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Benign intracranial hypertension in pregnancy
CASE REPORT
Benign Intracranial Hypertension in Pregnancy Stephen D. Henry, MD Skip Jacques, MD Pasadena, California
Benign intracranial hypertension, an unusual case of headaches, was associated with pregnancy in a gravid female with the chief complaint of headache. Symptoms resolved after three days of prednisone therapy. In benign intracranial hypertension, the possibilities of intracranial mass lesions, intracranial infection, obstruction of the cerebral ventricles, and hypertensive encephalopathy are excluded by evaluation with skull films, computerized axial tomography, electroencephalography and lumbar puncture. The only symptom may be headache and the only physical sign, papilledema. Therefore, neurological examination must include visualization of the fundi. Henry SD: Benign intracranial hypertension in pregnancy. JACEP 8:323-325, August, 1979. hypertension, pregnancy
INTRODUCTION Benign i n t r a c r a n i a ] h y p e r t e n s i o n (pseudotumor cerebri) is a n i n f r e q u e n t cause of headaches. Because t h e r e a r e very few physical findings, the diagnosis m a y be overlooked if a n a d e q u a t e funduscopic e x a m i n a t i o n is not performed. We report a case of benign i n t r a c r a n i a l hypertension associated w i t h p r e g n a n c y seen in the E m e r g e n c y D e p a r t m e n t of H u n t i n g t o n Memorial Hospital.
CASE REPORT A 24-year-old woman, g r a v i d a 6, p a r a 4, a b o r t a 1, came to t h e e m e r g e n c y d e p a r t m e n t c o m p l a i n i n g o f b i f r o n t a l headaches, occasional visual blurring, m i l d i n t e r m i t t e n t n a u s e a w i t h o u t vomiting, and occasional buzzing t i n n i t u s of one month's duration. Additionally, she r e p o r t e d n e a r l y continuous dizziness d u r i n g the preceding t h r e e days. The p a t i e n t h a d been using an oral contraceptive w i t h r e g u l a r menses until a p p r o x i m a t e l y 20 weeks prior to admission. A t t h a t time, she discontinued use and subsequently h a d no further menses. A p p r o x i m a t e l y 12 weeks prior to admission she h a d a positive urine p r e g n a n c y test but she did not p r e s e n t for a p r e n a t a l clinic e x a m i n a t i o n . She denied use of any medications or v i t a m i n preparations. The p a t i e n t ' s blood pressure was 130/76 m m Hg; pulse rate, 88/minute; resp i r a t o r y rate, 24/minute, and oral t e m p e r a t u r e was 36.9 C (98.5 F). E x a m i n a tion revealed a g r a v i d u t e r u s of a p p r o x i m a t e l y 16 w e e k s gestational size. H e r left pupil was 7 m m and the r i g h t pupil 5 to 6 m m d i a m e t e r . There was constriction of visual fields bitemporally, g r e a t e r on the r i g h t t h a n on the left. E x a m i n a t i o n of the fundi showed b i l a t e r a l grade III p a p i l l e d e m a w i t h h e m o r r h a g e , more pronounced in the r i g h t eye. T h e r e were b i l a t e r a l e x u d a t e s a n d m i l d a r t e r i a l - v e n o u s n i c k i n g (Figure). The results of the r e m a i n d e r of the neurologic From the Emergency Department, Huntington Memorial Hospital, Pasadena, California• Address for reprints: Stephen D• Henry, MD, Emergency Department, Huntington Memorial Hospital, 100 Congress Street, Pasadena, California 91105. 8"8 (August)1979
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F i g . Grade III papilledema with hemorrhages and exudates. examination were within normal limits. The p a t i e n t was a d m i t t e d to the obstetric service and neurological consultation was obtained. Visual field examination demonstrated a r i g h t c e n t r a l - t e m p o r a l a n d a left t e m p o r a l scotoma. W h i l e the neurological w o r k u p w a s in progress, the p a t i e n t suffered a m a r k e d deteriorat i o n in h e r v i s i o n a n d w a s subseq u e n t l y b e g u n on prednisone 60 mg daily. A complete blood cell count (CBC) showed a h e m o g l o b i n of 11.2, h e m a t o c r i t r e a d i n g , 33,6, and WBC of 9,600 cu m m w i t h a n o r m a l d i f f e r ential. S e r u m c h e m i s t r i e s were norm a l e x c e p t for a s