourth, normal CSF pressure values in childhood are not well established and diagnosis might even be justified if pressure is <20 cm H2O. Fifth, associated conditions are frequent (at least in our cohort); however, in most cases, a causative link cannot be proven. Finally, disease relapse is a serious problem (20% in our group), which stresses the importance of standardized follow-up programs.

Conclusions

PTC constitutes an important and possibly underrecognized disorder in children and adolescents. Considering the high percentage of possibly associated conditions in our study, a detailed diagnostic work-up is crucial to identify treatable underlying conditions.
Literature
2.
go back to reference Distelmaier F, Gobel U, Vandemeulebroecke N, Mayatepek E, Rosenbaum T, Laws HJ (2007) Secondary pseudotumor cerebri in pediatric oncology and hematology: an unpredictable condition of varying etiology. Pediatric Blood Cancer 49:1029–1033CrossRefPubMed Distelmaier F, Gobel U, Vandemeulebroecke N, Mayatepek E, Rosenbaum T, Laws HJ (2007) Secondary pseudotumor cerebri in pediatric oncology and hematology: an unpredictable condition of varying etiology. Pediatric Blood Cancer 49:1029–1033CrossRefPubMed
3.
go back to reference Quincke H (1897) Ueber Meningitis serosa und verwande Zustande. Dtsch Z Nervenheil 9:140–168 Quincke H (1897) Ueber Meningitis serosa und verwande Zustande. Dtsch Z Nervenheil 9:140–168
4.
go back to reference Distelmaier F, Tibussek D, Schneider DT, Mayatepek E (2007) Seasonal variation and atypical presentation of idiopathic intracranial hypertension in pre-pubertal children. Cephalalgia 27:1261–1264CrossRefPubMed Distelmaier F, Tibussek D, Schneider DT, Mayatepek E (2007) Seasonal variation and atypical presentation of idiopathic intracranial hypertension in pre-pubertal children. Cephalalgia 27:1261–1264CrossRefPubMed
5.
go back to reference Distelmaier F, Sengler U, Messing-Juenger M, Assmann B, Mayatepek E, Rosenbaum T (2006) Pseudotumor cerebri as an important differential diagnosis of papilledema in children. Brain Dev 28:190–195CrossRefPubMed Distelmaier F, Sengler U, Messing-Juenger M, Assmann B, Mayatepek E, Rosenbaum T (2006) Pseudotumor cerebri as an important differential diagnosis of papilledema in children. Brain Dev 28:190–195CrossRefPubMed
6.
go back to reference Distelmaier F, Mayatepek E, Tibussek D (2008) Probable idiopathic intracranial hypertension in pre-pubertal children. Arch Dis Child 93:356–357CrossRefPubMed Distelmaier F, Mayatepek E, Tibussek D (2008) Probable idiopathic intracranial hypertension in pre-pubertal children. Arch Dis Child 93:356–357CrossRefPubMed
7.
go back to reference Kromeyer-Hauschild K, Wabitsch M, Kunze D (2001) Perzentile für den Body Mass Index für Kinder im Alter von 0 bis 18 Jahren. Monatszeitschrift Kinderheilkunde 149:807–818CrossRef Kromeyer-Hauschild K, Wabitsch M, Kunze D (2001) Perzentile für den Body Mass Index für Kinder im Alter von 0 bis 18 Jahren. Monatszeitschrift Kinderheilkunde 149:807–818CrossRef
8.
go back to reference Lewis DW (2002) Headaches in children and adolescents. Am Fam Physician 65:625–632PubMed Lewis DW (2002) Headaches in children and adolescents. Am Fam Physician 65:625–632PubMed
9.
go back to reference Babikian P, Corbett J, Bell W (1994) Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol 9:144–149CrossRefPubMed Babikian P, Corbett J, Bell W (1994) Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol 9:144–149CrossRefPubMed
10.
go back to reference Scott IU, Siatkowski RM, Eneyni M, Brodsky MC, Lam BL (1997) Idiopathic intracranial hypertension in children and adolescents. Am J Ophthalmol 124:253–255PubMed Scott IU, Siatkowski RM, Eneyni M, Brodsky MC, Lam BL (1997) Idiopathic intracranial hypertension in children and adolescents. Am J Ophthalmol 124:253–255PubMed
11.
