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presenting feature may be a nasal CSF leak, as pointed out by Clarke and colleagues Self-audible bruits have been reported by some of our patients; this has been attributed to turbulence created by differences in pressure between the cranial and jugular veins The patient is then discovered to have agrant papilledema (Figs 13-8 and 13-9), immediately raising the specter of a brain tumor (rarely, papilledema is only minimally developed or absent) The risk of visual loss and the severity of headache in many instances make the term benign intracranial hypertension less acceptable The CSF pressure is found to be elevated, usually in the range of 250 to 450 mmH2O, but it is not clear whether the brain itself is swollen or, as is more likely, the increased pressure is due entirely to a change in the pressure within the CSF and venous compartments When the CSF pressure is monitored for many hours, there are uctuations taking the form of irregularly occurring plateau waves of increased pressure lasting 20 to 30 min and then falling abruptly nearly to normal (Johnston and Paterson) Aside from papilledema, there is remarkably little to be found on neurologic examination perhaps a slight unilateral or bilateral abducens palsy, ne uninterpretable nystagmus on far lateral gaze,

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or a minor sensory change Visual eld testing usually shows slight peripheral constriction with enlargement of the blind spots As vision diminishes, more severe constriction of the elds, with greater nasal or inferior nasal loss, is found Exceptionally, particularly in children, an otherwise typical Bell s palsy may be associated (Chutorian et al) Mentation and alertness are preserved, and the patient seems surprisingly well aside from the headaches, which infrequently becomes severe enough to limit daily activity Examinations by CT and MRI have shown the ventricles to be normal in size or small The sella may be enlarged and lled with CSF ( empty sella, page 577) There is no MRI evidence of change in the water density of the brain As remarked above, most of the patients are overweight young women, often with menstrual irregularities, but the condition also occurs in children or adolescents, in whom there is no clear sex predominance, and in men (Digre and Corbett) We have had experience with several familial instances eg, affecting mother and daughter Practically all of the women with this disease are obese and so are the men, but to a lesser degree (Durcan et al) In obese women without the pseudotumor syndrome, CSF pressure usually does not differ from that of normal persons (Corbett and Mehta) All forms of endocrine and menstrual abnormalities (particularly amenorrhea) as well as the use of oral contraceptives have been postulated as causative factors, but none has been substantiated Pathophysiology The mechanism of increased CSF pressure in the above-described idiopathic form of the disorder has remained elusive, but recent experience is converging on the possibility that there is a functional obstruction to out ow in the venous sinuses Karahalios and colleagues and others have found the cerebral venous pressure to be consistently elevated in pseudotumor cerebri; in half of their patients, there was a venous out ow obstruction demonstrated by venography, often with a pressure gradient across the site These authors proposed that venous hypertension increases the resistance to CSF absorption and is the proximate mechanism underlying pseudotumor Similarly, Farb and colleagues, using sophisticated MRI venography, visualized venous stenosis in 27 of 29 patients with pseudotumor (and in 4 of 59 control subjects) In both the aforementioned studies and others like them, the nature of the obstruction was not clear, but the fact that in some series it was bilateral and focal suggests that the stenosis was not simply the passive result of raised intracranial pressure This issue is not yet resolved, as noted below A related nding in some cases, pointed out to us some time ago by Fishman, is one of partial obstruction of the lateral sinuses by enlarged pacchionian granulations (seen during the venous phase of conventional angiography) It is here that recent evidence has been most provocative The intervention by stenting of a venous sinus at the sites of apparent obstruction has resulted in clinical improvement and a reduction in CSF pressure For example, 5 of 12 patients treated by Higgins and colleagues became asymptomatic, but this was a population selected by the demonstration of a focal gradient in the lateral sinus during venography Karakalios had similar success in several patients What is not clear is how prevalent these partial venous obstructions are and what precisely is their nature (if not simply enlarged granulations) In some series, the latter abnormality has been found in 10 to 25 percent of patients who lack any features of pseudotumor (Leach et al) In an attempt to settle the issue of the venous stenosis as being a secondary effect of raised intracranial pressure, King and colleagues have measured intracranial venous pressure while withdrawing spinal uid from the cervical subarachnoid space in pa-.

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