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We read with interest the suggestion for new criteria for the diagnosis of pseudotumor cerebri syndrome and idiopathic intracranial hypertension written by our esteemed colleagues.1 We disagree with the need for this exercise since the nomenclature we have used over the years and for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT), we believe, is simpler, is accurate, describes the condition, and is easily modified as new information surfaces. Diseases or syndromes should be named for what they are—“idiopathic intracranial hypertension”—rather than what they are not—“pseudotumor cerebri,” “primary pseudotumor cerebri” or “pseudotumor cerebri syndrome (PTCS).” This was impressed on us during a visit to the National Eye Institute many years ago when we were told “we are not going to fund a pseudo anything.” We do not believe there is need for the term PTCS. Secondary causes of intracranial hypertension should also be called what they are: e.g., vitamin A-induced intracranial hypertension, tetracycline-induced intracranial hypertension, steroid withdrawal-related intracranial hypertension, rather than subsuming them with the PTCS acronym. When criteria for IIH are not met and no secondary cause is found, “intracranial hypertension of unknown cause” should …

Background: Idiopathic intracranial hypertension (IIH) typically affects young, obese women. We examined 2 groups of atypical patients with IIH: those with a normal body mass index (BMI) and those at least 50 years of age. Methods: A retrospective cohort study of 407 consecutive adult patients with IIH with known BMI from 3 centers was undertaken. Demographics, associated factors, visual acuity, and visual fields were collected at presentation and follow-up. Results: We identified 18 IIH patients (4%) with normal BMI and 19 (5%) aged 50 years or older at the time of diagnosis who were compared with the remainder of the cohort. Medication-induced IIH was more frequent in patients with IIH with normal BMI (28 vs 7%, p = 0.008). No patient with IIH with a normal BMI had severe visual loss in either eye (0 vs 17%, p = 0.09). Older patients with IIH had a lower BMI, but were still generally obese (33 vs 38, p = 0.04). Older patients were less likely to report headache as initial symptom (37 vs 76%, p p = 0.03). Treatment of any type was less likely in older patients (significant for medications: 74 vs 91%, p = 0.004), and they were more likely to have persistent disc edema at last follow-up (median Frisen grade: 1 vs 0, p = 0.002), but had similar, if not better, visual outcomes compared with younger patients. A case-control study did not identify any new medication or risk factor associations. Conclusions: Patients with normal body mass index and those 50 years or older make up a small proportion of patients with idiopathic intracranial hypertension (IIH), but appear to have better visual outcomes than more typical patients with IIH.

To identify cutpoints for visual measures at baseline and 1 month predictive of abnormal 6-month vision that could be used as eligibility criteria in a clinical trial to test potential neuroprotection or myelin repair agents in patients with optic neuritis. To determine whether moderate-to-severe dysfunction in one or more visual measures at baseline or 1 month correlates with having major vision loss at 6 months.We used the Optic Neuritis Treatment Trial database to evaluate various cutpoints for baseline and 1-month vision levels that predicted abnormal 6-month vision. For selected cutpoints, we computed a 95% CI for positive predictive value and the required sample size if the cutpoint was to be used for clinical trial eligibility. We evaluated whether the degree of visual loss at baseline, 1 month, or change in visual function from baseline to 1 month correlated with 6-month visual acuity, contrast sensitivity, or threshold visual field.The best cutpoints for baseline and 1 month were visual acuityor= 20/50, contrast sensitivity1.0 log units, and visual field mean deviationor= -15 dB. The same levels of visual dysfunction at 1 month, but not at baseline, correlated with having 6-month moderate-to-severe loss for each of these measures (p = 0.01). A trial could require as few as 100 subjects for an outcome variable of one or more abnormal measures. Cutpoints at 1 month were highly predictive of abnormal 6-month vision, but the proportion of patients who would be eligible for a trial would be small.Provided data can be used either for the clinician to counsel patients on expected visual outcome or for designing studies to test therapies that might reduce the amount of permanent optic nerve damage due to optic neuritis in high-risk patients.

