The syndrome we have termed the pseudotumor cerebri syndrome (PTCS) was first characterized as a distinct clinical entity in the papers by Quincke and Nonne, published a little over a hundred years ago. The condition has subsequently received a somewhat bewildering variety of names and its mechanism has also remained controversial. Moreover, it is probably not as rare as was originally thought. In addition, the insights gained by the study of its pathophysiology and management undoubtedly have more general implications for our understanding of intracranial dynamics in other conditions. For these reasons, and because there has been a considerable proliferation of literature on the subject in recent decades, we thought it would be timely to bring together the later observations with the extensive older literature.

The original intention was to have this book ready for publication in 2004 to mark the centenary of Nonne's paper which introduced the name ‘pseudotumor cerebri’ for a condition sporadically described during the four decades prior to that paper. Like many such endeavours, the present work took rather longer to complete than initially anticipated. Nonetheless, the belated acknowledgement of Nonne's paper does signify one of the central arguments of this monograph – that the name he proposed for the condition, a name that has endured despite many challenges, should be retained. The only proposed modification is the addition of the term ‘syndrome’ to embrace the collection of conditions which, in practice, share a common presentation, clinical picture, treatment strategy, and outcome, as well as, it is argued, a common mechanism.