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[Relation between relative afferent pupillary defect and suprathreshold automated perimetry].

UNLABELLED: The purpose of this study was to test for correlation between relative afferent pupillary defect (RAPD) and visual acuity, central differential light threshold and visual field defect measured by suprasthreshold automated grid perimetry. METHODS: In 98 patients with optic neuropathies of different origin (inflammatory, ischemic, or compressive) in addition to a standard ophthalmological examination, static perimetry of the 30 degrees visual field with a dense grid of test spots, including measurement of the central differential light sensitivity, was done by means of the Tübingen Automated Perimeter. RAPD was tested using the swinging flashlight test quantified with neutral density filters. RESULTS: Visual acuity and central differential light sensitivity correlated only weakly with the RAPD. The visual field showed a better correlation (coeff. of corr. for all patients 0.58, for patients with optic neuritis 0.64. for patients with AION 0.53 and for patients with compressive neuropathies 0.84). One test spot not seen in perimetry corresponded to an RAPD of about 0.01 log-units. CONCLUSIONS: The results confirm those from previous studies obtained by threshold perimetry. The RAPD is a good parameter for the severity of an optic nerve lesion, especially of compressive origin.

Adult↗

[Dominant inheritance of pupillary pigment cysts. A follow-up study].

In 1969 Meyer described in this journal a family suffering from multiple congenital pigmented pupillary cysts with dominant mode of inheritance. The therapy--total iridectomy and photocoagulation of the cysts--resulted in only a temporary improvement in visual acuity. The cysts were between 1.5 and 5.0 mm in diameter. The present author recently examined a member of this family with severe contusion of the left eyeball. The trauma had evidently opened the residual embryonal ring sinus of the fetal iris system (von Szily). While the traumatic mydriasis persisted, the cysts did not refill.

Adolescent↗

[Manifestations of progressive paralysis. Not a theme of the past].

Investigations carried out on 293 cases of progressive paralysis showed that the widely held view of a "classical form" of progressive paralysis is not applicable. Thus megalomania was found in only 13% of the patients and a manic type state in less than half. Disturbances in affectivity, drive and intellectual functions in progressive paralysis are, in general, uncharacteristic and can appear in any psychotic syndrome. Moreover, neurological symptoms taken as characteristic for progressive paralysis such as the Argyll-Robertson phenomenon or the "mimic quivering" are more the exception than the rule.

Consciousness Disorders↗

Pupillary examination: do I really need to look?

A pupillary examination takes very little time and does not require expensive equipment. Unfortunately, many ophthalmic personnel do not take the time to thoroughly evaluate pupillary function. Pupillary examinations can be easy if divided into specific elements for assessment. These elements should include color, shape, size, equality, and reaction to stimulus. This article will briefly review some of the more common pupillary abnormalities and review the steps of the pupillary examination.

Humans↗

CT prognostic factors in acute subdural haematomas: the value of the 'worst' CT scan.

The relationship between radiological findings and outcome in patients with acute posttraumatic subdural haematomas (SDH) has been based on CT obtained upon hospital admission. This study was undertaken to investigate the effects on prognosis of SDH patients of lesions not present on admission, but detected by subsequent CT. We have also studied those findings present on admission CT that could predict worsening of the associated lesions. From 1 May 1989 to 30 April 1996, we admitted 206 patients harbouring acute SDH of thickness 5 mm or more. The admission GCS score ranged from 3 to 15. Each patient underwent CT on admission (always within 3 h from injury). Follow-up CT was performed within 12-24 h after injury and in the following days (an average of 4.3 examinations for each patient). These examinations were reviewed by a neuroradiologist and the 'worst' CT was determined. We defined the 'worst' examination as that showing the largest haematoma thickness/midline shift and/or with the most extensive degree of parenchymal damage. Clinical factors related to prognosis in this series are age, hypoxia/hypotension, GCS motor score and pupillary abnormalities. Time from injury to treatment was found relevant only in patients with isolated SDH. CT findings on admission that correlated with outcome were haematoma thickness, midline shift and status of the basal cisterns. Prognosis was also worsened by the presence of associated lesions; SAH alone or associated with brain contusions. The last of these was the single most powerful predictor of worse outcomes (Odds ratio 0.37, p < 0.004). Whereas the first CT showed parenchymal associated damage in 56 patients, the 'worst CT' showed such damage in 105 patients. Presence of SAH on admission was found significant (p < 0.02) in predicting evolving parenchymal damage. Haematoma thickness, midline shift, status of the basal cisterns and presence of SAH are related to outcome when identified on the initial (early) CT examination. However, early (within 3 h from injury) CT under-estimates the ultimate size of parenchymal contusions. Patients with SAH on early CT are those at highest risk for associated evolving contusions. The use of sequential CT should be included in the routine management of head-injured patients.

Adolescent↗

A new case of oculoectodermal syndrome.

An 11-month-old infant girl presented with right-sided features of aplasia cutis congenita of the scalp, unilateral epibulbar dermoids, eccentric pupil, coloboma of the right upper eyelid, and depigmentation of the fundus surrounding the right optic nerve. These findings were similar to the oculoectodermal syndrome reported by other clinicians and researchers.

