From the Guidelines
Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), can affect individuals of any age, with a characteristic demographic pattern that most commonly affects women of childbearing age, typically between 20 and 50 years old, and shows a strong predilection for obese women. The condition's age distribution reflects its association with hormonal factors and weight, which explains why reproductive-age women are disproportionately affected 1.
Demographic Patterns
- Pseudotumor cerebri can also occur in children, adolescents, men, and older adults, with less gender bias in the pediatric population, where boys and girls are affected more equally 1.
- In the pediatric population, the condition is typically seen in overweight girls in postpubertal children, but in prepubertal children, boys and girls are equally affected 1.
- Secondary causes of pseudotumor cerebri should be thoroughly investigated in men or non-obese individuals, as these cases are more likely to be associated with medications, underlying medical conditions, or venous sinus thrombosis 1.
Diagnostic Considerations
- Diagnostic criteria for pseudotumor cerebri syndrome include papilledema, normal neurological examination, normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure 1.
- In the absence of papilledema and sixth nerve palsy, diagnosis of pseudotumor cerebri can be suggested on neuroimaging based on findings including empty sella, flattening of the posterior aspect of the globes, distention of the perioptic subarachnoid space, and transverse sinus stenosis 1.
- MRI of the brain with and without contrast is the imaging modality of choice for detecting secondary signs of increased intracranial pressure, such as an empty sella, dilated optic sheaths, tortuous or enhancing optic nerves, and flattening of the posterior aspects of the globes 1.
From the Research
Pseudotumor Ages
- The age of patients with orbital pseudotumor can vary, with a mean age of 43.75 years reported in one study 2.
- The age range of patients with orbital pseudotumor is not explicitly stated in the provided studies, but it can be inferred that the condition can occur in adults, as most of the studies reported a mean age in the range of 40-50 years.
- There is no specific information available on the age of onset of orbital pseudotumor, but it is likely that the condition can occur at any age, given the variability in patient demographics reported in the studies.
Demographic Characteristics
- Orbital pseudotumor can occur in both males and females, with a roughly equal distribution reported in one study 2.
- The condition can affect one or both eyes, with unilateral disease reported in 73.5% of patients in one study 2.
- The clinical features of orbital pseudotumor can vary, but common presenting signs include proptosis, ocular motor deficit, pain, lid swelling or lid mass, ptosis, and chemosis 2, 3.
Treatment Outcomes
- The treatment of orbital pseudotumor typically involves systemic corticosteroids, which can be effective in improving symptoms and reducing inflammation 4, 2, 3.
- The response to corticosteroid treatment can vary, with some patients experiencing a complete response and others requiring additional treatment, such as radiotherapy 5.
- Radiotherapy can be an effective treatment for orbital pseudotumor, particularly in patients who do not respond to corticosteroids or who experience recurrence 5.