l i g h t increase in the globulin fraction of t h e total protein. S e r u m electrophoresis showed a g a m m a fraction of 2.7 gm/dl (0.7-1.7 n o r m a l ) a n d I g G o f 2,987 m g / d l (600-1,800 normal). Immunoelectrophoresis was normal. Erythrocyte s e d i m e n t a t i o n r a t e was e l e v a t e d at 102. A n RA, VDRL, A N A and lupus erythematosus preparation were n e g a t i v e . T h e r e s u l t s of a s k u l l series, e l e c t r o e n c e p h a l o g r a m , echoencephalogram, and computerized a x i a l t o m o g r a p h y scan were normal. S e l l a r t o m o g r a m s showed m i l d dem i n e r a l i z a t i o n of t h e l a m i n a duralis. On l u m b a r p u n c t u r e , o p e n i n g
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p r e s s u r e w a s 520 m m H 2 0 . T h e c e r e b r o s p i n a l fluid w a s n o r m a l exc e p t for a s l i g h t i n c r e a s e in t h e g a m m a a n d p r e a l b u m i n fractions on electrophoresis. T h r e e d a y s a f t e r i n i t i a t i o n of prednisone t h e r a p y , visual symptoms l e s s e n e d a n d v i s u a l field e x a m i n a t i o n showed m a r k e d i m p r o v e m e n t . The p a t i e n t was s u b s e q u e n t l y disc h a r g e d to be followed as a n outpat i e n t by t h e obstetric and neurology services.
DISCUSSION Benign intracranial hypertension is a syndrome of increased int r a c r a n i a l p r e s s u r e in which the possibilities of i n t r a c r a n i a l m a s s lesions, i n t r a c r a n i a l infection, obstruction of t h e c e r e b r a l ' v e n t r i c l e s , and hypertensive encephalopathy have been excluded. 1 All clinical manifestations are due to i n c r e a s e d i n t r a c r a n i a l pressure. 2 The most common s y m p t o m is headache, which may range from m i l d to severe, arid is most commonly diffuse or b i f r o n t a l . 2-6 The second m o s t f r e q u e n t c o m p l a i n t is v i s u a l abnormalities, including diplopia, v i s u a l b l u r r i n g , i n t e r m i t t e n t visual d i m m i n g , or a m a u r o s i s fugax. 2-6 O t h e r occasional s y m p t o m s present are nausea, vomiting, dizziness,
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syncopal attacks, t i n n i t u s , and paresthesias. 2-6 Physical findings are minimal. P a p i l l e d e m a is a l m o s t u n i v e r s a l l y p r e s e n t b u t m a y r a n g e from mild to severe. 1-6 H e m o r r h a g e s a n d exudates m a y be p r e s e n t . 3,4,6 V i s u a l a c u i t y m a y be i m p a i r e d b u t c a n be normal. ~'6 O t h e r signs occasionally inc l u d e e n l a r g e d b l i n d spots, v i s u a l field defects, scotoma, s i x t h cranial n e r v e palsy, h o r i z o n t a l n y s t a g m u s , a n d s l u g g i s h l y ~r e a c t i n g p u p i l s . 2-~ The presence of any lateralizing signs (even if mild) or any alteration of m e n t a l s t a t u s m a k e s the diagnosis of benign i n t r a c r a n i a l hypertension suspect. 2-~ The l a b o r a t o r y and radiographic e v a l u a t i o n of these p a t i e n t s is most r e m a r k a b l e for t h e l a c k o f positive findings. Skull x - r a y films are normal or show only signs of increased i n t r a c r a n i a l p r e s s u r e , such as erosion of the base of t h e sella turcica or p o s t e r i o r clinoids, or s e p a r a t i o n of t h e s u t u r e s in childhood.2-6,8, 9 The r e s u l t s of computerized axial tomogr a p h y are n o r m a l or show s l i g h t l y n a r r o w e d l a t e r a l ventricles.3,~,s4°, 11 E l e c t r o e n c e p h a l o g r a m is most freq u e n t l y n o r m a l b u t m a y show mild d i f f u s e changes~-S,12,13 or e v e n l a t e r a l i z e d a b n o r m a l i t i e s in infants and young children. 6 A lumbar p u n c t u r e i n v a r i a b l y shows elevated c e r e b r o s p i n a l fluid p r e s s u r e (CSF), but m u s t show a n o r m a l or low protein.2, v If t h e s e e v a l u a t i v e c r i t e r i a are not met, the diagnosis of benign i n t r a c r a n i a l h y p e r t e n s i o n should be excluded.2, 7
