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Show Journal of'A] euro- Ophthalmology 21( 1): 15- 17, 2001. © 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Do Men With Pseudotumor Cerebri Share the Same Characteristics as Women? A Retrospective Review of 141 Cases A. Kesler, MD, Y. Goldhammer, MD, and N. Gadoth, MD Objective: To determine whether males with pseudotumor cerebri ( PTC) differ from females by clinical presentation, risk factors, and outcome. Methods: The medical records of patients diagnosed with PTC or idiopathic intracranial hypertension ( IIH) in two major university hospitals were obtained. Diagnostic criteria, clinical features, presence of obesity, mode of treatment, and outcome were tabulated. Results: A total of 134 patients ( 18 males and 116 females) fulfilled the Dandy diagnostic criteria for PTC. Females and males shared similar clinical features and outcome. There was a substantial difference between the groups regarding body weight. The majority of females ( 77.8%) were considered significantly overweight, compared to 25% of the males. Conclusion: Pseudotumor cerebri in males is relatively rare. The clinical features are identical to those found in females. The fact that the majority of the male patients had a normal body weight may indicate that increased body weight does not play a major role in causing PTC in men, whereas it is an established major risk factor in women. Key Words: Pseudotumor cerebri- Intracranial hypertension- Obesity- Papilledema. Pseudotumor cerebri ( PTC), or idiopathic intracranial hypertension ( IIH), is a disorder associated with intracranial pressure greater than 250 mm of water, normal neuroimaging ( apart from possible small ventricles), and normal cerebrospinal fluid content ( 1). The annual incidence of PTC in the general population is 0.9 per 100,000 people. Case control studies performed to date have shown a substantial association between PTC and obesity ( 2,3), which may be present in more than 90% of adult women with PTC. Although the established peak incidence of PTC in obese females aged 15 to 44 years is 7.9 per 100,000 people ( 4- 6), there are Manuscript received April 7, 2000; accepted October 27, 2000. From the Departments of Neurology, Meir General Hospital ( AK, NG), Sapir Medical Center, Kfar Saba, and Chaim Sheba Medical Center ( YG), Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Israel. Address correspondence and reprint requests to Anat Kesler, MD, Department of Neurology, Meir General Hospital, Kfar Saba 44281, Israel; e- mail: kesler@ netvision. net. il. no such data regarding males. Digre and Corbett ( 7) were able to provide only a relative percentage ( 16%) of males with PTC among their 187 patients; however, they quote rates ranging from 17 to 35% in a large series with more than 50 patients. With PTC in males ranging from 6 to 50%, depending on the study ( 8- 13), we were able to trace only one study in which the issue of PTC in males was addressed ( 7). According to this study by Digre and Corbett, 16% of the patients were males. The present study was designed to review the symptoms, clinical course, and risk factors in males with PTC and to compare them with similarly affected females. PATIENTS AND METHODS We reviewed the medical records of 141 consecutive patients with PTC older than 14 years who fulfilled the Dandy criteria for PTC ( 10). All patients were diagnosed by two of the authors ( AK and YG) during the period of time from 1982 to 1999, in the Neuro- Ophthalmology outpatient clinics of Meir General Hospital, Kfar Saba, or Sheba Medical Center, Tel- Hashomer, Israel. Patients were regarded as significantly overweight (" obese") according to the examiner's impression. Outcome was considered " resolved" when, after a year from onset, there was no evidence of optic atrophy, visual field defect, headache, or visual complaints. The designation " improved" was used for patients free of headache but with residual optic nerve damage. For statistical analysis, the Mann- Whitney U test was applied for age and diagnostic delay, whereas a chi- square value was calculated for the other parameters. RESULTS All 141 patients underwent contrast- enhanced cranial CT, and 47 patients also had a contrast- enhanced MRI. Only ten patients underwent additional magnetic resonance venography ( MRV). None had a cerebral angiogram. Seven patients ( three women and four men) were excluded because of sinus vein thrombosis ( SVT). Thus, the study population consisted of 134 patients with PTC ( 116 females and 18 males). Fourteen males were diagnosed since 1990, and eight of those patients had an MRI ( MRI was not available in Israel for routine use before 15 16 A. KESLERETAL. 