Journal of Pediatric Neurology 2015; 13(01): 058-061
DOI: 10.1055/s-0035-1555157
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Management of Pediatric Pseudotumor Cerebri Syndrome

Valeria Dipasquale
1   Department of Pediatrics, University of Messina, Messina, Italy
,
Gabriella Di Rosa
2   Division of Infantile Neuropsychiatry, Department of Pediatrics, University of Messina, Messina, Italy
,
Salvatore Savasta
3   Department of Pediatrics, University of Pavia, IRCCS San Matteo, Pavia, Italy
,
Ornella Merlo
4   Faculty of Medicine and Surgery, University of Messina, Messina, Italy
,
Daniela Concolino
5   Department of Pediatrics, Magna Graecia University, Catanzaro, Italy
,
Teresa Arrigo
1   Department of Pediatrics, University of Messina, Messina, Italy
› Author Affiliations
Further Information

Publication History

17 January 2015

15 February 2015

Publication Date:
13 July 2015 (online)

Abstract

Pseudotumor cerebri syndrome (PTCS) is a clinical condition characterized by signs and symptoms of elevated intracranial pressure without any radiographic evidence of a mass lesion in the brain, and no abnormalities of the brain parenchyma and cerebrospinal fluid (CSF) composition. Children with PTCS typically present with various combinations of headache, diplopia, and pulsatile tinnitus. Although this syndrome has long been considered a benign condition, it may lead to permanent visual impairment if not promptly and correctly treated. Complete remission of PTCS is associated with a decrease in intracranial pressure, the disappearance of clinical symptoms, and the reversion of papilledema at the funduscopic examination. PTCS has been associated with several comorbidities, but its exact pathophysiology has yet to be fully elucidated. Because the origin of PTCS remains unclear, an etiologically targeted treatment has not yet been identified. Current medical treatments include diuretics (e.g., acetazolamide, furosemide) able to reduce CSF production and pressure, and CSF diversion procedures in the most severe cases. Future elucidation of the pathophysiologic mechanism(s) underlying impaired CSF dynamics in PTCS should help control more finely the CSF formation by choroid plexus with novel drugs having fewer side effects than the commonly used diuretics (e.g., acetazolamide). In this review, we briefly summarize the current therapeutic options in pediatric PTCS.

 
  • References

  • 1 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
  • 2 Radhakrishnan K, Ahlskog JE, Cross SA, Kurland LT, O'Fallon WM. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol 1993; 50 (1) 78-80
  • 3 Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59 (10) 1492-1495
  • 4 Salpietro V, Polizzi A, Di Rosa G , et al. Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications. Int J Endocrinol 2014; 2014: 282489
  • 5 Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007; 52 (6) 597-617
  • 6 Salpietro V, Ruggieri M. Pseudotumor cerebri pathophysiology: the likely role of aldosterone. Headache 2014; 54 (7) 1229
  • 7 Salpietro V, Chimenz R, Arrigo T, Ruggieri M. Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a role for weight gain. J Pediatr 2013; 162 (5) 1084
  • 8 Standridge SM. Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol 2010; 43 (6) 377-390
  • 9 Paley GL, Sheldon CA, Burrows EK, Chilutti MR, Liu GT, McCormack SE. Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome. Am J Ophthalmol 2015; 159 (2) 344-52.e1
  • 10 Soiberman U, Stolovitch C, Balcer LJ, Regenbogen M, Constantini S, Kesler A. Idiopathic intracranial hypertension in children: visual outcome and risk of recurrence. Childs Nerv Syst 2011; 27 (11) 1913-1918
  • 11 Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr 2010; 74 (6) 381-389
  • 12 Galgano MA, Deshaies EM. An update on the management of pseudotumor cerebri. Clin Neurol Neurosurg 2013; 115 (3) 252-259
  • 13 Spennato P, Ruggiero C, Parlato RS , et al. Pseudotumor cerebri. Childs Nerv Syst 2011; 27 (2) 215-235
  • 14 Salpietro V, Ruggieri M, Sancetta F , et al. New insights on the relationship between pseudotumor cerebri and secondary hyperaldosteronism in children. J Hypertens 2012; 30 (3) 629-630
  • 15 Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol 2004; 24 (2) 138-145
  • 16 King JO, Mitchell PJ, Thomson KR, Tress BM. Cerebral venography and manometry in idiopathic intracranial hypertension. Neurology 1995; 45 (12) 2224-2228
  • 17 Mathews MK, Sergott RC, Savino PJ. Pseudotumor cerebri. Curr Opin Ophthalmol 2003; 14 (6) 364-370
  • 18 Johnson LN, Krohel GB, Madsen RW, March Jr GA. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology 1998; 105 (12) 2313-2317
  • 19 Liu GT, Volpe NJ, Galetta SL. Pseudotumor cerebri and its medical treatment. Drugs Today (Barc) 1998; 34 (6) 563-574
  • 20 McCarthy KD, Reed DJ. The effect of acetazolamide and furosemide on cerebrospinal fluid production and choroid plexus carbonic anhydrase activity. J Pharmacol Exp Ther 1974; 189 (1) 194-201
  • 21 Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD. Idiopathic intracranial hypertension: risk of recurrences. Neurology 2004; 63 (9) 1737-1739
  • 22 Kahle KT, Walcott BP, Staley KJ. Resolution of headache and papilledema in idiopathic intracranial hypertension associated with inhibition of Na+-K+-2Cl- cotransport. J Child Neurol 2011; 26 (2) 205-208
  • 23 Celebisoy N, Gökçay F, Sirin H, Akyürekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand 2007; 116 (5) 322-327
  • 24 Deftereos SN, Panagopoulos G, Georgonikou D, Karageorgiou CE, Karageorgiou E, Piadites G. Treatment of idiopathic intracranial hypertension: is there a place for octreotide?. Cephalalgia 2011; 31 (16) 1679-1680
  • 25 Salpietro V, Ruggieri M, Alterio T , et al. Is there a risk of pubertal worsening in primary intestinal lymphangiectasia?. J Endocrinol Invest 2013; 36 (11) 1128
  • 26 Burness CB, Dhillon S, Keam SJ. Lanreotide Autogel(®): a review of its use in the treatment of patients with acromegaly. Drugs 2014; 74 (14) 1673-1691
  • 27 Khan MU, Khalid H, Salpietro V, Weber KT. Idiopathic intracranial hypertension associated with either primary or secondary aldosteronism. Am J Med Sci 2013; 346 (3) 194-198
  • 28 Salpietro V, Mankad K, Kinali M , et al. Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study. J Pediatr Endocrinol Metab 2014; 27 (1-2) 107-115
  • 29 Salpietro V, Polizzi A, Bertè LF , et al. Idiopathic intracranial hypertension: a unifying neuroendocrine hypothesis through the adrenal-brain axis. Neuroendocrinol Lett 2012; 33 (6) 569-573
  • 30 Binder DK, Horton JC, Lawton MT, McDermott MW. Idiopathic intracranial hypertension. Neurosurgery 2004; 54 (3) 538-551 , discussion 551–552
  • 31 Chumas PD, Kulkarni AV, Drake JM, Hoffman HJ, Humphreys RP, Rutka JT. Lumboperitoneal shunting: a retrospective study in the pediatric population. Neurosurgery 1993; 32 (3) 376-383 , discussion 383
  • 32 Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology 1996; 46 (6) 1524-1530
  • 33 Thuente DD, Buckley EG. Pediatric optic nerve sheath decompression. Ophthalmology 2005; 112 (4) 724-727
  • 34 Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol 1997; 17 (2) 86-91