PATHOPHYSIOLOGY Benign intracranial hypertension shows m a r k e d f e m a l e preponderance in the a d u l t age groups (3:1 to 8:1), w h e r e a s the opposite is true in c h i l d r e n . 13 M u l t i p l e d i s o r d e r s h a v e b e e n e t i o l o g i c a l l y associated, including otitis media, ~,3 mastoiditis, o b s t r u c t i o n of t h e s u p e r i o r v e n a c a v a , h e a d t r a u m a , pregnancy,l,s, ~5 menarche,1, ~6 m e n s t r u a l i r r e g u l a r i t i e s , l , 9 , ~7 o r a l c o n t r a c e p t i v e s , ~ , 3,s obesity,~,~,9, ~s w i t h d r a ~ c a l of corticosteroids,~,3, ~9-2a h y p 0 p a r a t h y r o i d ism, 1 acute iron deficiency anemia, ~ W i s k o t t - A l d r i c h syndrome, ~ chronic h y p e r v i t a m i n o s i s A, ~,2,~2,23 tetracyc l i n e t h e r a p y , T M n a l i d i x l c acid t h e r a p y , ~ v i t a m i n D deficiency rickets, 27 n i t r o f u r a n t o i n t h e r a p y , 25 ind o m e t h a c i n t h e r a p y , 26 p a t e n t ductus a r t e r i o s u s l i g a t i o n , 2s a n d h y p e r a l i mentation. 29 No d e f i n i t i v e p a t h o p h y s i o l o g i c mechanism has been demonstrated
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to explain adequately all the diverse etiologic associations t h a t m a y lead to b e n i g n i n t r a c r a n i a l hypertension. T h e r e is, h o w e v e r , evidenceT,13, 3° that suggests the p r i mar y pathophysiologic defect is i n c r e a s e d int r a c r a n i a l pressure secondary to decreased CSF absorption r e s u l t i n g from a n increase i n superior saggital sinus pressure a n d n o r m a l arachnoid villi. As p r e s s u r e w i t h i n the s i n u s increases, the r e s u l t i n g increased pressure will be distributed e q u a l l y to t h e C S F a n d the b r a i n p a r e n chyma. This yields hydrostatic loading of both c o m p a r t m e n t s w i t h o u t ventriculomegaly in adults with closed sutures. 13 Greer 15 reviewed the course of eight patients with pregnancyassociated b e n i g n i n t r a c r a n i a l hypertension. He noted t h a t the majority had a short d u r a t i o n of symptoms a n d w e r e d i a g n o s e d at 16 to 20 weeks gestation. He suggested t h a t the b e n i g n i n t r a c r a n i a l h y p e r t e n s i o n i n t h e s e p a t i e n t s w a s due to t h e physiologic decrease in s e r u m cortisol from a previously high level at 18 days gestation, to a lower level at four months, before rising to a peak at seven months. He postulated t h a t these p a t i e n t s have a r e l a t i v e adr e n o c o r t i c a l i n s u f f i c i e n c y , b u t the course would be short-lived due to the n a t u r a l l y r i s i n g cortisol levels t h r o u g h o u t the r e m a i n d e r of pregnancy. 1~
TREATMENT AND PROGNOSIS T h e r a p y i n most s i t u a t i o n s is expectant. ~-3,~ If t h e r e is a k n o w n p h a r m a c o l o g i c p r e c i p i t a t i n g factor (eg, tetracycline, nalidixic acid, Vit a m i n A), it should be withdrawn. 1 In c h i l d r e n , the course of t h i s disease is almost always b e n i g n and short-lived. 3 In adults, there is often s p o n t a n e o u s remission.2, T M In refractory cases, repeated l u m b a r punctures, steroids, and/or acetazol a m i d e h a v e b e e n used w i t h success.l-3,5 If there is a rapid deterioration of v i s i o n u n r e s p o n s i v e to the a b o v e m e a s u r e s , s u b t e m p o r a l decompression is indicated. 1 Regardless of the etiology, prognosis is very favorable except for the possibility of recurrence and persisting visual deficits.l,2, 5
CONCLUSION Although benign intracranial h y p e r t e n s i o n is a n i n f r e q u e n t cause of headaches, our case illustrates the
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i m p o r t a n c e of a n adequate neurological e x a m i n a t i o n i n the emergency department including visualization o f the fundi. The only symptom of this entity m a y be headache, and the only positive neurologic finding may be p a p i l l e d e m a . F r e q u e n t l y , no therapeutic i n t e r v e n t i o n need be initiated b u t even i n these cases, close o b s e r v a t i o n is i n d i c a t e d . V i s u a l s y m p t o m s m a y occur a n d progress rapidly, even to blindness, 11 and immediate i n t e r v e n t i o n will be necessary. Special thanks to Zolton Uhasz for supplying Fundus photographs.
