THE CLINICAL SYNDROME

• Pseudotumor cerebri is a rather common cause of headaches, despite the fact that its diagnosis is frequently overlooked.

  • It occurs in nearly the same number of individuals, 2.2 out of every 100,000, making it comparable to cluster headache in terms of its occurrence.

• Pseudotumor cerebri, which is also known as idiopathic intracranial hypertension, is most commonly diagnosed in obese women between the ages of 20 and 45.

  • When only obese women are considered in epidemiological research, the incidence rises to roughly 20 instances per 100,000 patients.

  • In addition, there is a correlation between pregnancy and an increased risk of developing pseudotumor cerebri. It has not been determined what exactly causes pseudotumor cerebri, but a problem with the reabsorption of cerebral fluid seems to be a common denominator among those who have the condition (CSF).

• Ingestion of a variety of drugs, such as tetracycline, vitamin A, corticosteroids, or nalidixic acid, is one of the risk factors that can lead to this condition.

  • In addition to these, chronic respiratory insufficiency, blood dyscrasias, anemias, endocrinopathies, and other conditions can also play a role.

  • However, the precise cause of pseudotumor cerebri in a significant number of cases continues to be unknown.

SIGNS AND SYMPTOMS

• Over ninety percent of patients with pseudotumor cerebri present with the complaint of headache, are female, and have headaches that get worse when they perform the vasovagal maneuver.

• It is possible for associated nonspecific signs and symptoms of the central nervous system, such as dizziness, visual disturbances including diplopia, tinnitus, photophobia, nausea and vomiting, and ocular pain, to obscure what should otherwise be a reasonably straightforward diagnosis.

• This is the case despite the fact that virtually all patients suffering from pseudotumor cerebri (1) have papilledema on fundoscopic examination, (2) are female, and (3) are obese.

• The severity of papilledema varies from patient to patient, and it is sometimes coupled with modest defects in the patient's vision field, such as an expanded blind spot or problems in the nose region of the inferior visual field.

• In the absence of treatment for the illness, blindness is a potential outcome.

TESTING

• It is conventionally accepted that a diagnosis of pseudotumor cerebri can be made when the following four criteria are met: (1) signs and symptoms, such as papilledema, that are suggestive of increased intracranial pressure; (2) normal results of magnetic resonance imaging (MRI) or computed tomography (CT) of the brain; (3) increased CSF pressure documented by lumbar puncture; and (4) normal CSF chemistry, cultures, and cytology.

  • These criteria are listed in this order. To rule out intracranial mass and infection, among other conditions, an urgent MRI and CT scan of the patient's brain should be performed in conjunction with contrast medium.

  • This should be done on all patients who have a suspicion of having high intracranial pressure.

• Neuroimaging reveals that patients suffering from pseudotumor cerebri have ventricles that are tiny to normal in size, but the scan is otherwise normal.

• It is safe to proceed with a lumbar puncture to monitor the pressure of the cerebrospinal fluid (CSF) and obtain fluid for chemistry, cultures, and cytology once it has been determined through neuroimaging that there are no space-occupying lesions in the dilated ventricles.

• The use of optical coherence tomography (OTC) in the monitoring of the effectiveness of therapies designed to reduce CSF pressure is another potential application of this imaging technique.

DIFFERENTIAL DIAGNOSIS

• If there is a known reason for a patient's intracranial hypertension, then that patient's condition is no longer considered idiopathic but rather falls under the category of a particular secondary form of intracranial hypertension.

• There are a number of potential causes of secondary intracranial hypertension that need to be taken into consideration before a patient is labeled as having idiopathic intracranial hypertension.

TREATMENT

• Patients who display all four of the necessary criteria for the diagnosis of pseudotumor cerebri should have their therapy started with oral acetazolamide, as this is a reasonable first step in the treatment process.

  • In cases where the drug is not well tolerated, furosemide or chlorthalidone can be a better option.

• If the patient does not react to treatment with diuretics, another treatment option is to administer a brief course of systemic corticosteroids such as dexamethasone.

  • In some cases, the elimination of symptoms caused by pseudotumor cerebri can be sped up by using acetazolamide in conjunction with the implementation of weight loss techniques.

  • In the treatment of treatment-resistant pseudotumor cerebri, preliminary clinical findings show that octreotide, a synthetic somatostatin analogue, may be useful.

• Neurosurgical techniques, such as CSF shunt operations, are a viable next step for treating instances that are resistant to medical treatment.

  • Both bariatric surgery and decompression techniques on the optic nerve sheath have been suggested as possible treatments in the event that papilledema continues to worsen.

COMPLICATIONS AND PITFALLS

• As was stated previously, if a case of pseudotumor cerebri goes untreated, it can lead to irreversible vision loss as well as considerable morbidity.

• In addition, a failure to effectively diagnose and treat the secondary causes of increasing intracranial hypertension can lead to terrible outcomes for the patient, including the possibility of death that could have been avoided.