Role of ultrasonographic optic nerve sheath diameter in the diagnosis and follow-up of papilledema and its correlation with Frisén's severity grading.

Purpose: The aim of this study was to compare the ultrasonographic optic nerve sheath diameter (ONSD) in different grades of papilledema and in controls and to evaluate ONSD in atrophic papilledema/optic atrophy when raised ICP was suspected. Methods: Prospective cross-sectional case-control study. Following an ocular examination, papilledema was graded clinically using modified Frisén's grading. An ultrasonographic cross section of the retrobulbar optic nerve was obtained with a posterior transverse scan. Independent t-test and analysis of variance were the statistical tools used in the study. Results: The study included 55 cases and 55 age- and gender-matched controls; mean (± standard deviation) age was 37.17 (±11.25) years and male: female ratio was 49:61. There was a statistically significant difference in the mean ultrasonographic ONSD between cases [4.89 (±0.65) mm] and controls [3.12 (±0.22) mm] (P < 0.001). There was a significant difference in the mean ONSD across Frisén's grades of papilledema (P < 0.001). The mean ONSD in atrophic papilledema was 6.2 (±0.75) mm. Conclusion: In the presence of symptoms, ultrasonographic ONSD >4 mm is diagnostic of papilledema. Ultrasonographic ONSD correlates well with the severity of papilledema and can be used to follow-up patients with chronically elevated ICP. It is useful in detecting raised ICP in the presence of optic atrophy and to distinguish true papilledema from pseudopapilledema.

SCA1 patients may present as hereditary spastic paraplegia and must be included in spastic-ataxias group.

INTRODUCTION: The combination of cerebellar ataxia and spasticity is common. However, autosomal dominant genetic diseases presenting with spastic-ataxia are a smaller group. Pyramidal signs have been frequently observed in several SCA subtypes, particularly in spinocerebellar ataxia type 1. METHODS: We prospectively evaluated the pyramidal signs and spasticity in SCA1 patients, and correlated the data with genetic and clinical features. RESULTS: In this study, we observed that spasticity may be an early and presenting feature of SCA1, since 3 patients had pyramidal signs and spasticity as the first neurological sign. SCA1 patients with spasticity were significantly younger. CONCLUSION: SCA1 may rarely present with pure spastic paraplegia, resembling hereditary spastic paraplegia, before the appearance of cerebellar signs. This observation may confuse the neurologist when a genetic testing is requested for an autosomal dominant spastic paraplegia, directing research to hereditary spastic paraplegia group.

Differentiation of parapapillary atrophy using spectral-domain optical coherence tomography.

PURPOSE: To develop a classification of parapapillary atrophy (PPA) based on its relationship with the location of Bruch's membrane (BM) termination in primary open-angle glaucoma (POAG) patients. DESIGN: Cross-sectional observational study. PARTICIPANTS: This study analyzed 161 eyes from 161 POAG patients who had temporal ß-zone PPA, the width of which was more than 200 µm on at least 1 horizontal scan image obtained by spectral-domain optical coherence tomography within the mid horizontal one third of the optic nerve. METHODS: Based on the extent of BM within the PPA area, eyes were categorized as group A (intact BM; 76 eyes), group B (discontinuous BM; 65 eyes), and group C (lacking BM; 20 eyes). Differences in the demographic, clinical, and ocular characteristics were compared using analysis of variance and chi-square tests among the 3 groups. The distance from the temporal optic disc margin to the temporal margin of the ß-zone PPA (PPA width) and to the edge of the BM (width of PPA without BM [PPA-BM]) were measured on 3 horizontal scans within the mid horizontal one third of the optic nerve, and the averages of the measured values were analyzed. The configuration of the border tissue of Elschnig at the temporal disc margin was assessed. MAIN OUTCOME MEASURES: Factors and configuration of the border tissue of Elschnig associated with each PPA type. RESULTS: The mean age of group A was significantly higher than that of groups of B and C (P<0.001). The mean axial length was greatest in group C (group C>group B>group A; P<0.001). In group A, the border tissue mainly had a nonoblique configuration (49/76 eyes; 64.5%), whereas most of the eyes in group B (59/65 eyes; 90.8%) and all eyes in group C (20 eyes) it had an externally oblique configuration (P<0.001). A longer axial length was correlated significantly with a larger PPA-BM width (r = 0.478; P<0.001). CONCLUSIONS: A morphologic classification of PPA, which may reflect differing pathogenesis among the groups, is proposed. Parapapillary atrophy with intact BM may be an age-related atrophic change, whereas PPA lacking BM may result from scleral stretching associated with elongation of the globe. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Miopia patológica / Myopia

Os autores fazem uma revisão bibliográfica sobre a miopia patológica, sua incidência, fisiopatologia, prognóstico e tratamento

Nonglaucomatous optic disk atrophy and excavation in the elderly.

