However, the time course of the orthostatic aggravation may vary substantially and with increasing disease duration may even disappear completely. The clinical picture is dominated by an orthostatic headache which develops in temporal relation to a decrease in intracranial pressure. The leaks are commonly localized in the cervicothoracic junction or along the thoracic spine. In contrast to a chronic elevation in intracranial pressure which may be primary (idiopathic intracranial hypertension) or secondary, spontaneous intracranial hypotension is in almost all cases secondary to a meningeal rupture with a resulting leak of cerebrospinal fluid. Invasive treatments should only be considered in exceptional therapy-resistant cases as long-term data regarding the safety and long-term benefit of these procedures is scarce. Treatment usually consists of a combination of weight reduction and a pharmacological treatment with carbonic anhydrase inhibitors such as acetazolamide and topiramate. Given the severity and potential irreversibility of these symptoms, a quick and accurate diagnosis as well as an early initiation of treatment is mandatory. In addition patients may suffer from cranial nerve palsies, cognitive deficits, a pulsatile tinnitus and olfactory deficits adding to the significant loss in quality of life. In addition to the headache patients commonly suffer from a papilledema that leads to a progressive visual deficit which, if untreated, may results in a complete and irreversible visual loss. However, an aggravation upon physical exercise, coughing and sneezing as well as nausea and photophobia may occur.

Patients suffer from an unspecific headache, which in most cases presents as a daily and bilateral headache without accompanying symptoms. The clinical syndrome of idiopathic intracranial hypertension results from an increase in intracranial pressure without an identifiable cause.

As in these disorders headache is frequently unspecific, highly variable in its clinical presentation and may occasionally even mimic primary headaches including migraine, diagnosis may in some cases be challenging. Headache is one of the most prominent symptoms during a change in intracranial pressure.

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