From the Guidelines
Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a condition characterized by increased pressure inside the skull without an identifiable cause such as a tumor or infection. It occurs most commonly in overweight women of childbearing age [ 1 ]. The primary symptoms include:
- Severe headaches
- Vision problems (particularly blurred or double vision)
- Pulsatile tinnitus (whooshing sound in the ears) Other symptoms may include transient visual obscurations, back pain, dizziness, neck pain, visual blurring, cognitive disturbances, radicular pain, and typically horizontal diplopia [ 1 ].
Management
Treatment typically begins with weight loss for overweight patients, as even modest weight reduction can significantly improve symptoms. Management of headache should be the same as typical IIH, and all patients should be managed as typical IIH and counselled about weight management [ 1 ]. Medications are often prescribed, with acetazolamide (Diamox) being a common treatment option. For severe cases or when vision is threatened, procedures such as lumbar punctures to remove excess fluid or surgical interventions like optic nerve sheath fenestration or cerebrospinal fluid shunting may be necessary. Regular monitoring of visual fields and eye examinations are essential as untreated pseudotumor cerebri can lead to permanent vision loss [ 1 ].
Key Principles
The management of IIH is based on three main principles:
- To treat the underlying disease
- To protect the vision
- To minimise the headache morbidity [ 1 ].
Ongoing Management
The condition can resolve with treatment but may recur, requiring ongoing management. It is essential to work with a multidisciplinary team to ensure the best possible outcomes for patients with pseudotumor cerebri [ 1 ].
From the Research
Definition and Characteristics
- Pseudotumor 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, 3, 4.
- It is a diagnosis of exclusion, and other causes of increased intracranial pressure must be sought with history, imaging, and cerebrospinal fluid examination before the diagnosis can be made 2.
- The condition is characterized by increased intracranial pressure without clinical, laboratory, or radiologic evidence of an intracranial space-occupying lesion, meningeal inflammation, or venous outflow obstruction 5, 6.
Symptoms and Signs
- Patients with IIH present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis 3.
- The most common presentation is isolated headache, and many patients are asymptomatic 5.
- Obesity is a common feature, with 87.8% of patients evaluated for body dimensions being described as obese 5.
Treatment and Management
- The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision 2.
- Treatment approaches 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.
- Early recognition and medical or surgical treatment of the disease can prevent permanent visual impairment 6.