Eur Neurol 14:340-350, 1976.
13. Portnoy HD, Croissant PD: Megalencephaly in infants and children. Arch Neur 35:306-316, 1978. 14. Weisberg SA: Benign intracranial hypertension. Medicine 54:197-207, 1975. 15. Greer M: Benign intracranial hypertension: III Pregnancy. Neurology (Minneapolis) 13:670-672, 1963. 16. Greer M: Benign intracranial hypertension: IV Menarche. Neurology (Minneapolis) 14:569-573, 1964. 17. Greer M: Benign intracranial hypertension: V Menstrual dysfunction, Neurology (Minneapolis) I4:668-673, 1964.
REFERENCES
18. Greer M: Benign intracranial hypertension: VI Obesity. Neurology (Minneapolis) 15:382-388, 1965.
1. Greer M: Management of benign intracranial hypertension (pseudotumor cerebri). Clin N e u r o s u r g 13:161-174, 1968.
19. Greer M: Benign intracranial hypertension: II Following corticosteroid therapy. Neurology (Minneapolis) 13:439-441, 1963.
2. Johnston I, Paterson A: Benign intracranial hypertension: diagnosis and prognosis. Brain 97:289-300, 1974. 3. Weisberg JA, Chutorina AM: Pseudotumor cerebri of childhood. A m J Dis Child 131:1243-1248, 1977.
20. Fourie DT: Benign i n t r a c r a n i a l hypertension after topical steroid withdrawal. S A f t Med J 52:301, 1977. 21. Roussonis SH: Benign intracranial hypertension after withdrawal of topical steroids in an infant. Br Med J 2:564, 1976.
4. Wilson DH, Gardner J: Benign intracranial hypertension with particular reference to its occurrence in fat young women. J Can Med A s s Soc 95:102105, 1966. 5. Boddie HG, Banna M, Bradley WD: "Benign" intracranial hypertension. A survey of the clinical and radiological features, and long-term prognosis. Brain 97:313-326, 1974. 6. Bedensteiner J, Matsuo F: EEG in benign intracranial hypertension. Dis Nerv Syst 38:1007-1010, 1977. 7. Johnston I, Paterson A: Benign intracranial hypertension. CSF pressure and circulation. Brain 97:301-312, 1974. 8. Delaney P: Schellinger D: Computerized tomograpby and benign intracranial hypertension. J A M A 236:951-952, 1976. 9. Traviesa DC, Schwartzman RJ, Glaser JS, et al: Familial benign intracranial hypertension. J Neurol Neurosurg Psychiatry 39:420-423, 1976. 10. Huckman MS, Fox JS, Ramsey RG, et al: Computerized tomography in the diagnosis of pseudotumor cerebri. Radiology 119:593-597, 1976. 11. Evens RG, Rujunavech N, Mikhael MA: Utilization, reliability, and cost effectiveness of cranial computed temography in evaluating pseudotumor cerebri. A m J Roentgenol 129:263-265, 1977. 12. Lombaert A, Carton H: Benign intracranial hypertension due to a hyperavitaminosis in adults and adolescents.
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22. Hosking GP, Elliston H: Benign intracranial hypertension in a child with eczema treated with topical steroids. Br Med J 1:550-551, 1978. 23. Pasquariello PS, Schut L, Barnes P: Benign increased intracranial hypertension due to chronic vitamin A overdosage in a 26-month-old child. Clin Pediatr 16:379-382, 1977. 24. Ohlrich GD, Ohlrich J G : Papilloedema in an adolescent due to tetracycline. Med J Aust 1:334-335, 1977. 25. Mushet GR: Pseudomotor and nitrofurantoin therapy. Arch Neur 34:257, 1977. 26. Konomi H, Tada H: Indomethacin causing pseudotumor cerebri in Barrter's syndrome. N Engl J Med 298:855, 1978. 27. Hochman HI, MejIszenkier JD: Cataracts and pseudotumor cerebri in an infant with vitamin D-deficiency rickets. J Pediatr 90:252-254, 1977. 28. Di Liberta J, O'Brien MD: Pseudotumor cerebri following patent ductus arteriosus ligation. J Pediatr 87:489, 1975. 29. Saxena VK, Heilpern J, Murphy SF: Pseudotumor cerebri: A complication of parenteral hyperalimentation. J A M A 235:2124, 1976. 30. Johnston I: The definition of a reduced CSF absorption syndrome: A reappraisal of benign intracranial hypertension and related conditions. Med Hypotheses 1:10-14, 1975.
