Abnormalities of cerebrospinal fluid (CSF) pressure are relatively common and may lead to a variety of symptoms, with
headache usually being the most prominent one. The clinical presentation of alterations in CSF pressure may vary significantly and show a striking similitude to several primary
headache syndromes. While an increase in CSF pressure may be of primary or secondary origin, a pathologic decrease of CSF pressure is usually the result of a meningeal
rupture with a resulting leakage of CSF. The pathophysiologic mechanisms of
idiopathic intracranial hypertension (IIH) remain largely unknown. However recent evidence indicates that an abnormality in CSF outflow and absorption is likely to play a significant role. Treatment usually consists of a combination of
weight loss and a pharmacologic approach using
carbonic anhydrase inhibitors. Recent results of the first randomized, double-blind, placebo-controlled trial (RCT) with
acetazolamide proved its efficacy in reducing
headache and visual disturbances. Clinical evidence suggests efficacy for
topiramate and
furosemide but no RCT has been conducted to date to confirm these results. In contrast to IIH,
spontaneous intracranial hypotension frequently remits spontaneously without specific treatment. If necessary, treatment options range from conservative methods to epidural blood or
fibrin sealant patches and surgical interventions.