Comprehensive Review of Pseudotumor Cerebri: Neurological and Ophthalmological Perspectives

• DOI: https://doi.org/10.22533/at.ed.1594552414065

• Palavras-chave: Idiopathic Intracranial Hypertension; Pseudotumor Cerebri; Papilledema; Intracranial Pressure; Neuro-Ophthalmology.

• Keywords: Idiopathic Intracranial Hypertension; Pseudotumor Cerebri; Papilledema; Intracranial Pressure; Neuro-Ophthalmology.

• Abstract: INTRODUCTION Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is characterized by elevated intracranial pressure without an identifiable mass. The condition primarily affects obese women of childbearing age, with symptoms mimicking those of brain tumors, including headaches and visual disturbances. Despite extensive research, the exact etiology remains unclear, although theories suggest dysregulation of cerebrospinal fluid dynamics, hormonal changes, and venous outflow obstruction. The clinical presentation includes severe headaches, papilledema, and visual field defects, necessitating early diagnosis and intervention to prevent permanent vision loss. OBJETIVE To provide a comprehensive review of the pathophysiology, diagnosis, management, highlight current treatment strategies, including both pharmacological and surgical interventions of pseudotumor cerebri. METHODS This is a narrative review which included studies in the MEDLINE – PubMed (National Library of Medicine, National Institutes of Health), COCHRANE, EMBASE and Google Scholar databases, using as descriptors: “Idiopathic Intracranial Hypertension” AND “Pseudotumor Cerebri” AND Papilledema” AND “Cerebrospinal Fluid Dynamics” AND “Neuro-Ophthalmology” in the last years. RESULTS AND DISCUSSION Recent epidemiological studies show an increasing incidence of IIH, closely linked to rising obesity rates. The pathophysiology involves complex interactions of CSF dynamics, hormonal influences, and possibly genetic factors. Neurological and ophthalmological symptoms are significant, with papilledema being a critical feature that can lead to irreversible visual impairment if untreated. Diagnostic tools include MRI, CT, and lumbar puncture, with MRI often revealing an empty sella and transverse venous sinus stenosis. Treatment focuses on reducing intracranial pressure through weight management, pharmacological interventions like acetazolamide, and surgical options such as optic nerve sheath fenestration and CSF shunting. CONCLUSION Idiopathic intracranial hypertension poses significant challenges due to its potential for causing permanent vision loss and its strong association with obesity. Effective management requires a multidisciplinary approach, including weight reduction, pharmacological therapy, and surgical interventions when necessary. Advances in neuroimaging and a better understanding of the condition’s pathophysiology are essential for improving diagnostic accuracy and treatment outcomes. Future research should focus on identifying genetic and molecular mechanisms, developing targeted therapies, and exploring the impact of lifestyle modifications on disease progression.