The authors compared the accuracy of clinical detection (by 279 physician observers) of internuclear ophthalmoparesis (INO) with that of quantitative infrared oculography. For the patients with mild adduction slowing, INO was not identified by 71%. Intermediate dysconjugacy was not detected by 25% of the evaluators. In the most severe cases, INO was not identified by only 6%. Oculographic techniques significantly enhance the precision of INO detection compared to the clinical exam.

To compare the characteristics of idiopathic intracranial hypertension (IIH) in men vs women in a multicenter study.Medical records of all consecutive patients with definite IIH seen at three university hospitals were reviewed. Demographics, associated factors, and visual function at presentation and follow-up were collected. Patients were divided into two groups based on sex for statistical comparisons.We included 721 consecutive patients, including 66 men (9%) and 655 women (91%). Men were more likely to have sleep apnea (24% vs 4%, p0.001) and were older (37 vs 28 years, p = 0.02). As their first symptom of IIH, men were less likely to report headache (55% vs 75%, p0.001) but more likely to report visual disturbances (35% vs 20%, p = 0.005). Men continued to have less headache (79% vs 89%, p = 0.01) at initial neuro-ophthalmologic assessment. Visual acuity and visual fields at presentation and last follow-up were significantly worse among men. The relative risk of severe visual loss for men compared with women was 2.1 (95% CI 1.4-3.3, p = 0.002) for at least one eye and 2.1 (95% CI 1.1-3.7, p = 0.03) for both eyes. Logistic regression supported sex as an independent risk factor for severe visual loss.Men with idiopathic intracranial hypertension (IIH) are twice as likely as women to develop severe visual loss. Men and women have different symptom profiles, which could represent differences in symptom expression or symptom thresholds between the sexes. Men with IIH likely need to be followed more closely regarding visual function because they may not reliably experience or report other symptoms of increased intracranial pressure.

The cause of idiopathic intracranial hypertension (IIH) has long been a mystery and continues to be. There are three major theories of the cause of IIH1: 1) increased resistance to CSF absorption; 2) increased CSF production; and 3) within the past few years, increased venous sinus pressure. In fact, increased venous sinus pressure is now hypothesized to be the cause of IIH. Venous sinus occlusion and elevated venous sinus pressure are a proven cause of elevated intracranial pressure. MRI and MR venography now make it easier to identify venous sinus occlusion. Biousse et al.6 have shown that 54 of 160 patients with venous sinus occlusion had only symptoms of headache and papilledema that could have been construed as IIH. Many had normal CSF constituents and elevated CSF pressure. In Melbourne, King et al.2 have measured intracranial venous sinus pressures with transducers in …

We studied the relationship between the CSF protein concentration and the degree of elevation of the CSF opening pressure in 177 patients with idiopathic intracranial hypertension (pseudotumor cerebri). There was no linear correlation between the CSF protein and opening pressure. In addition, low CSF protein (less than 20 mg/dl) was present in only 26% of the patients. Our findings fail to confirm previous reports of an inverse relationship between the CSF opening pressure and CSF protein as well as low CSF protein in these patients.

The neuro-ophthalmic community lost a special friend, colleague, and teacher when Dan Jacobson died on July 27, 2003, at age 47. A leader in his field, and known to his colleagues as an unassuming and gentle teacher, Dan fought a 3-year battle with esophageal cancer. Dan graduated from the University of Texas with a degree in biochemistry and attended the University of Texas Medical School in Houston. He came to the University of Iowa for a fellowship in neuro-ophthalmology in 1985 after a Neurology residency at the University of Pittsburgh. Under the mentorship of Drs. James Corbett and Stanley Thompson, he immediately fit in and found a number of projects he wanted to work on, which included eye movements, ophthalmoscopy, and pupillary problems. It was clear that he loved what he did and was always eager to see patients and compare notes with colleagues over “coffee and napkin rounds” in …

