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Background: We previously reported that an abnormal CSF opening pressure (OP) in children was greater than 28 cm H(2)O. Since elevated intracranial pressure can cause optic nerve head edema (ONHE), we would expect that most patients with ONHE would have an OP greater than 28 cm H(2)O. This study describes the range of OP for children with ONHE and compared them to age-matched controls without ONHE., Methods: Case subjects were children (1-18 years of age) enrolled in a prospective study of CSF OP that demonstrated ONHE at time of lumbar puncture and that the ONHE later resolved. Patients with ONHE secondary to infectious, inflammatory, or ischemic conditions were excluded. Control subjects from the same study, but without ONHE, were matched to cases., Results: Of the 472 subjects enrolled in the study, 41 OP measurements were obtained from 33 patients with ONHE who did not have any exclusionary criteria and matched to 41 control subjects without ONHE. Case subjects had a significantly higher OP (mean, 41.4 cm H(2)0; range, 22-56) than control subjects (mean, 18.9 cm H(2)O; range, 9-29; p < 0.01). Forty of 41 (97.6%) case subjects and 2 of 41 (4.8%) control subjects had OP measures >28 cm H(2)O., Conclusions: Children with ONHE not related to infectious, inflammatory, or ischemic causes typically have an OP >28 cm H(2)O, significantly higher than age-matched controls without ONHE. This study provides further support to our previously published findings that suggests an abnormal OP in children is typically above 28 cm H(2)O.

Background: Acute optic neuritis occurs with and without papillitis. The presence of papillitis has previously been thought to imply an anterior location of the neuritis, but imaging studies seeking to test this hypothesis have been inconclusive., Methods: This prospective observational cohort study included 41 patients with unilateral optic neuritis and 19 healthy volunteers. All patients were evaluated and examined within 28 days of onset of symptoms. The peripapillary retinal nerve fiber layer thickness (RNFLT), an objective quantitative measure of optic nerve head edema, was measured by optical coherence tomography and the length and location of the inflammatory optic nerve lesion were evaluated using MRI., Results: Ophthalmoscopically, 34% of the patients had papillitis. The retinal nerve fiber layer in affected eyes (mean 123.1 microm) was higher during the acute phase than that of fellow eyes (mean 98.1 microm, p < 0.0001) and higher than that in healthy control eyes (mean 97.1 microm, p < 0.0001). The RNFLT was related to the length of the optic nerve lesion (p = 0.0002), but not to the location of the optic nerve lesions (p = 0.72)., Conclusions: In this study of the acute phase of optic neuritis, the degree of optic nerve head edema depended upon the extent of the optic nerve lesion, but not on its location. This suggests that factors other than inflammation, such as compromised venous drainage, vascular leakage, impaired axonal transport, and other mechanisms, are involved in the development of optic nerve head edema in optic neuritis.

The syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal CSF composition, previously referred to as pseudotumor cerebri, is a diagnosis of exclusion now termed idiopathic intracranial hypertension (IIH). Diagnostic criteria of this disorder have not been updated since the Modified Dandy Criteria were articulated in 1985. Since then, new developments, including advances in neuroimaging technology and recognition of additional secondary causes of intracranial hypertension, have further enhanced the ability to diagnose conditions that may mimic IIH. These factors are not addressed in the Modified Dandy Criteria. This report describes updated diagnostic criteria for IIH that may be used for routine patient management and for research purposes.

Truly unilateral papilledema is rare and poses a diagnostic problem. The authors have prospectively looked for patients with truly unilateral papilledema and found 15 patients, 10 of whom had idiopathic intracranial hypertension. Neuroimaging did not indicate a reason for the lack of swelling in the other nerve. The visual deficits and outcomes were similar to those of patients with bilateral papilledema. Although monocular papilledema is uncommon, a lumbar puncture with opening pressure measurement should be considered.

I compared six subjects with pseudotumor cerebri and unilateral or highly asymmetric papilledema with 20 such patients with bilateral papilledema. Patients with unilateral papilledema were significantly older than those with bilateral papilledema, but there were no significant differences between the two groups in disease duration, elevation of intracranial pressure, visual fields and acuity, and presence of headaches or transient visual obscurations. I hypothesize that, in patients with unilateral papilledema, one optic nerve is protected from pressure effects by optic nerve sheath anomalies or lamina cribrosa changes due to increased collagen and decreased elasticity with aging.