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Benign Intracranial Hypertension in Pregnancy Stephen D. Henry, MD Skip Jacques, MD Pasadena, California
Benign intracranial hypertension, an unusual case of headaches, was associated with pregnancy in a gravid female with the chief complaint of headache. Symptoms resolved after three days of prednisone therapy. In benign intracranial hypertension, the possibilities of intracranial mass lesions, intracranial infection, obstruction of the cerebral ventricles, and hypertensive encephalopathy are excluded by evaluation with skull films, computerized axial tomography, electroencephalography and lumbar puncture. The only symptom may be headache and the only physical sign, papilledema. Therefore, neurological examination must include visualization of the fundi. Henry SD: Benign intracranial hypertension in pregnancy. JACEP 8:323-325, August, 1979. hypertension, pregnancy
INTRODUCTION Benign i n t r a c r a n i a ] h y p e r t e n s i o n (pseudotumor cerebri) is a n i n f r e q u e n t cause of headaches. Because t h e r e a r e very few physical findings, the diagnosis m a y be overlooked if a n a d e q u a t e funduscopic e x a m i n a t i o n is not performed. We report a case of benign i n t r a c r a n i a l hypertension associated w i t h p r e g n a n c y seen in the E m e r g e n c y D e p a r t m e n t of H u n t i n g t o n Memorial Hospital.
CASE REPORT A 24-year-old woman, g r a v i d a 6, p a r a 4, a b o r t a 1, came to t h e e m e r g e n c y d e p a r t m e n t c o m p l a i n i n g o f b i f r o n t a l headaches, occasional visual blurring, m i l d i n t e r m i t t e n t n a u s e a w i t h o u t vomiting, and occasional buzzing t i n n i t u s of one month's duration. Additionally, she r e p o r t e d n e a r l y continuous dizziness d u r i n g the preceding t h r e e days. The p a t i e n t h a d been using an oral contraceptive w i t h r e g u l a r menses until a p p r o x i m a t e l y 20 weeks prior to admission. A t t h a t time, she discontinued use and subsequently h a d no further menses. A p p r o x i m a t e l y 12 weeks prior to admission she h a d a positive urine p r e g n a n c y test but she did not p r e s e n t for a p r e n a t a l clinic e x a m i n a t i o n . She denied use of any medications or v i t a m i n preparations. The p a t i e n t ' s blood pressure was 130/76 m m Hg; pulse rate, 88/minute; resp i r a t o r y rate, 24/minute, and oral t e m p e r a t u r e was 36.9 C (98.5 F). E x a m i n a tion revealed a g r a v i d u t e r u s of a p p r o x i m a t e l y 16 w e e k s gestational size. H e r left pupil was 7 m m and the r i g h t pupil 5 to 6 m m d i a m e t e r . There was constriction of visual fields bitemporally, g r e a t e r on the r i g h t t h a n on the left. E x a m i n a t i o n of the fundi showed b i l a t e r a l grade III p a p i l l e d e m a w i t h h e m o r r h a g e , more pronounced in the r i g h t eye. T h e r e were b i l a t e r a l e x u d a t e s a n d m i l d a r t e r i a l - v e n o u s n i c k i n g (Figure). The results of the r e m a i n d e r of the neurologic From the Emergency Department, Huntington Memorial Hospital, Pasadena, California• Address for reprints: Stephen D• Henry, MD, Emergency Department, Huntington Memorial Hospital, 100 Congress Street, Pasadena, California 91105. 8"8 (August)1979
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F i g . Grade III papilledema with hemorrhages and exudates. examination were within normal limits. The p a t i e n t was a d m i t t e d to the obstetric service and neurological consultation was obtained. Visual field examination demonstrated a r i g h t c e n t r a l - t e m p o r a l a n d a left t e m p o r a l scotoma. W h i l e the neurological w o r k u p w a s in progress, the p a t i e n t suffered a m a r k e d deteriorat i o n in h e r v i s i o n a n d w a s subseq u e n t l y b e g u n on prednisone 60 mg daily. A complete blood cell count (CBC) showed a h e m o g l o b i n of 11.2, h e m a t o c r i t r e a d i n g , 33,6, and WBC of 9,600 cu m m w i t h a n o r m a l d i f f e r ential. S e r u m c h e m i s t r i e s were norm a l e x c e p t for a s l i g h t increase in the globulin