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Several diseases that are associated with raised CSF protein concentration have given rise to a pseudotumor syndrome, the more common ones being the Guillain-Barre syndrome, systemic lupus, and spinal tumors, particularly oligodendroglioma Elevated spinal uid pressure has been attributed to a blockage of CSF absorption by the proteinaceous uid, but this mechanism has never been validated and fails to explain those few instances in which the Guillain-Barre and pseudotumor syndromes have been associated with a near normal protein content of the CSF This explanation is even less compelling if one recalls that the protein concentration of the uid in the cerebral spaces is considerably lower than in the spinal ones Also, as we have pointed out, when calculated correctly, neither the resistance to CSF absorption nor the colloid osmotic effect attributable to an increased protein content in the spinal uid is adequate to explain the pressure elevation (Ropper and Marmarou) The mechanism of this type of pseudotumor syndrome is presently unknown In addition to mechanical factors, a number of toxic and metabolic disturbances may give rise to a pseudotumor syndrome In children, as chronic corticosteroid therapy is withdrawn, there may be a period of headache, papilledema, and elevated ICP with little or no enlargement of the lateral ventricles Lead toxicity in children may be marked by brain swelling and papilledema Excessive doses of tetracycline and vitamin A (particularly in the form of isotretinoin, an oral vitamin A derivative used in the treatment of severe acne) have also been shown to cause intracranial hypertension in children and adolescents Ingestion of large quantities of animal (bear) liver is another curious source of intoxication with vitamin A underlying pseudotumor Isolated instances of hypo- or hyperadrenalism, myxedema, and hypoparathyroidism have been associated with increased CSF pressure and papilledema, and occasionally the administration of estrogens, phenothiazines, the antiarrhythmic drug amiodarone, and quinolone antibiotics has the same effect, for reasons not known The rst step in differential diagnosis is to exclude an underlying tumor or the nontumorous causes of raised ICP, mainly dural venous occlusion and meningeal in ammation This can be accomplished by CT and MRI, although it should be borne in mind that certain chronic meningeal reactions (eg, those due to sarcoidosis or to tuberculous or carcinomatous meningitis), which give rise to headache and papilledema, may sometimes elude detection by these imaging procedures In these cases, however, lumbar puncture will disclose the diagnosis It should not need to be emphasized that the diagnosis of idiopathic pseudotumor cerebri should not be accepted when the CSF content is abnormal Visual Loss in Pseudotumor Careful evaluations of the visual elds and of acuity is required soon after the diagnosis of idiopathic pseudotumor is established Repeated examination of visual function, preferably in collaboration with an ophthalmologist, is essential in detecting early and potentially reversible visual loss However, it must be acknowledged that measurements of visual acuity (and of confrontation elds and visual evoked potentials) are relatively insensitive means of detecting early visual eld loss and that abnormalities in these tests indicate that serious damage to the optic nerve head has already occurred Quantitative perimetry, using the kinetic Goldmann technique, has been more informative The neurologist caring for a patient with pseudotumor is probably best advised to measure visual acuity serially in the of ce and refer the patient for computed perimetry as an adjunctive test Fundus photographs are also a reliable means of assessing the course of papilledema A reduction in previously normal acuity to less than.

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20/100, an enlargement of the blind spot, or the appearance of sector eld defects is an indication for prompt and aggressive treatment If intracranial hypertension and papilledema are left untreated or fail to respond to the measures outlined above, there is danger of permanent visual loss from compressive damage to the optic nerve bers and compression of the central retinal veins Corbett and associates, who described a group of 57 patients followed for 5 to 41 years, found severe visual impairment in 14, and Wall and George, using highly re ned perimetric methods, have reported an even higher incidence of visual loss Moreover, children with pseudotumor share the same visual risks as adults (Lessell and Rosman) Sometimes vision is lost abruptly, either without warning or following one or more episodes of monocular or binocular visual obscurations Treatment Most patients with idiopathic intracranial hypertension will be found initially to have minor visual changes aside from the papilledema; the headache and lumbar puncture pressure then guide treatment Besides symptomatic relief of headaches, the progression of visual loss mentioned above is the main concern At least one-quarter of our patients have recovered within 6 months after only repeated lumbar punctures and drainage of suf cient CSF to maintain the pressure at normal or near-normal levels (less than 200 mmH2O) The lumbar punctures were performed daily or on alternate days at rst and then at longer intervals, according to the level of pressure Evidently this was suf cient to restore the balance between CSF formation and absorption for at least several months At the same time, weight loss has been encouraged and the best results have been reported when this was successful Weight Reduction This is always advised but is dif cult to accomplish In two pathologically obese patients, we have resorted to surgical gastric plication, which had a bene cial effect on the pseudotumor but left the patient for a time with the severe gastrointestinal disturbances that commonly complicate this procedure Sugerman et al (1999) studied 24 morbidly obese women who had similar operations and found the results to be satisfactory over several years Two of our patients developed a sensory polyneuropathy after surgery The use of gastric plication and related procedures is currently undergoing re-evaluation for obesity in general; but in the dire circumstances of psuedotumor with visual loss, it is probably a reasonable alternative Lumbar-Peritoneal Shunting In patients who are unresponsive to the usual therapeutic measures, one treatment method that may be considered is a lumbar-peritoneal shunt Only a few of our patients have undergone this surgical procedure It has been relatively safe and effective, but because of a tendency for the shunt to become obstructed or to be dislodged in obese patients, sometimes causing back or sciatic pain, the procedure has fallen out of favor Burgett et al, who treated 30 patients in this way, reported success in reducing headache in almost all and in improving vision in 70 percent Despite its shortcomings, this procedure may be preferable to the optic nerve fenestration described below We have not had to resort to cranial subtemporal decompression, a procedure that was formerly used when vision was threatened Corticosteroids Prednisone (40 to 60 mg/day), oral hyperosmotic agents such as glycerol (15 to 60 mg four to six times daily), or carbonic anhydrase inhibitors (acetazolamide 500 mg twice or thrice daily; furosemide 20 to 80 mg twice daily) to reduce CSF formation all have their advocates We have occasionally observed a gradual recession of papilledema and a lowering of CSF pressure.

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