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in response to each of these measures, but such responses were not consistent or sustained and it was always dif cult to decide whether they represented the effect of treatment or the natural course of the disease Greer, who has reported on 110 patients, 11 of whom were treated with these agents, decided that they were of no value Moreover, in patients whose papilledema seems to recede under the in uence of corticosteroids, there is always a danger that it will recur when the drug is tapered Considering the potential for undesirable side effects of corticosteroids, we have used them very sparingly and only for brief periods while preparing the patient for more de nitive treatment Many authorites eschew their use entirely and objected strenuously to their relative endorsement in previous editions of this book Fenestration of the Optic Nerve Sheath For patients who are progressively or rapidly losing vision, a procedure that was in past years favored by some ophthalmologists is unilateral fenestration of the optic nerve sheath According to Corbett and colleagues, this procedure which consists of partial unroo ng of the orbit and intraorbital incision of the dural-arachnoid sheaths surrounding the optic nerve effectively preserved or restored vision in 80 to 90 percent of patients Even when this procedure is performed on only one side, its effect on vision is often bilateral, and about two-thirds of patients have some relief of headache as well, albeit this has been transient in most of our patients However, the operation carries a moderately high risk of vascular obstruction and unilateral visual loss, as happened in two of our patients The causes of visual loss after this operation are mainly vascular and have included central retinal artery or vein occlusion, choroidal infarction, optic nerve trauma, hemorrhage into the nerve sheath, and infection Over the past decade, enthusiasm for the procedure has diminished after reports of several series where there was visual loss in 2 to 11 percent of patients Some of these data come from studies of sheath fenestration for ischemic optic neuropathy, a condition not comparable to the disc swelling of pseudotumor Perhaps more compelling are follow-up studies indicating that the reduction in intracranial pressure was limited to a year or less As a result of recent re-examination of the complications of this procedure, most of our colleagues recommend a lumboperitoneal shunt as the rst approach, even while acknowledging that half such shunts become occluded or displaced over time and then require replacement In a large number of patients, the CSF pressure remains elevated and the papilledema becomes chronic (Fig 13-10) It is the management of this group that is most dif cult and controversial At the moment, the clinician is left with little clear direction as to the course of treatment of pseudotumor cerebri In cases with no visual impairment and with tolerable headaches, we favor aggressive weight reduction and repeated lumbar punctures For more severe cases, we have advised lumbar drainage to the peritoneum and administration of acetazolamide, or if vision is threatened, optic sheath fenestration by an experienced surgeon We have also been impressed with the persistence of a complex migraine- or tension-like headache in some patients whose CSF pressure has been adequately reduced by repeated lumbar punctures, shunting, or optic nerve fenestration After it has been con rmed that the pressure is not elevated, these headaches may be treated in a manner similar to the usual types of chronic headaches, as outlined in Chap 10 Therapeutic lumbar puncture in pseudotumor may be followed transiently by the type of low-pressure headache described further on; it generally does not warrant treatment in these circumstances
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Pneumocephalus and Pneumocranium These disorders, in which air enters the ventricular system or the subarachnoid spaces, are discussed in relation to cranial injury and the postoperative state (page 749 and Fig 35-3) In the case of pneumocranium, the collection of air may act as a mass that compresses adjacent brain tissue and requires relief by aspiration Ventricular Dilatation without Raised Pressure or Brain Atrophy The wide application of CT and MRI reveals cases of lateral and third ventricular enlargement in which there is no evidence of parenchymal lesions of the cerebrum Usually it is slight in degree and represents hydrocephalus ex vacuo This has been reported in patients with anorexia nervosa and Cushing disease and in those receiving corticosteroids for a long period of time It has also been observed in children with protein malnutrition and in some schizophrenics and chronic alcoholics In all of the aforementioned conditions, slight widening of the cerebral sulci accompanies the ventricular enlargement In alcoholic patients, there is usually no associated irreversible neurologic or mental abnormality After a prolonged period without steroids or of abstinence from alcohol, the ventricles tend to become smaller and the sulcal widening is less apparent This change in ventricular size is probably related to a shift of brain tissue uids
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