1990). Three males also had MRV. The mean age and age range for males and females were almost identical, with no significant difference regarding the age of diagnosis. There was a significant diagnostic delay in females compared to males. Data referring to body weight were recorded for 16 males and 107 females ( 88.8% and 92.2%, respectively). The majority of females ( 77.6%) were considered significantly overweight, compared to 25% of males. In addition to papilledema, unilateral abducens nerve palsy was present in eight women and one man. The rest of the neurologic examination findings were normal in all 134 patients. Headache was the most common complaint in both sexes. Transient visual obscurations ( TVO) were more common in men ( 68.8% and 42.9%, respectively). Tinnitus was relatively rare. Twenty- one females ( 18.1%) suffered permanent damage to both optic nerves. Seven ( 6.3%) had optic atrophy, and 18 ( 15.5%) had either a nasal field defect or constricted visual fields. Among the 15 males for which data regarding outcome were documented, ten were considered " resolved," five suffered permanent optic nerve damage, two had optic atrophy, one had optic atrophy and nasal field defect, and two had a nasal field defect only. Headache was the sole complaint reported by 50 females ( 43.10%) and 4 males ( 22.2%). Forty- four females and 11 males reported more than one symptom ( 37.93% and 61.1%, respectively). There was no difference in clinical features or outcome between overweight and normal- weight males. The details of the various diagnostic, demographic, and clinical features for the entire patient population is shown in Table 1, and the characteristic data for each male patient is shown in Table 2. Outcome was calculated separately for those patients whose symptoms and signs resolved or improved. Of the females, 57.5% were considered resolved, and 42.5% improved. Of the males, 68.8% resolved, and 31.3% improved. This difference was not significant ( p = 0.3, Fisher exact test). A logistic regression model disclosed that all study parameters, including obesity, did not significantly influence outcome. DISCUSSION This study summarizes our experience with a relatively large group of males with PTC. Previously, only one study, by Digre and Corbett ( 7), which was conducted prior to 1988, dealt specifically with PTC in males. Because obesity and endocrine abnormalities are not considered major risk factors for PTC in males today, occult causes, such as partial thrombosis of the cerebral sinuses, coagulopathies, and dural arteriovenous malformations ( DAVM), should be considered. Indeed, most of our patients ( 14 of 18) were diagnosed after 1990 and thus had a CT with a much better resolution than previously available. Moreover, 8 of the 14 ( 57%) also had a contrast- enhanced MRI. With this finding in mind, we believe that SVT and significant DAVM were excluded. The more advanced neuroimaging may explain the lower rate of PTC in males ( 9.7%) in this study, compared to the 16% found by Digre and Corbett ( 7). The diagnosis of PTC is readily suggested in a young obese female with papilledema. However, in a normal-weight male, headache and papilledema usually cause more concern about a mass lesion. Headache is such a common complaint that unless ophthalmoscopy is not routinely and carefully performed, a significant diagnostic delay of PTC may result. It is not surprising that this delay was greater in our female patients, because women tend to