go back to reference Wraige E, Chandler C, Pohl KR (2002) Idiopathic intracranial hypertension: is papilloedema inevitable? Arch Dis Child 87:223–224CrossRefPubMed Wraige E, Chandler C, Pohl KR (2002) Idiopathic intracranial hypertension: is papilloedema inevitable? Arch Dis Child 87:223–224CrossRefPubMed
12.
go back to reference Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal paediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 127:178–182CrossRefPubMed Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal paediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 127:178–182CrossRefPubMed
13.
go back to reference Lim M, Kurian M, Penn A, Calver D, Lin JP (2005) Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 90:206–210CrossRefPubMed Lim M, Kurian M, Penn A, Calver D, Lin JP (2005) Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 90:206–210CrossRefPubMed
14.
go back to reference Weig SG (2002) Asymptomatic idiopathic intracranial hypertension in young children. J Child Neurol 17:239–241CrossRefPubMed Weig SG (2002) Asymptomatic idiopathic intracranial hypertension in young children. J Child Neurol 17:239–241CrossRefPubMed
15.
go back to reference Bassan H, Berkner L, Stolovitch C, Kesler A (2008) Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 118:251–255CrossRefPubMed Bassan H, Berkner L, Stolovitch C, Kesler A (2008) Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 118:251–255CrossRefPubMed
16.
go back to reference Talke P, Caldwell JE, Richardson CA (1999) Sevoflurane increases lumbar cerebrospinal fluid pressure in normocapnic patients undergoing transsphenoidal hypophysectomy. Anesthesiology 91:127–130CrossRefPubMed Talke P, Caldwell JE, Richardson CA (1999) Sevoflurane increases lumbar cerebrospinal fluid pressure in normocapnic patients undergoing transsphenoidal hypophysectomy. Anesthesiology 91:127–130CrossRefPubMed
17.
go back to reference Ben Yehuda Y, Watemberg N (2006) Ketamine increases opening cerebrospinal pressure in children undergoing lumbar puncture. J Child Neurol 21:441–443PubMed Ben Yehuda Y, Watemberg N (2006) Ketamine increases opening cerebrospinal pressure in children undergoing lumbar puncture. J Child Neurol 21:441–443PubMed
18.
go back to reference Engelhard K, Werner C (2006) Inhalational or intravenous anesthetics for craniotomies? Pro inhalational. Curr Opin Anaesthesiol 19:504–508CrossRefPubMed Engelhard K, Werner C (2006) Inhalational or intravenous anesthetics for craniotomies? Pro inhalational. Curr Opin Anaesthesiol 19:504–508CrossRefPubMed
19.
go back to reference Gallagher A, Pike M, Berg S (2004) Beware CSF pressure measured under general anaesthesia. Arch Dis Child 89:691CrossRefPubMed Gallagher A, Pike M, Berg S (2004) Beware CSF pressure measured under general anaesthesia. Arch Dis Child 89:691CrossRefPubMed
20.
go back to reference Mishra A, Mordekar SR, Rennie IG, Baxter PS (2007) False diagnosis of papilloedema and idiopathic intracranial hypertension. Eur J Paediatr Neurol 11:39–42CrossRefPubMed Mishra A, Mordekar SR, Rennie IG, Baxter PS (2007) False diagnosis of papilloedema and idiopathic intracranial hypertension. Eur J Paediatr Neurol 11:39–42CrossRefPubMed
21.
go back to reference Johnston I, Paterson A (1974) Benign intracranial hypertension. II. CSF pressure and circulation. Brain 97:301–312CrossRefPubMed Johnston I, Paterson A (1974) Benign intracranial hypertension. II. CSF pressure and circulation. Brain 97:301–312CrossRefPubMed
22.
go back to reference Gucer G, Viernstein LJ (1978) Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg 49:256–263CrossRefPubMed Gucer G, Viernstein LJ (1978) Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg 49:256–263CrossRefPubMed
23.
go back to reference Rangwala LM, Liu GT (2007) Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 52:597–617CrossRefPubMed Rangwala LM, Liu GT (2007) Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 52:597–617CrossRefPubMed
24.
go back to reference Lin A, Foroozan R, Danesh-Meyer HV, De Salvo G, Savino PJ, Sergott RC (2006) Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophthalmology 113:2281–2284CrossRefPubMed Lin A, Foroozan R, Danesh-Meyer HV, De Salvo G, Savino PJ, Sergott RC (2006) Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophthalmology 113:2281–2284CrossRefPubMed
25.