The causes of nonglaucomatous optic disk atrophy and excavation are enumerated in people 65 years or older: congenital anomalies, myopia, ischemic disorders, transsynaptic degeneration, traumatic, compressive, hereditary, toxic and infectious optic neuropathy.

PEHO or PEHO-like syndrome?

PEHO syndrome is a rare progressive infantile encephalopathy, with variable age of onset of hypotonia, convulsions, mental retardation, oedema, and optic atrophy. Neuroimaging shows cerebellar and brainstem atrophy in most instances. A PEHO-like syndrome has been described in which those affected do not have the typical changes on neuroimaging. We report four new cases, two isolated cases and two sisters, who might be part of the PEHO-like syndrome.

[The parapapillary region of normal and glaucoma eyes. I. Planimetric values of 312 glaucoma and 125 normal eyes]. / Die parapapilläre Region in Normal- und Glaukomaugen. I. Planimetrische Werte von 312 Glaukom- und 125 Normalaugen.

Magnification-corrected planimetry of the parapapillary region was performed according to Littmann's method in 312 unselected eyes with chronic primary open-angle glaucoma and in 125 normal eyes of an age- and refraction-matched control group using optic disk photographs. The glaucoma group was divided into five pathomorphologic subgroups. High myopics (less than -8.00 D) and "ocular hypertensives" had been excluded. The coefficient of variation ranged intraindividually from 0.0 to 0.17 and interindividually from 0.0 to 0.16. Two different morphologic variants were defined and examined: 1) Zone "Alpha" with incipient to advanced parapapillary chorio-pigmentepithelio-retinal atrophy - characterized by irregular hypo- and hyperpigmentation - was statistically proven in the control group (0.60 +/- 0.44 mm2; p less than 0.05; Wilcoxon-Mann-Whitney test) to be smaller than in the glaucoma group (0.81 +/- 0.70 mm2). It increased significantly (p = 0.0000) with advancing glaucoma stage. In the glaucoma and normal group it was broadest in the temporal horizontal sector (p less than 0.001; Wilcoxon test), followed by the temporal lower (p less than 0.001), temporal upper (p less than 0.001) and nasal sectors (p less than 0.001). There was no significant difference in prevalence between the two groups. 2) Zone "Beta" with subtotal to total parapapillary chorio-pigment-epithelio-retinal atrophy was also smaller in the normal eyes (0.18 +/- 0.52 mm2, prevalence: 20.0%; p = 0.0000) than in the glaucomatous ones (0.85 +/- 1.42 mm2, prevalence 66.7%) and was also, in both groups, broadest in the temporal horizontal sector, followed by the temporal lower, temporal upper and nasal sectors. In the control group it was smaller than zone "Alpha" (p less than 0.00001), while in the glaucoma group there was no difference. 3) The difference between normal eyes and earliest glaucoma stage I was for zone "Beta" (p = 0.0000); the difference between the normal eyes and those of glaucoma stage II was significant for both zones (p = 0.0000 and p less than 0.05, respectively). In both groups and in all glaucoma stages both zones were larger in the lower half of the optic disk than in the upper half. 4) "Conus pigmentosus" and the peripapillary scleral rim in normal and glaucomatous eyes showed no significant difference as regards their area and frequency. The parapapillary chorio-pigmentepithelio-retinal alterations are precursors of, or are equivalent to the so-called "halo glaucomatosus".(ABSTRACT TRUNCATED AT 400 WORDS)

Congenital anomalies of the retina.

Review of 50 histopathologic cases and a number of clinical cases of congenital retinal anomalies has permitted classification under the following headings: 1) Coloboma-orbital cyst--"anophthalmos" group due to aberrant closure of the embryonic fissure; 2) Retinal fold-central stalk-detachment group comprising a series that varies from simple retinal folds to total retinal detachment and anomalous stalk formation. Cases of the 13-15 trisomy syndrome constitute a special subgroup in this rubric; 3) Retrolental fibroplasia, due to hyperoxia of premature infants, is manifest by "dragged" disks and gliovascular proliferation with occasional detachment; 4) Persistent hyaloid system is occasionally associated with mild anomalies of the retina; 5) Massive gliosis of the retina is usually a hamartomatous manifestation; 6) Congenital absence of ganglion cells occurs with cerebral maldevelopment and 7) Congenital absence of the photoreceptors is the congenital form of retinitis pigmentosa.