Octreotide: A Therapeutic Option for Pediatric Pseudotumor Cerebri Syndrome?

Abstract

Pseudotumor cerebri syndrome (PTCS) is a condition of unclear etiology, characterized by increased intracranial pressure (ICP) with normal cerebrospinal fluid contents and no associated anomalies evident in the brain parenchyma. It may be associated with concomitant risk factors, including obesity, endocrine disorders, chronic medical illness, and medications. Clinical signs and symptoms of PTCS in children are heterogeneous, but they usually include headache, papilledema at the funduscopic examination, and visual disturbances (e.g., diplopia and/or vision loss). Irreversible blindness, due to persistently raised ICP, has been also reported in up to 10% of cases. Treatment goal of PTCS is the relief of symptoms and the preservation of visual function, by reducing ICP. Diuretics (e.g., acetazolamide, furosemide) could be regarded as the first-line treatments in PTCS; however, up to 30% of patients do not have a clinical response, making important the identification of alternative (conservative) treatment's choices to avoid the PTCS-related complications (e.g., permanent visual loss) and the necessity of invasive surgical (cerebrospinal fluid diverting) procedures. In this context, octreotide (i.e., a synthetic somatostatin analog) has been previously proposed in some studies as a potential (etiologically targeted) treatment able to revert to PTCS-related manifestations. We hereby discuss the possible role of octreotide in PTCS and hypothesize the possible mechanism of action of this drug in reducing ICP.

Keywords

idiopathic intracranial hypertension - pseudotumor cerebri - children - treatment - octreotide

• References

• 1 Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007; 52 (6) 597-617
• 2 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
• 3 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
• 4 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
• 5 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
• 6 Curry Jr WT, Butler WE, Barker II FG. Rapidly rising incidence of cerebrospinal fluid shunting procedures for idiopathic intracranial hypertension in the United States, 1988–2002. Neurosurgery 2005; 57 (1) 97-108 , discussion 97–108
• 7 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
• 8 Niotakis G, Grigoratos D, Chandler C, Morrison D, Lim M. CSF diversion in refractory idiopathic intracranial hypertension: single-centre experience and review of efficacy. Childs Nerv Syst 2013; 29 (2) 263-267
• 9 Antaraki A, Piadites G, Vergados J, Andreou A, Chlouverakis C. Octreotide in benign intracranial hypertension. Lancet 1993; 342 (8880) 1170
• 10 Panagopoulos GN, Deftereos SN, Tagaris GA , et al. Octreotide: a therapeutic option for idiopathic intracranial hypertension. Neurol Neurophysiol Neurosci 2007; 10: 1
• 11 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
• 12 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
• 13 Sun L, Coy DH. Somatostatin and its analogs. Curr Drug Targets 2014; ; In press
• 14 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
• 15 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
• 16 Malozowski S, Tanner LA, Wysowski D, Fleming GA. Growth hormone, insulin-like growth factor I, and benign intracranial hypertension. N Engl J Med 1993; 329 (9) 665-666
• 17 Reeves GD, Doyle DA. Growth hormone treatment and pseudotumor cerebri: coincidence or close relationship?. J Pediatr Endocrinol Metab 2002; 15 (Suppl. 02) 723-730
• 18 Rogers AH, Rogers GL, Bremer DL, McGregor ML. Pseudotumor cerebri in children receiving recombinant human growth hormone. Ophthalmology 1999; 106 (6) 1186-1189 , discussion 1189–1190
• 19 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
• 20 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
• 21 Lampit M, Nave T, Hochberg Z. Water and sodium retention during short-term administration of growth hormone to short normal children. Horm Res 1998; 50 (2) 83-88
• 22 Salpietro V, Ruggieri M. Pseudotumor cerebri pathophysiology: the likely role of aldosterone. Headache 2014; 54 (7) 1229
• 23 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