Update on Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

In 1904 the German neurologist Max Nonne introduced the term pseudotumor cerebri (PTC) to describe the clinical syndrome of chronically elevated intracranial pressure of unknown etiology. As diagnostic options grew broader and more effective over time, however, numerous pathological conditions that can cause chronically high intracranial pressure were identified, which occasioned its classification into a primary and secondary form. For years the term pseudotumor cerebri has been used as an umbrella term for chronic intracranial hypertension without further specifying its underlying cause. Pseudotumor cerebri has a primary form, idiopathic intracranial hypertension (IIH), and various secondary forms that can co-occur with other diseases, such as cerebral venous sinus thrombosis or hormonal changes[1, 2]. The following review is limited to the primary form of PTC. The prevalence of IIH is 8.6 cases per 100 000 in the general population [3, 4], although the number is probably much higher considering that existing epidemiological studies are several decades old and apply different diagnostic criteria. Moreover, because the clinical presentation of IIH can be so similar to other primary headache disorders such as migraine or tension-type headache, especially their chronic forms, the disease may be underdiagnosed [5, 6]. Furthermore, most persons suffering from IIH are overweight [7, 8]; against the backdrop of increasing worldwide obesity, the Update on Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)