fraction of t h e total protein. S e r u m electrophoresis showed a g a m m a fraction of 2.7 gm/dl (0.7-1.7 n o r m a l ) a n d I g G o f 2,987 m g / d l (600-1,800 normal). Immunoelectrophoresis was normal. Erythrocyte s e d i m e n t a t i o n r a t e was e l e v a t e d at 102. A n RA, VDRL, A N A and lupus erythematosus preparation were n e g a t i v e . T h e r e s u l t s of a s k u l l series, e l e c t r o e n c e p h a l o g r a m , echoencephalogram, and computerized a x i a l t o m o g r a p h y scan were normal. S e l l a r t o m o g r a m s showed m i l d dem i n e r a l i z a t i o n of t h e l a m i n a duralis. On l u m b a r p u n c t u r e , o p e n i n g
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p r e s s u r e w a s 520 m m H 2 0 . T h e c e r e b r o s p i n a l fluid w a s n o r m a l exc e p t for a s l i g h t i n c r e a s e in t h e g a m m a a n d p r e a l b u m i n fractions on electrophoresis. T h r e e d a y s a f t e r i n i t i a t i o n of prednisone t h e r a p y , visual symptoms l e s s e n e d a n d v i s u a l field e x a m i n a t i o n showed m a r k e d i m p r o v e m e n t . The p a t i e n t was s u b s e q u e n t l y disc h a r g e d to be followed as a n outpat i e n t by t h e obstetric and neurology services.
DISCUSSION Benign intracranial hypertension is a syndrome of increased int r a c r a n i a l p r e s s u r e in which the possibilities of i n t r a c r a n i a l m a s s lesions, i n t r a c r a n i a l infection, obstruction of t h e c e r e b r a l ' v e n t r i c l e s , and hypertensive encephalopathy have been excluded. 1 All clinical manifestations are due to i n c r e a s e d i n t r a c r a n i a l pressure. 2 The most common s y m p t o m is headache, which may range from m i l d to severe, arid is most commonly diffuse or b i f r o n t a l . 2-6 The second m o s t f r e q u e n t c o m p l a i n t is v i s u a l abnormalities, including diplopia, v i s u a l b l u r r i n g , i n t e r m i t t e n t visual d i m m i n g , or a m a u r o s i s fugax. 2-6 O t h e r occasional s y m p t o m s present are nausea, vomiting, dizziness,
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syncopal attacks, t i n n i t u s , and paresthesias. 2-6 Physical findings are minimal. P a p i l l e d e m a is a l m o s t u n i v e r s a l l y p r e s e n t b u t m a y r a n g e from mild to severe. 1-6 H e m o r r h a g e s a n d exudates m a y be p r e s e n t . 3,4,6 V i s u a l a c u i t y m a y be i m p a i r e d b u t c a n be normal. ~'6 O t h e r signs occasionally inc l u d e e n l a r g e d b l i n d spots, v i s u a l field defects, scotoma, s i x t h cranial n e r v e palsy, h o r i z o n t a l n y s t a g m u s , a n d s l u g g i s h l y ~r e a c t i n g p u p i l s . 2-~ The presence of any lateralizing signs (even if mild) or any alteration of m e n t a l s t a t u s m a k e s the diagnosis of benign i n t r a c r a n i a l hypertension suspect. 2-~ The l a b o r a t o r y and radiographic e v a l u a t i o n of these p a t i e n t s is most r e m a r k a b l e for t h e l a c k o f positive findings. Skull x - r a y films are normal or show only signs of increased i n t r a c r a n i a l p r e s s u r e , such as erosion of the base of t h e sella turcica or p o s t e r i o r clinoids, or s e p a r a t i o n of t h e s u t u r e s in childhood.2-6,8, 9 The r e s u l t s of computerized axial tomogr a p h y are n o r m a l or show s l i g h t l y n a r r o w e d l a t e r a l ventricles.3,~,s4°, 11 E l e c t r o e n c e p h a l o g r a m is most freq u e n t l y n o r m a l b u t m a y show mild d i f f u s e changes~-S,12,13 or e v e n l a t e r a l i z e d a b n o r m a l i t i e s in infants and young children. 6 A lumbar p u n c t u r e i n v a r i a b l y shows elevated c e r e b r o s p i n a l fluid p r e s s u r e (CSF), but m u s t show a n o r m a l or low protein.2, v If t h e s e e v a l u a t i v e c r i t e r i a are not met, the diagnosis of benign i n t r a c r a n i a l h y p e r t e n s i o n should be excluded.2, 7
