What is Pseudotumor Cerebri? Diagnosis and Treatment

Pseudotumor cerebri is a not very rare condition that can be overlooked among the causes of headache. It is also called idiopathic intracranial hypertension. It is seen with a frequency of 2-3 in a hundred thousand people. It is more common in obese women between the ages of 20-45. The incidence only among obese women rises to 20 per hundred thousand. The risk of occurrence increases during pregnancy. Although the exact cause is not known, there may be a defect in the reabsorption of cerebrospinal fluid (CSF). Drugs such as tetracycline, vitamin A, corticosteroids, nalidixic acid may cause pseudotumor cerebri. Anemia, chronic respiratory failure, hormonal disorders are other risk factors. However, in most patients, pseudotumor cerebri may exist without any cause being detected.

Signs and Symptoms

More than 90% of patients with pseudotumor cerebri complain of headache. Pain may increase with straining (val salva maneuver). Symptoms such as dizziness, visual disturbances such as double vision, tinnitus, photophobia, eye pain, nausea and vomiting may also accompany. Such different symptoms can make diagnosis difficult. In pseudotumor cerebri, papilledema finding is detected in fundus examination. The severity of papilledema can be very variable. The blind spot in the visual field may enlarge, there may be loss of visual field in the lower part of the nose, and it may even lead to blindness if not treated.

Diagnosis

By definition, four criteria must be present to diagnose pseudotumor cerebri: (1) signs and symptoms indicating increased intracranial pressure, including papilledema; (2) normal brain tomography or magnetic resonance imaging; (3) demonstration of increased CSF pressure with lumbar puncture (removal of water from the waist), and (4) normal CSF biochemistry, culture, and cytology results. Immediate CT and MRI should be performed in all patients with suspected increased intracranial pressure to rule out intracranial mass, infection, and other possible causes. Imaging results in patients with pseudotumor cerebri are normal except that the ventricles are small or of normal size. After excluding space-occupying lesion and ventricular enlargement with imaging, lumbar puncture can be performed safely, CSF pressure is measured, biochemistry, Samples are taken for culture and cytology. Optical coherence tomography (OTC) may also be useful in monitoring the effect of interventions to reduce CSF pressure.

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Differential diagnosis

If a patient has a specific cause of intracranial hypertension, it is not idiopathic by definition, but secondary type intracranial hypertension. Therefore, the absence of secondary causes must be proven before making the diagnosis of idiopathic. These include cerebral hemorrhage, intracranial tumors , abnormalities such as Arnold-Chiari malformation, cerebral venous sinus thrombosis, ventricular system abnormalities, liver failure, intracranial infections. Missing a treatable cause of increased intracranial pressure can result in an increased risk of death and disability.

Treatment

Oral acetazolamide is the first-line treatment for patients who meet the criteria for pseudotumor cerebri. If not tolerated, administration of furosemide or chlorthalidone may also be considered. If there is no response to diuretic treatment, short-term systemic corticosteroids can be used. Octreotide, a synthetic somatostatin analog, may also be effective in treatment. Shunt surgery can be performed if drug therapy is insufficient. If papilledema does not resolve, decompression can be applied to the optic nerve sheath. Weight loss accelerates the improvement of complaints. Bariatric surgery may be recommended for this.

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