J Pediatr Neurol 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
Department of Pediatrics, University of Messina, Messina, Italy
,
Anna Claudia Romeo
Department of Pediatrics, University of Messina, Messina, Italy
,
Rosa Morabito
Department of Radiology, University of Messina, Messina, Italy
,
Dominique De Vivo
Department of Pediatrics, University of Messina, Messina, Italy
,
Emanuele David
Department of Radiology, University of Messina, Messina, Italy
Department of Radiology, Anatomopathology and Oncology, Sapienza University of Rome, Italy
,
Daniela Impollonia
Department of Radiology, University of Messina, Messina, Italy
,
Giovanni Stroscio
Department of Radiology, University of Messina, Messina, Italy
,
Francesca Granata
Department of Radiology, University of Messina, Messina, Italy
,
Antonio Simone Laganà
Department of Pediatric, Gynecological, Microbiological and Biomedical Sciences, University of Messina, Messina, Italy
› Author Affiliations
Further Information

Publication History

22 October 2014

18 January 2015

Publication Date:
13 July 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.