PATHOPHYSIOLOGY Benign intracranial hypertension shows m a r k e d f e m a l e preponderance in the a d u l t age groups (3:1 to 8:1), w h e r e a s the opposite is true in c h i l d r e n . 13 M u l t i p l e d i s o r d e r s h a v e b e e n e t i o l o g i c a l l y associated, including otitis media, ~,3 mastoiditis, o b s t r u c t i o n of t h e s u p e r i o r v e n a c a v a , h e a d t r a u m a , pregnancy,l,s, ~5 menarche,1, ~6 m e n s t r u a l i r r e g u l a r i t i e s , l , 9 , ~7 o r a l c o n t r a c e p t i v e s , ~ , 3,s obesity,~,~,9, ~s w i t h d r a ~ c a l of corticosteroids,~,3, ~9-2a h y p 0 p a r a t h y r o i d ism, 1 acute iron deficiency anemia, ~ W i s k o t t - A l d r i c h syndrome, ~ chronic h y p e r v i t a m i n o s i s A, ~,2,~2,23 tetracyc l i n e t h e r a p y , T M n a l i d i x l c acid t h e r a p y , ~ v i t a m i n D deficiency rickets, 27 n i t r o f u r a n t o i n t h e r a p y , 25 ind o m e t h a c i n t h e r a p y , 26 p a t e n t ductus a r t e r i o s u s l i g a t i o n , 2s a n d h y p e r a l i mentation. 29 No d e f i n i t i v e p a t h o p h y s i o l o g i c mechanism has been demonstrated
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to explain adequately all the diverse etiologic associations t h a t m a y lead to b e n i g n i n t r a c r a n i a l hypertension. T h e r e is, h o w e v e r , evidenceT,13, 3° that suggests the p r i mar y pathophysiologic defect is i n c r e a s e d int r a c r a n i a l pressure secondary to decreased CSF absorption r e s u l t i n g from a n increase i n superior saggital sinus pressure a n d n o r m a l arachnoid villi. As p r e s s u r e w i t h i n the s i n u s increases, the r e s u l t i n g increased pressure will be distributed e q u a l l y to t h e C S F a n d the b r a i n p a r e n chyma. This yields hydrostatic loading of both c o m p a r t m e n t s w i t h o u t ventriculomegaly in adults with closed sutures. 13 Greer 15 reviewed the course of eight patients with pregnancyassociated b e n i g n i n t r a c r a n i a l hypertension. He noted t h a t the majority had a short d u r a t i o n of symptoms a n d w e r e d i a g n o s e d at 16 to 20 weeks gestation. He suggested t h a t the b e n i g n i n t r a c r a n i a l h y p e r t e n s i o n i n t h e s e p a t i e n t s w a s due to t h e physiologic decrease in s e r u m cortisol from a previously high level at 18 days gestation, to a lower level at four months, before rising to a peak at seven months. He postulated t h a t these p a t i e n t s have a r e l a t i v e adr e n o c o r t i c a l i n s u f f i c i e n c y , b u t the course would be short-lived due to the n a t u r a l l y r i s i n g cortisol levels t h r o u g h o u t the r e m a i n d e r of pregnancy. 1~
TREATMENT AND PROGNOSIS T h e r a p y i n most s i t u a t i o n s is expectant. ~-3,~ If t h e r e is a k n o w n p h a r m a c o l o g i c p r e c i p i t a t i n g factor (eg, tetracycline, nalidixic acid, Vit a m i n A), it should be withdrawn. 1 In c h i l d r e n , the course of t h i s disease is almost always b e n i g n and short-lived. 3 In adults, there is often s p o n t a n e o u s remission.2, T M In refractory cases, repeated l u m b a r punctures, steroids, and/or acetazol a m i d e h a v e b e e n used w i t h success.l-3,5 If there is a rapid deterioration of v i s i o n u n r e s p o n s i v e to the a b o v e m e a s u r e s , s u b t e m p o r a l decompression is indicated. 1 Regardless of the etiology, prognosis is very favorable except for the possibility of recurrence and persisting visual deficits.l,2, 5
CONCLUSION Although benign intracranial h y p e r t e n s i o n is a n i n f r e q u e n t cause of headaches, our case illustrates the
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i m p o r t a n c e of a n adequate neurological e x a m i n a t i o n i n the emergency department including visualization o f the fundi. The only symptom of this entity m a y be headache, and the only positive neurologic finding may be p a p i l l e d e m a . F r e q u e n t l y , no therapeutic i n t e r v e n t i o n need be initiated b u t even i n these cases, close o b s e r v a t i o n is i n d i c a t e d . V i s u a l s y m p t o m s m a y occur a n d progress rapidly, even to blindness, 11 and immediate i n t e r v e n t i o n will be necessary. Special thanks to Zolton Uhasz for supplying Fundus photographs.
