EN | TR
E-ISSN: 1307-5608
Retrospective Evaluation of Children With Pseudotumor Cerebri: A Single Center Experience
PDF
Cite
Share
Request
Research Article
VOLUME: 74 ISSUE: 3
P: 283-288
December 2021

Retrospective Evaluation of Children With Pseudotumor Cerebri: A Single Center Experience

J Ankara Univ Fac Med 2021;74(3):283-288
DOI: 10.4274/atfm.galenos.2021.27879
Miraç Yıldırım
1. Ankara Üniversitesi Tıp Fakültesi, Nöroloji Anabilim Dalı, Çocuk Nörolojisi Bilim Dalı, Ankara, Türkiye
No information available.
No information available
Received Date: 12.05.2021
Accepted Date: 02.06.2021
Publish Date: 17.09.2021
PDF
Cite
Share
Request

ABSTRACT

Objectives:

Pseudotumor cerebri is a rare neurological condition in which signs of increased intracranial pressure are observed and there are no intracranial space-occupying pathologies. The aim of our study is to determine the demographic characteristics, diagnosis, treatments and prognosis of patients with pseudotumor cerebri.

Materials and Methods:

The data of 15 patients (9 girls, 6 boys) who were followed up with the diagnosis of pseudotumor cerebri between September 2017 and March 2019 in a tertiary training and research hospital pediatric neurology outpatient clinic were retrospectively evaluated.

Results:

The mean age of the patients was 10.26±4.75 years (range: 1-16 years). The most common signs and symptoms during the first admission were papilledema , headache, nausea-vomiting, weakness, blurred vision and double vision. Brain magnetic resonance imaging (MRI) was performed in all patients. Supporting findings of increased intracranial pressure were detected in three patients. The mean cerebrospinal fluid inlet pressure was 306±75 mmH2O (range: 260-470 mmH2O). Etiologically, the use of growth hormone (n=2), Down syndrome (n=1), Guillain-Barré syndrome (n=1), familial Mediterranean fever (n=1), Chiari malformation type 1 (n=1), acute lymphoblastic leukemia (n=1), vitamin A deficiency (n=1), obesity (n=1) and MODY tip 3 (n=1) were detected. Acetazolamide was started in all patients as initial therapy. Nine of 15 patients had full recovery, five of 15 patients had significant improvement in papilledema findings, and one of 15 patients had partial improvement. None of the patients developed permanent vision loss or visual field defect. No recurrence was observed in any patient after treatment discontinuation or during treatment.

Conclusion:

Pseudotumor cerebri is more common in childhood, especially in the postpubertal period. Obesity is a rare cause in childhood. An etiological cause that may cause pseudotumor cerebri can be detected in more than half of the pediatric patients, and the use of growth hormone was the most common in our study. Pseudotumor cerebri is a rare disease that requires long-term treatment and follow-up, but the outcome is generally well in patients who are diagnosed early, receive particular treatment and comply well with treatment.