go back to reference Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ (2003) Anemia and papilledema. Am J Ophthalmol 135:437–446CrossRefPubMed Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ (2003) Anemia and papilledema. Am J Ophthalmol 135:437–446CrossRefPubMed
26.
go back to reference Friedman DI, Jacobson DM (2002) Diagnostic criteria for idiopathic intracranial hypertension. Neurology 59:1492–1495CrossRefPubMed Friedman DI, Jacobson DM (2002) Diagnostic criteria for idiopathic intracranial hypertension. Neurology 59:1492–1495CrossRefPubMed
27.
go back to reference Youroukos S, Psychou F, Fryssiras S, Paikos P, Nicolaidou P (2000) Idiopathic intracranial hypertension in children. J Child Neurol 15:453–457CrossRefPubMed Youroukos S, Psychou F, Fryssiras S, Paikos P, Nicolaidou P (2000) Idiopathic intracranial hypertension in children. J Child Neurol 15:453–457CrossRefPubMed
28.
go back to reference Soler D, Cox T, Bullock P, Calver DM, Robinson RO (1998) Diagnosis and management of benign intracranial hypertension. Arch Dis Child 78:89–94CrossRefPubMed Soler D, Cox T, Bullock P, Calver DM, Robinson RO (1998) Diagnosis and management of benign intracranial hypertension. Arch Dis Child 78:89–94CrossRefPubMed
29.
go back to reference Loh Y, Labutta RJ, Urban ES (2004) Idiopathic intracranial hypertension and postlumbar puncture headache. Headache 44:170–173CrossRefPubMed Loh Y, Labutta RJ, Urban ES (2004) Idiopathic intracranial hypertension and postlumbar puncture headache. Headache 44:170–173CrossRefPubMed
30.
go back to reference Orssaud C, Dureau P, Zerah M, Cinalli G, Sainte Rose C, Kahn AP, Dufier JL (2001) Benign childhood intracranial hypertension. J Fr Ophtalmol 24:54–59PubMed Orssaud C, Dureau P, Zerah M, Cinalli G, Sainte Rose C, Kahn AP, Dufier JL (2001) Benign childhood intracranial hypertension. J Fr Ophtalmol 24:54–59PubMed
31.
go back to reference Weiss B, Collmann H, Schropp C (2007) Idiopathic intracranial hypertension—a review over 16 cases in childhood. Presented at the Annual German Neuropediatrics Meeting, Passau, Germany, 22–25 March 2007 Weiss B, Collmann H, Schropp C (2007) Idiopathic intracranial hypertension—a review over 16 cases in childhood. Presented at the Annual German Neuropediatrics Meeting, Passau, Germany, 22–25 March 2007
32.
go back to reference Frisén L (1982) Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 45:13–18CrossRefPubMed Frisén L (1982) Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry 45:13–18CrossRefPubMed
33.
go back to reference Ng YT, Bodensteiner JB (2003) Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 18:440PubMed Ng YT, Bodensteiner JB (2003) Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 18:440PubMed
34.
go back to reference Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD (2004) Idiopathic intracranial hypertension: risk of recurrences. Neurology 63:1737–1739PubMed Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD (2004) Idiopathic intracranial hypertension: risk of recurrences. Neurology 63:1737–1739PubMed
35.
go back to reference Winnick S, Lucas DO, Hartman AL, Toll D (2005) How do you improve compliance? Pediatrics 115:e718–e724CrossRefPubMed Winnick S, Lucas DO, Hartman AL, Toll D (2005) How do you improve compliance? Pediatrics 115:e718–e724CrossRefPubMed
36.
go back to reference Stiebel-Kalish H, Kalish Y, Lusky M, Gaton DD, Ehrlich R, Shuper A (2006) Puberty as a risk factor for less favorable visual outcome in idiopathic intracranial hypertension. Am J Ophthalmol 142:279–283CrossRefPubMed Stiebel-Kalish H, Kalish Y, Lusky M, Gaton DD, Ehrlich R, Shuper A (2006) Puberty as a risk factor for less favorable visual outcome in idiopathic intracranial hypertension. Am J Ophthalmol 142:279–283CrossRefPubMed
Metadata
Title
Clinical spectrum of the pseudotumor cerebri complex in children
Authors
Daniel Tibussek
Dominik T. Schneider
Nicola Vandemeulebroecke
Bernd Turowski
Martina Messing-Juenger
Peter H. G. M. Willems
Ertan Mayatepek
Felix Distelmaier
Publication date
01-03-2010
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 3/2010
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-009-1018-0

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