The primary purpose was to investigate whether serum vitamin A concentration is associated with idiopathic intracranial hypertension (IIH). The secondary aim was to obtain pilot data regarding the amount of vitamin A ingested by patients and controls.Vitamin A is an attractive candidate mediator of IIH as many of the symptoms and signs of hypervitaminosis A mimic those of IIH.We prospectively determined serum retinol and retinyl ester concentration in 16 women with IIH and 70 healthy young women. Using a survey instrument, we also determined the average daily vitamin A ingestion in a convenience sample of patients and controls.Serum retinol concentration was significantly higher in the patient group (median 752 ug/L) compared with the control group (median 530 ug/L), even after adjusting for age and body mass index (p0.001). Retinyl ester concentration, however, was similar in the patient (median 48 ug/L) and control (median 41 ug/L) groups (p = 0.32). There was no significant correlation between serum retinol concentration and body mass index in the patients (r = 0.16) or controls (r = -0.02). Finally, there was no significant difference in the amounts of vitamin A ingested by the patients or controls, although the small number of subjects in both groups reduced the power of this conclusion.Elevated serum retinol concentration is associated with IIH. Obesity, by itself, does not explain these higher levels. Patients may ingest an abnormally large amount of vitamin A, metabolize it abnormally, or be unusually sensitive to its effects. Alternatively, elevated level of serum retinol may reflect an epiphenomenon of another variable we did not measure or a nonspecific effect of elevated retinol binding capacity.

We reviewed the efficacy of CSF diversion for pseudotumor cerebri (PTC) in patients from six different institutions. Thirty-seven patients underwent a total of 73 lumboperitoneal shunts and nine ventricular shunts. Only 14 patients remained “cured” after a single surgical procedure. The average time between shunt insertion and shunt replacement was 9 months, although 64% of shunts lasted less than 6 months. Shunt failure (55%) and low-pressure headaches (21%) were the most common causes for reoperation. The vision of most patients improved (13) or stabilized (13) postoperatively. However, three who had initially improved subsequently lost vision. Six had a postoperative decrease in vision. Two patients improved in one eye but worsened postoperatively in the other. Four lost vision despite apparently adequate shunt function. Shunt failure with relapse of PTC occurred as late as 7 years after insertion. CSF diversion procedures have a significant failure rate as well as a high frequency of side effects.

Ten patients with pseudotumor cerebri associated with renal insufficiency had papilledema and elevated intracranial pressure, but neurologic examinations and CT were normal. The 40% frequency of severe visual loss, 20/100 or worse, was higher than expected for pseudotumor cerebri. While furosemide was effective in 3 patients with renal transplants, administration of corticosteroids to 2 other patients did not halt progressive loss of vision. The visual function of 2 patients deteriorated even after lumboperitoneal shunting. Five patients underwent fenestration of the optic nerve sheath. This procedure improved the visual acuity of 3 patients and stabilized vision in the others. While the pathogenesis of pseudotumor cerebri in renal insufficiency is unknown, optic nerve sheath fenestration is the surgical treatment of choice for visual loss unresponsive to medical therapy.

We studied three patients with spontaneous dorsal mesencephalic hemorrhages. One patient had Parinaud's syndrome with a unilateral hemorrhage confined to the rostral tectal plate. The second patient had vertical gaze palsy, skew deviation, and bilateral Horner's syndrome due to a unilateral hemorrhage that involved the superior colliculus and extended anteriorly into the midbrain tegmentum and inferiorly to the rostral dorsal pons. The third patient had a hemorrhage in the caudal tectal plate, with bilateral fourth cranial nerve palsies, unilateral Horner's syndrome, and ataxia. There was hydrocephalus due to obstruction of the aqueduct in two patients. The patients recovered, but with some degree of disability.