Keywords: Pseudotumor Cerebri, Papilledema, Childhood, Treatment

References

1
Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81:1159-1165.
2
Mosquera Gorostidi A, Iridoy Zulet M, Azcona Ganuza G, et al. Pseudotumour cerebri in children: Aetiology, clinical features, and progression. Neurologia (Engl Ed). 2019;34:89-97.
3
Aylward SC, Waslo CS, Au JN, et al. Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry. Pediatr Neurol. 2016;61:76-82.
4
Per H, Canpolat M, Gümüş H, et al. Clinical spectrum of the pseudotumor cerebri in children: etiological, clinical features, treatment and prognosis. Brain Dev. 2013;35:561-568.
5
Spennato P, Ruggiero C, Parlato RS, et al. Pseudotumor cerebri. Childs Nerv Syst. 2011;27:215-235.
6
Dessardo NS, Dessardo S, Sasso A, et al. Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol. 2010;34 Suppl 2:217-221.
7
Tibussek D, Schneider DT, Vandemeulebroecke N, et al. Clinical spectrum of the pseudotumor cerebri complex in children. Childs Nerv Syst. 2010;26:313-321.
8
Digre KB. Idiopathic intracranial hypertension headache. Curr Pain Headache Rep. 2002;6:217-225.
9
Pearce JM. From pseudotumour cerebri to idiopathic intracranial hypertension. Pract Neurol. 2009;9:353-356.
10
Görkem SB, Doğanay S, Canpolat M, et al. MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst. 2015;31:373-380.
11
Kohli AA, Vossough A, Mallery RM, et al. Magnetic Resonance Imaging Findings in Pediatric Pseudotumor Cerebri Syndrome. Pediatr Neurol. 2019;99:31-39.
12
Matthews YY. Drugs used in childhood idiopathic or benign intracranial hypertension. Arch Dis Child Educ Pract Ed. 2008;93:19-25.
13
Ahmad SR, Moss HE. Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension. Semin Neurol. 2019;39:682-691.
14
Hamedani AG, Thibault DP, Revere KE, et al. Trends in the Surgical Treatment of Pseudotumor Cerebri Syndrome in the United States. JAMA Netw Open. 2020;3:e2029669.
15
Phillips PH, Repka MX, Lambert SR. Pseudotumor cerebri in children. J AAPOS. 1998;2:33-38.
16
İncecik F, Hergüner MO, Altunbaşak S. Evaluation of sixteen children with pseudotumor cerebri. Turk J Pediatr. 2011;53:55-58.
17
Standridge SM. Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol. 2010;43:377-390.
18
Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol. 2002;17:745-748.
19
Ravid S, Shahar E, Schif A, et al. Visual Outcome and Recurrence Rate in Children With Idiopathic Intracranial Hypertension. J Child Neurol. 2015;30:1448-1452.
20
Soiberman U, Stolovitch C, Balcer LJ, et al. Idiopathic intracranial hypertension in children: visual outcome and risk of recurrence. Childs Nerv Syst. 2011;27:1913-1918.
21
Greener DL, Akarca D, Durnford AJ, et al. Idiopathic Intracranial Hypertension: Shunt Failure and the Role of Obesity. World Neurosurg. 2020;137:e83-e88.
22
Faz G, Butler IJ, Koenig MK. Incidence of papilledema and obesity in children diagnosed with idiopathic “benign” intracranial hypertension: case series and review. J Child Neurol. 2010;25:1389-1392.
23
Sheldon CA, Paley GL, Xiao R, et al. Pediatric Idiopathic Intracranial Hypertension: Age, Gender, and Anthropometric Features at Diagnosis in a Large, Retrospective, Multisite Cohort. Ophthalmology. 2016;123:2424-2431.
24
Burkett JG, Ailani J. An Up to Date Review of Pseudotumor Cerebri Syndrome. Curr Neurol Neurosci Rep. 2018;18:33.
25
Değerliyurt A, Teber S, Karakaya G, et al. Pseudotumor cerebri/idiopathic intracranial hypertension in children: an experience of a tertiary care hospital. Brain Dev. 2014;36:690-699.
26
Barmherzig R, Szperka CL. Pseudotumor Cerebri Syndrome in Children. Curr Pain Headache Rep. 2019;23:58.
27
Zaki SA, Lad V, Abdagire N. Vitamin D deficiency rickets presenting as pseudotumor cerebri. J Neurosci Rural Pract. 2013;4:464-466.
28
Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89:1088-1100.
29
Babikian P, Corbett J, Bell W. Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol. 1994;9:144-149.
30
Hacifazlioglu Eldes N, Yilmaz Y. Pseudotumour cerebri in children: etiological, clinical features and treatment modalities. Eur J Paediatr Neurol. 2012;16:349-355.
31
Spitze A, Malik A, Lee AG. Surgical and endovascular interventions in idiopathic intracranial hypertension. Curr Opin Neurol. 2014;27:69-74.
32
Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull. 2006;79-80:233-244.
2024 ©️ Galenos Publishing House