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PURPOSE: To report the features of idiopathic intracranial hypertension in prepubertal children, with emphasis on presentation, treatment, and outcome. METHODS: We retrospectively reviewed the charts of all patients 11 years and younger diagnosed with idiopathic intracranial hypertension at two university-affiliated medical centers. Inclusion critera included papilledema, normal brain computed tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 200 mm H 2 O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy. Patients with concomitant systemic illness were excluded. RESULTS: Of the 10 patients, four were girls and six were boys. Only one patient was obese. The most common presenting symptoms were stiff neck (four patients) and diplopia (four patients), and the most common presenting sign was strabismus (eight patients). Six of eight patients with strabismus had abducens nerve palsy (four bilateral), one patient had a sensory exotropia, and one had a comitant esotropia. Visual field abnormalities were present in 11 of 13 eyes (85%), and severe visual loss resulting in no light perception vision occurred in one eye of one patient. Nine patients were treated medically, four with a combination of prednisone or dexamethasone and acetazolamide and five with acetazolamide alone. One patient in this group also required a lumboperitoneal shunt. One patient was treated with lumbar puncture only. Resolution of papilledema occurred rapidly in all patients, with a mean of 4.7 ± 2.6 months. Resolution of sixth nerve palsy also occurred rapidly in four of six patients in a mean of 1.6 ± 1.2 months. One patient required strabismus surgery for persistent esotropia and one was lost to follow-up. CONCLUSIONS: Idiopathic intracranial hypertension in prepubertal children is rare and is different than the disease in adults. In our series, there appeared to be no sex predilection, and obesity was uncommon. Children are likely to present with strabismus and stiff neck rather than headache or pulsatile tinnitus. Both papilledema and sixth nerve palsy resolved rapidly with treatment. However, children can sustain loss of visual field and visual acuity despite treatment.

To compare the age- and gender-specific testability rates for the Amblyopia Treatment Study (ATS) HOTV visual acuity testing protocol using the electronic visual acuity (EVA) tester in African-American and Hispanic preschool children.Population-based, cross-sectional study.Measurement of presenting monocular distance visual acuity using the ATS HOTV protocol was attempted in all African-American and Hispanic children aged 30 to 72 months from the population-based Multi-Ethnic Pediatric Eye Disease Study (MEPEDS). Children able to be tested monocularly in both eyes were considered able. Age-, gender-, and ethnicity-specific testability rates were calculated. Comparisons of testability among different groups were performed using Chi-square analyses and the Cochran trend test.Testing was attempted on 3,126 children (1,471 African-American, 1,655 Hispanic; 50% female). Overall, 84% (83% African-American, 85% Hispanic; 86% female, 82% male) were testable. Older children were more likely to complete testing successfully than younger children (P.0001). Age-specific testability in children 30 to 36 months of age, 37 to 48 months of age, 49 to 60 months of age, and 61 to 72 months of age was 39%, 84%, 98%, and 100%, respectively. After stratifying by age, there were no ethnicity-related differences in children testable (P = .12). Girls (86%) were slightly more likely to be testable than boys (82%; P.003).Monocular threshold visual acuity testing using the ATS HOTV protocol on the EVA tester (Jaeb Center for Health Research, Tampa, Florida, USA) can be completed by most African-American and Hispanic preschool children, particularly those older than 36 months of age. This protocol therefore may be used in minority preschool children as an integral part of the diagnosis and management of amblyopia and other forms of visual impairment.

PURPOSE: To report preliminary linkage analysis of a large Hispanic family showing autosomal dominant inheritance for Duane retraction syndrome. METHODS: Microsatellite analysis was used to examine genomic DNA isolated from members of a large family with autosomal dominant Duane retraction syndrome for linkage to candidate loci for Duane retraction syndrome. Chromosomes 4, 8, and 22 were chosen for study because previous reports had documented karyotypic abnormalities in unrelated patients with Duane retraction syndrome. RESULTS: No lod scores over 0.5 were found for markers on chromosomes 4, 8, or 22. This analysis excludes these candidate sites. CONCLUSIONS: Studies do not support linkage between Duane retraction syndrome in this family and chromosomes 4, 8, and 22. Duane retraction syndrome may result from mutations in a heterogeneous group of genes.

Because there is a broad spectrum of visual dysfunction in eyes with optic nerve hypoplasia, we determined whether any clinical observations, including optic disk measurements, could predict visual outcome in eyes with hypoplastic optic nerves.We prospectively studied the eyes of 19 children (eight consecutive and 11 randomly selected), ages 7 to 92 months, in whom a diagnosis of optic nerve hypoplasia was made by an ophthalmologist. Clinical assessment included visual acuity, pupillary responses, and nystagmus. The ratio of the horizontal disk diameter to the disk-macula distance was calculated from fundus photographs. The amplitude and latency of the flash visual-evoked potential as well as the threshold response to pattern reversal checks were recorded.Visual acuity was associated with pupil reactivity, nystagmus, the ratio of the horizontal disk diameter to the disk-macula distance, pattern visual-evoked potential threshold, and flash visual-evoked potential amplitude (P.001 for all parameters). All eyes with a ratio of the horizontal disk diameter to the disk-macula distance greater than .30 had good visual acuity, and all eyes with a ratio of the horizontal disk diameter to the disk-macula distance less than .30 had poor visual acuity. No eye with a ratio of the horizontal disk diameter to the disk-macula distance less than or equal to .15 had visual acuity better than light perception.Visual-evoked potential and photogrammetric measurement of relative disk size can be used to approximate visual acuity in eyes with optic nerve hypoplasia. This finding could lead to a diagnostic criterion or a severity classification for optic nerve hypoplasia.

We examined ten patients with nonarteritic anterior ischemic optic neuropathy in whom the visual deficit progressed over a number of weeks or who suffered a recurrence in the same eye. The visual deficits progressed over a month or more in five patients, four patients suffered a recurrence in the same eye after a long interval, and one patient had bilateral progression and a recurrence.

We examined five patients with Alzheimer's disease who complained of poor vision. Two patients had mild Alzheimer's disease; they complained of problems with reading and of "bumping into things," yet both had normal visual acuities. One patient with moderate Alzheimer's disease had abnormal eye movements, visual-evoked potentials, and contrast sensitivity. The other two patients had severe Alzheimer's disease. Despite difficulties in performing the examination, we were able to see moderate impairments in visual acuity and visual fields, as well as marked dyschromatopsia, severe deficits in contrast sensitivity, and markedly abnormal eye movements and visual-evoked potentials.