Recognition and learning of complex images may depend on spatial processing characteristics of the visual system. Prosopagnosia, an impairment of visual learning and recognition of faces, might result from impaired perception in spatial channels carrying crucial information. We studied spatial contrast sensitivity (SCS) in two subjects with stable facial recognition defects. One had relative SCS reduction for high-frequency gratings but could process high frequencies in room light. The other had normal SCS. Both had intact spatial processing relative to image size. The results suggest that impairments in visual spatial channels are not necessary for the development of prosopagnosia.

We evaluated 14 patients with idiopathic optic neuritis, 50 years of age or older, within 1 month of symptomatic onset from 1980 through 1985. We obtained follow-up information from case records an average of 13 months after initial examination and from telephone interviews an average of 57 months after initial evaluation. Four patients were men and ten were women. Visual loss was monocular in ten cases and binocular in four. Only one of 18 affected eyes initially had an acuity of 20/30 or better. Eleven eyes recovered acuities of 20/30 or better. There was no apparent benefit to those patients treated with corticosteroid agents. Six patients (43%) developed other neurologic sequelae an average of 16 months later; four (28%) had a second bout of optic neuritis and three (21%) developed clinically definite MS. Optic neuritis in the elderly behaves similarly to that disorder in younger age groups.

Cerebrospinal fluid (CSF) pressure was measured in four groups of patients: 116 with acute pseudotumor cerebri, 18 with chronic pseudotumor cerebri, 41 obese and 15 nonobese normal patients undergoing spinal anesthesia. Spinal fluid pressure between 200 and 250 mm H2O was found in members of each group. This suggests that when elevated CSF pressure is suspected, confirmation requires values greater than 250 mm H2O. The CSF pressure and degree of obesity could not be correlated in any meaningful way. There was no significant statistical difference between the mean CSF pressures obtained in the obese and nonobese normal populations.

Pseudotumor cerebri (PTC) is most commonly seen in obese women of reproductive age. We studied 109 women with PTC between ages 16 and 44 years. In 11, PTC started during pregnancy. Thirteen women with previous diagnosis of PTC, including two of the aforementioned 11, had an additional 17 documented pregnancies. Patients were matched by age and parity with controls. Obstetric complications occurred more frequently in the controls. Visual loss occurred with the same frequency in pregnant and nonpregnant patients. Treatment of PTC patients in pregnancy should be the same as for nonpregnant PTC patients, except that calorie restriction and diuretic use are contraindicated. Obstetric management is no different from that of normal pregnancy.

We studied eight patients with CNS sarcoidosis, including two patients with postmortem evidence of sarcoid granulomatous angiitis. Neuro-ophthalmologic findings included perivenular sheathing of retinal veins resembling the dripping of candle wax, vitreous cells, anterior uveitis, optic disk edema or granulomas, and conjunctival granulomas. Perivenous sheathing in the retina and the perivascular meningeal lesions in sarcoid angiitis were identical.

A 35-year-old woman developed upbeating nystagmus while recovering from presumed Wernicke-Korsakoff syndrome. The upbeating nystagmus changed to downbeating nystagmus with convergence. The slow-phase velocity of the downbeating nystagmus varied with the convergence effort. We believe that this unusual nystagmus pattern implies convergence input to the supranuclear centers for vertical gaze.

A patient with rheumatoid arthritis and seizures had rheumatoid meningovasculitis on brain biopsy. Infection was excluded as a cause of the seizures and cerebrospinal fluid abnormalities, which resolved with corticosteroids and azathioprine therapy.

We studied serial brainstem auditory evoked potentials (BAEPs) in a patient who had episodic basilar migraine. During migraine attack, significant alteration of wave IV-V was revealed only by rarefaction clicks. The alteration remained unchanged throughout the headache and reverted to normal when the headache subsided. In contrast, BAEPs with condensation clicks were normal during and after the headache.