Journal of Pediatric Neurology 2015; 13(01): 022-025
DOI: 10.1055/s-0035-1555148
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Remission of Pseudotumor Cerebri Syndrome after Initiating Pregnancy and Menarche: The Puzzling Role of Female Sex Hormones

Valeria Dipasquale
1   Department of Pediatrics, University of Messina, Messina, Italy
,
Anna Claudia Romeo
1   Department of Pediatrics, University of Messina, Messina, Italy
,
Rosa Morabito
2   Department of Radiology, University of Messina, Messina, Italy
,
Dominique De Vivo
1   Department of Pediatrics, University of Messina, Messina, Italy
,
Emanuele David
2   Department of Radiology, University of Messina, Messina, Italy
3   Department of Radiology, Anatomopathology and Oncology, Sapienza University of Rome, Italy
,
Daniela Impollonia
2   Department of Radiology, University of Messina, Messina, Italy
,
Giovanni Stroscio
2   Department of Radiology, University of Messina, Messina, Italy
,
Francesca Granata
2   Department of Radiology, University of Messina, Messina, Italy
,
Antonio Simone Laganà
4   Department of Pediatric, Gynecological, Microbiological and Biomedical Sciences, University of Messina, Messina, Italy
› Institutsangaben
Weitere Informationen

Publikationsverlauf

22. Oktober 2014

18. Januar 2015

Publikationsdatum:
13. Juli 2015 (online)

Abstract

Pseudotumor cerebri syndrome (PTCS) is characterized by increased intracranial pressure with normal cerebrospinal fluid components and no detectable anomalies of the brain parenchyma on magnetic resonance imaging. Presenting signs and symptoms are heterogeneous, but they commonly include headache, visual disturbances (i.e., vision loss and/or double vision), and papilledema. The etiology is uncertain, but several underlying risk factors associated with PTCS have been identified, including obesity, endocrine abnormalities (e.g., hyperaldosteronism, Cushing syndrome, hyperandrogenism, and Addison disease), medications (e.g., tetracycline, recombinant growth hormone therapy), and viral infections (e.g., chickenpox). Patients with postpubertal PTCS are more typically females in their reproductive age, thereby making crucial (although not fully understood) the role of female hormones in the pathophysiology of this condition. We describe two female patients with PTCS who were followed up at our institution. They experienced prompt (and full) remission of headache and visual disturbances during the first trimester of pregnancy and within the first 2 months after the onset of menarche (i.e., at 20 and 11 years of age), respectively. We reviewed the literature searching for similar cases and hypothesized on the pathophysiologic (and still unclear) involvement of female sex hormones in regulating intracranial pressure, making some patients prone to 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 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 (6) 561-568
  • 4 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
  • 5 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
  • 6 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
  • 7 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
  • 8 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
  • 9 Andrews LE, Liu GT, Ko MW. Idiopathic intracranial hypertension and obesity. Horm Res Paediatr 2014; 81 (4) 217-225
  • 10 Salpietro V, Ruggieri M. Pseudotumor cerebri pathophysiology: the likely role of aldosterone. Headache 2014; 54 (7) 1229
  • 11 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
  • 12 Shehata HA, Okosun H. Neurological disorders in pregnancy. Curr Opin Obstet Gynecol 2004; 16 (2) 117-122
  • 13 Sheldon CA, Kwon YJ, Liu GT, McCormack SE. An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics. Pediatr Res 2015; 77 (2) 282-289
  • 14 Johnston I. The historical development of the pseudotumor concept. Neurosurg Focus 2001; 11 (2) E2
  • 15 Tessler Z, Biedner B, Yassur Y. Benign intracranial hypertension in menarche. Compr Ther 1985; 11 (5) 16-18