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1 Expert Opinion 495 The management of Pseudotumor Cerebri During Pregnancy Case Histories Submitted by Randolph W. Evans, MD Expert Opinion by Deborah I. Friedman, MD Key words: pseudotumor cerebri, pregnancy, lumbar punctureAbbreviations: PTC pseudotumor cerebri ( Headache 2000;40:495-497) The management of pseudotumor cerebri (PTC)in pregnancy, while not a common problem, raisesseveral difficult issues. CLINICAL HISTORY This 20-year-old woman developed mild headachesand was found to have papilledema in December height was 5 9 4 0 , and her weight was 205 lb. A neu-rologist and an ophthalmologist diagnosed pseudotu-mor cerebri after an evaluation that included a magneticresonance imaging (MRI) scan of the brain with normalfindings, a lumbar puncture revealing an opening pres-sure of 31 cm of water, and a normal cerebrospinal fluid(CSF) examination, except for a decreased proteinlevel.

2 She was prescribed acetazolamide (Diamox), 500mg twice daily. When I saw her in September 1999, shewas 5 months pregnant and had continued taking theacetazolamide throughout the pregnancy. She reportedhaving had no headaches for the previous 4 months andno visual symptoms. Her weight was then 220 lb. Thefindings of the physical examination were normal ex-cept for mild optic disk edema and enlarged blind spots. Question. How would you recommend manag-ing PTC during pregnancy? Expert COMMENTARY The patient was diagnosed with PTC prior to thebeginning of her pregnancy, based on the modifiedDandy criteria for diagnosis: (1) symptoms and signs ofincreased intracranial pressure; (2) normal neuroimag-ing studies, except for an empty sella; (3) CSF pressureof 250 mm of water or greater measured in the lateraldecubitus position with the patient relaxed; (4) normalCSF contents; and (5) other causes cerebri is a syndrome of increasedintracranial pressure without hydrocephalus.

3 It gener-ally occurs in obese women of childbearing age. Themost common symptoms are headache (in more than90% of patients) and visual disturbances (up to 70%of patients), including transient visual obscurations,visual field loss, and loss of central visual acuity. Manypatients experience pulsatile tinnitus, a symptom thatmust be specifically queried since it is not often volun-teered. Other symptoms include neck or back pain,dependent edema of the extremities, diplopia (usuallyfrom unilateral or bilateral abducens palsies, a nonlo-calizing sign of increased intracranial pressure), facialpalsy, ataxia, and types of medication are associated with thedevelopment of PTC, some only reported anecdot-ally. This patient did not use tetracycline or vitaminA-containing medications prior to her diagnosis.

4 Athorough medication history is imperative, since PTCis also associated with various antibiotics, and PTCwithout papilledema may be a manifestation of an-algesic overuse headache. 1 Although there is no in- Address correspondence to Dr. Randolph W. Evans, Suite1370, 1200 Binz, Houston, TX 77004 or Dr. Deborah I. Fried-man, SUNY Health Science Center, 750 East Adams Street,Syracuse, NY 13210. 496 June 2000 creased risk with oral contraceptives, cases of PTChave been reported with Norplant only case-control study looking at PTC inpregnancy showed no increased incidence comparedwith a population matched for age and parity. 2 About15% of women with PTC seek medical attention forsymptoms during pregnancy or relate the onset ofsymptoms to a prior pregnancy. Weight gain, fluid re-tention, and hormonal fluctuations are common toboth conditions.

5 Most patients develop symptomsduring the first trimester. Some women relapse withsubsequent is no contraindication to pregnancy inwomen with PTC, and most patients can be managedduring pregnancy with minimal intervention. There isno demonstrated contraindication to fertility treat-ments in patients with PTC. The prenatal vitaminsshould, however, be checked for their vitamin A con-tent. I generally advise discontinuing any nonessen-tial medication and treat patients with occasionallumbar punctures if needed. Weight gain should belimited to 20 lb. Acetazolamide can be used after 20weeks if necessary, but thiazide diuretics should the most worrisome feature of PTC is visualloss, patients should be followed throughout theirpregnancy by an ophthalmologist or neuro-ophthal-mologist to monitor their vision.

6 Monitoring shouldinclude quantitative visual field testing. In a stable pa-tient with no visual loss, visits every 2 to 3 months areadequate. Patients should be told to call if they detectany change in their vision in the interim. Generally,visual loss in PTC occurs dramatically in the presenceof papilledema. However, treatment should not bebased on the appearance of the optic nerves alone,since many patients have residual optic disk elevationafter their intracranial pressure there is significant visual decline, the patientmay be treated acutely with corticosteroids and a sur-gical procedure performed to relieve the increasedpressure. Depending on the local expertise, either op-tic nerve sheath decompression or lumboperitonealshunting is acceptable. Optic nerve sheath decom-pression usually requires less anesthesia time, alwaysa consideration during treatment of chronic headaches associ-ated with PTC is particularly challenging during preg-nancy.

7 Analgesic rebound headache occurs in pa-tients with PTC, and some women have worsening ofprevious headaches related to hormonal , not all headaches in pregnancy complicated byPTC are caused by increased intracranial instruct patients to submit a diet and symptomjournal and recommend a low tyramine diet. 3 Noneof the commonly used prophylactic medications areconsidered category A (safe to use during preg-nancy). Tricyclic antidepressants must be discontin-ued 2 weeks prior to delivery, and no serious adversereactions have been reported using propranolol forthe treatment of gestational hypertension. The safestanalgesics are meperidine or acetaminophen with co-deine, but neither is appropriate for frequent use. Oc-casional lumbar punctures can often ease headachesymptoms. Surgery is not advocated for the treat-ment of headache prognosis for PTC in pregnancy is excellentfor both mother and baby.

8 Some patients may con-tinue to have symptoms after delivery, but others willhave remission or a fluctuating course. Women withPTC who are considering starting a family should beadvised to plan each pregnancy, in order to adjustmedications in advance and to arrange appropriatemonitoring by a neurologist and with the obstetrician throughout thecourse of pregnancy is imperative, although PTCdoes not generally place a woman at high risk. Theincreased intracranial pressure occurring during la-bor is transient and not harmful. A cesarean sectionis not required, and no special precautions are neces-sary for anesthetic agents at the time of delivery. REFERENCES 1. Wang SJ, Silberstein SD, Patterson S, Young WB. Id-iopathic intracranial hypertension without papille-dema: a case-control study in a headache center.

9 Neurology. 1998;51 Digre KB, Varner MW, Corbett JJ. Pseudotumorcerebri and pregnancy. Neurology. 1984;34 Friedman DI. Low tyramine diet in the treatment ofidiopathic intracranial hypertension: a pilot study[abstract]. Neurology. 1998;50:A5. Headache 497 Invitation to Submit Clinical DilemmasThe Expert Opinion section of Headache isedited by Randolph W. Evans, MD, who invitesreaders to submit clinical problems, case reports,or other headache-related questions for commen-tary by one or more experts. Comments on the ex-pert s Opinion as letters to the editor are also submitted for Expert Opinion , not toexceed 750 words, should be submitted to the jour-nal editor. (All submissions should include theoriginal and four double-spaced copies and thecomputer disk.)