What is the diagnosis and treatment for Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)?

From the Guidelines

Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), should be managed with weight loss for overweight patients and first-line medication therapy including acetazolamide (Diamox) or topiramate (25-50 mg twice daily), with regular ophthalmologic monitoring to prevent permanent vision loss from optic nerve damage. The condition is characterized by increased pressure around the brain without a tumor or other identifiable cause, and treatment should focus on reducing symptoms and preventing complications. According to the most recent guidelines, patients with IIH should be counselled about weight management and headache management should be the same as typical IIH 1. Surgical management to control elevated intracranial pressures should not routinely be considered unless advised by experienced clinicians within the multidisciplinary team setting 1. Some studies suggest that impaired cerebrospinal fluid absorption, possibly related to increased venous pressure or hormonal factors, may contribute to the development of IIH 1. Key symptoms of IIH include headaches, pulsatile tinnitus, transient visual obscurations, and papilledema on examination, and the condition predominantly affects overweight women of childbearing age. Regular ophthalmologic monitoring is essential to prevent permanent vision loss from optic nerve damage, and medication-resistant cases may require surgical interventions like optic nerve sheath fenestration or cerebrospinal fluid shunting procedures. The 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 summary, the management of pseudotumor cerebri should prioritize weight loss, medication therapy, and regular ophthalmologic monitoring to improve symptoms and prevent complications. Some key points to consider in the management of IIH include:

• Weight loss for overweight patients
• First-line medication therapy with acetazolamide (Diamox) or topiramate (25-50 mg twice daily)
• Regular ophthalmologic monitoring to prevent permanent vision loss from optic nerve damage
• Surgical management should not be routinely considered unless advised by experienced clinicians
• Impaired cerebrospinal fluid absorption may contribute to the development of IIH
• Key symptoms of IIH include headaches, pulsatile tinnitus, transient visual obscurations, and papilledema on examination.

From the Research

Definition and Symptoms of Pseudo Rumor Cerebri

• Pseudo rumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a syndrome of elevated intracranial pressure of unknown cause that occurs predominantly in obese women of childbearing age 2.
• The condition is characterized by symptoms and signs of increased intracranial pressure, including papilledema, daily headache, pulse-synchronous tinnitus, transient visual obscurations, and diplopia from sixth nerve paresis 3.

Diagnosis and Treatment

• IIH is a diagnosis of exclusion, and other causes of increased intracranial pressure must be ruled out with history, imaging, and cerebrospinal fluid examination before the diagnosis can be made 2, 4.
• The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision 2.
• Treatment options include weight management, low-salt diet, medical treatment with acetazolamide, and surgical interventions such as optic nerve sheath fenestration or cerebrospinal fluid shunting 2, 3.
• Topiramate has also been shown to be effective in the treatment of IIH, with prominent weight loss recorded in patients taking this medication 5.

Prognosis and Complications

• If left untreated, papilledema can cause progressive irreversible visual loss and optic atrophy 2.
• The main morbidity of IIH is visual loss, which is present in most patients and can usually be reversed if recognized early in the course of the disease and treated 3.
• The Idiopathic Intracranial Hypertension Treatment Trial has provided valuable insights into the efficacy of acetazolamide with weight loss in the treatment of IIH 6.