complain about all types of headache ( 14) and are usually given the diagnosis of migraine. In spite of the lack of data on precise body dimension measurements in our patients, it is still striking that only 25% of the males were considered significantly overweight, compared to 77.6% of females. In a recent unpublished study ( Nitzan and Kaluski, Personal communication, Israel Health Nutrition Institute) conducted on 1100 healthy Israeli adults between March 1999 and January 2000, 37.1% of males and 28.1% of females were considered overweight ( body mass index [ BMI]: 25- 30), whereas 16.7% of the females and 10.8% of males were considered obese ( BMI > 30). Hormonal changes are implicated as one of the causative factors for PTC ( 15). Accordingly, 17 of 116 females ( 14.65%) had evidence of hormonal dysfunction TABLE 1. Clinical features of 134 patients with pseudotumor cerebri Mean age, y ( range) Diagnostic delay Significant overweight, % Headache, % TVO, % Abducens palsy, % Tinnitus, % Females ( 116) 31.5 ( 14- 57) 28.4 weeks ( 3 days- 1.5 years) ( n = 83) 77.6 ( 83/ 107) 91.4 42.9 8.62 2.5 Males ( 18) 35 ( 14- 61) 14.57 weeks ( 3 days- 1 year) ( n = 14) 25 ( 4/ 16) 81.3 68.8 0 5.5 Statistical significance p = 0.57 ( MW) p = 0.25 ( MW) p = 0.0002 p = 0.2 p = 0.053 - - MW, Mann- Whitney U test; TVO, transient visual obscurations. / Neuro- Ophthalmol, Vol. 21, No. 1, 2001 MEN AND WOMEN WITH PSEUDOTUMOR CEREBRI 17 TABLE 2. Clinical features of 18 males with pseudotumor cerebri Patient number/ age ( y) 1/ 50 2/ 70 3/ 21 4/ 18 5/ 29 6/ 53 7/ 63 8/ 36 9/ 58 10/ 33 11/ 37 12/ 50 13/ 49 14/ 39 15/ 56 16/ 22 17/ 22 18/ 35 Age of onset ( y) 43 63 16 14 26 46 61 30 54 25 23 36 37 33 55 17 21 30 Year of diagnosis AN BN AN AN AN BN AN AN AN AN BN BN AN AN AN AN AN AN Significant overweight + + + ND + ND Associated conditions Hypertension H yp ercholestero lemia Hypertension Strabismus Hypertension Hypertension Heavy smoker Rheumatic fever Hypertension H yp ercholestero lemia Duration of symptoms before diagnosis 1 year 2 months 6 months 7 days 3 months 3 days 7 days 1 month 2 months 2 months 3 months 1 year ND 1 month 2 months ND ND ND Symptoms Headache Headache, Headache, Headache, Headache, TVO Headache Headache, Headache, Headache, Headache, Headache Headache, TVO TVO TVO TVO TVO blurred vision TVO TVO TVO TVO, Tinnitus, vertigo Headache Recurrence + + + + + Treatment Diamox Diamox, Neptazane Diamox Diamox Diamox, weight reduction Diamox Diamox Diamox Diamox Diamox, Prednisone L- P shunt, Prednisone Diamox, Prednisone ND Diamox, Prednisone Diamox Diamox, Prednisone ND ND Outcome Resolved Improved, nasal field defect Resolved Resolved Resolved Resolved Improved nasal field defect Resolved Resolved Optic atrophy Optic atrophy Resolved ND Optic atrophy, nasal field defect Resolved Resolved ND ND AN, after 1990; BN, before 1990; Diamox ® ( ESI Lederle Generics, Philadelphia, PA), acetazolamide; L- P, lumbo- peritoneal; ND, no data; Neptazane ® ( ESI Lederle Generics, Philadelphia, PA), methazolamide; TVO, transient visual obscurations. ( nine with hypothyroidism, five with polycystic ovaries, two with hyperprolactinemia, and one with hGH deficiency). No hormonal aberrations were recorded in our male patients. There was no difference in the sexes regarding clinical presentation, response to drug treatment, and outcome. The present results should be regarded with some caution. The obesity data lacked standardization concerning height, weight, and body frame, as well as age and gender. Thus, it was not possible to compare our data to those available on a small sample of the adult Israeli population for which morphometric data were recently recorded. The possibility that women may be more anxious and therefore overutilize health services- thus receiving the diagnosis of PTC more frequently- could be ruled out by the finding that the diagnostic delay in females was not significantly different from that in males. Our results seem to suggest that SVT should be seriously considered, particularly in men presenting with PTC. 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