Eur Neurol 14:340-350, 1976.
13. Portnoy HD, Croissant PD: Megalencephaly in infants and children. Arch Neur 35:306-316, 1978. 14. Weisberg SA: Benign intracranial hypertension. Medicine 54:197-207, 1975. 15. Greer M: Benign intracranial hypertension: III Pregnancy. Neurology (Minneapolis) 13:670-672, 1963. 16. Greer M: Benign intracranial hypertension: IV Menarche. Neurology (Minneapolis) 14:569-573, 1964. 17. Greer M: Benign intracranial hypertension: V Menstrual dysfunction, Neurology (Minneapolis) I4:668-673, 1964.
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4. Wilson DH, Gardner J: Benign intracranial hypertension with particular reference to its occurrence in fat young women. J Can Med A s s Soc 95:102105, 1966. 5. Boddie HG, Banna M, Bradley WD: "Benign" intracranial hypertension. A survey of the clinical and radiological features, and long-term prognosis. Brain 97:313-326, 1974. 6. Bedensteiner J, Matsuo F: EEG in benign intracranial hypertension. Dis Nerv Syst 38:1007-1010, 1977. 7. Johnston I, Paterson A: Benign intracranial hypertension. CSF pressure and circulation. Brain 97:301-312, 1974. 8. Delaney P: Schellinger D: Computerized tomograpby and benign intracranial hypertension. J A M A 236:951-952, 1976. 9. Traviesa DC, Schwartzman RJ, Glaser JS, et al: Familial benign intracranial hypertension. J Neurol Neurosurg Psychiatry 39:420-423, 1976. 10. Huckman MS, Fox JS, Ramsey RG, et al: Computerized tomography in the diagnosis of pseudotumor cerebri. Radiology 119:593-597, 1976. 11. Evens RG, Rujunavech N, Mikhael MA: Utilization, reliability, and cost effectiveness of cranial computed temography in evaluating pseudotumor cerebri. A m J Roentgenol 129:263-265, 1977. 12. Lombaert A, Carton H: Benign intracranial hypertension due to a hyperavitaminosis in adults and adolescents.
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22. Hosking GP, Elliston H: Benign intracranial hypertension in a child with eczema treated with topical steroids. Br Med J 1:550-551, 1978. 23. Pasquariello PS, Schut L, Barnes P: Benign increased intracranial hypertension due to chronic vitamin A overdosage in a 26-month-old child. Clin Pediatr 16:379-382, 1977. 24. Ohlrich GD, Ohlrich J G : Papilloedema in an adolescent due to tetracycline. Med J Aust 1:334-335, 1977. 25. Mushet GR: Pseudomotor and nitrofurantoin therapy. Arch Neur 34:257, 1977. 26. Konomi H, Tada H: Indomethacin causing pseudotumor cerebri in Barrter's syndrome. N Engl J Med 298:855, 1978. 27. Hochman HI, MejIszenkier JD: Cataracts and pseudotumor cerebri in an infant with vitamin D-deficiency rickets. J Pediatr 90:252-254, 1977. 28. Di Liberta J, O'Brien MD: Pseudotumor cerebri following patent ductus arteriosus ligation. J Pediatr 87:489, 1975. 29. Saxena VK, Heilpern J, Murphy SF: Pseudotumor cerebri: A complication of parenteral hyperalimentation. J A M A 235:2124, 1976. 30. Johnston I: The definition of a reduced CSF absorption syndrome: A reappraisal of benign intracranial hypertension and related conditions. Med Hypotheses 1:10-14, 1975.
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