Coloboma↗

Rieger's anomaly and other ocular abnormalities in association with osteogenesis imperfecta and a COL1A1 mutation.

A patient with osteogenesis imperfecta (OI) and some features of Ehlers-Danlos syndrome had Rieger's anomaly and other associated ocular abnormalities. He carried a COL1A1 mutation (c.3313delA) that has only rarely been seen in OI. The association of ocular anterior chamber abnormalities with OI has not been reported previously, while OI with Ehlers-Danlos syndrome features has only been described in some kindreds. The patient had serious complications as a result of his ocular anomalies. We speculate that the course of his disease and, perhaps, its co-existence with OI could be exacerbated by his collagen type-I defect, although no causality can be established by this report of a single case.

Abnormalities, Multiple↗

Association between cerebrospinal fluid interleukin-6 concentrations and outcome after severe human traumatic brain injury.

Acute inflammation plays a significant role in the pathophysiology of traumatic brain injury (TBI). However, the specific relationships between inflammatory mediators and patient outcome following TBI have not been fully established. In this study, we measured plasma and cerebrospinal fluid interleukin-1 (IL-1) and interleukin-6 (IL-6) concentrations in 36 patients, following severe TBI. Patients were monitored with continuous measurements of somatosensory-evoked potentials (SSEP) to derive an established surrogate outcome measurement, the 96-h evoked potential (SSEP96). Clinical outcomes were assessed at 3 months using the Glasgow Outcome Scale (GOS). Peak cerebrospinal fluid (CSF) IL-1 and IL-6 concentrations were significantly higher than those observed in the plasma [median 6.5 pg/mL (range 1.4-25.0) vs. 3.0 (0.8-7.6) for IL-1, and 650 (130-7,214) vs. 253 (52-1,506) for IL-6, p < 0.001 for both]. Peak CSF IL-6 levels correlated with SSEP96 (r = 0.42; p = 0.0133), and peak CSF IL-6 levels were higher with improved GOS (p = 0.024). Multiple regression analysis identified that age (p = 0.0072), pupillary abnormality (p = 0.021), the presence of mass lesion (p = 0.023), and peak CSF IL-6 concentrations (p = 0.026) were all statistically significant predictors of clinical outcome following TBI. These results suggest that peak CSF IL-6 concentrations correlate with improved outcome following TBI. This finding helps to characterize the inflammatory reaction associated with TBI and may help to develop improved treatment strategies for patients with TBI.

Adolescent↗

Haploinsufficiency for Phox2b in mice causes dilated pupils and atrophy of the ciliary ganglion: mechanistic insights into human congenital central hypoventilation syndrome.

Dilp1 is a semi-dominant mouse mutation that causes dilated pupils when heterozygous and is lethal when homozygous. We report here that it is caused by a point mutation that introduces a stop codon close to the start of the coding sequence of the paired-like homeobox transcription factor Phox2b. Mice carrying a targeted allele of Phox2b also have dilated pupils and the two alleles do not complement. Phox2b is necessary for the development of the autonomic nervous system and when absent one of the consequences is that all parasympathetic ganglia fail to form. Constriction of the pupil is a parasympathetic response mediated by the ciliary ganglion and we find that in Phox2b heterozygous mutants it is highly atrophic. The development of other parasympathetic and sympathetic ganglia appears to be largely unaffected indicating that the ciliary ganglion is exquisitely sensitive to a reduction in dose of this transcription factor. PHOX2B has been implicated in human disease. Mutations, principally leading to polyalanine expansions within the protein, have been found in patients with congenital central hypoventilation syndrome (CCHS), the cardinal feature of which is an inability to breathe unassisted when asleep. Additionally, some CCHS patients have ocular abnormalities, including pupillary defects, although they principally have constricted rather than dilated pupils. The apparent phenotypic differences observed between mice carrying a loss-of-function mutation of Phox2b and CCHS patients indicate that PHOX2B mutations found in CCHS patients, all of which can produce proteins with intact DNA-binding domains, are gain-of-function mutations that alter rather than abolish protein function.

Alleles↗

Pupillary contagion: central mechanisms engaged in sadness processing.

Empathic responses underlie our ability to share emotions and sensations with others. We investigated whether observed pupil size modulates our perception of other's emotional expressions and examined the central mechanisms modulated by incidental perception of pupil size in emotional facial expressions. We show that diminishing pupil size enhances ratings of emotional intensity and valence for sad, but not happy, angry or neutral facial expressions. This effect was associated with modulation of neural activity within cortical and subcortical regions implicated in social cognition. In an identical context, we show that the observed pupil size was mirrored by the observers' own pupil size. This empathetic contagion engaged the brainstem pupillary control nuclei (Edinger-Westphal) in proportion to individual subject's sensitivity to this effect. These findings provide evidence that perception-action mechanisms extend to non-volitional operations of the autonomic nervous system.

Adult↗

Orbital decompression for Grave's disease leaving the periosteum intact.

A prospective trial was carried out to determine whether or not transorbital floor and medial wall decompression without opening the orbital periosteum would relieve compressive optic neuropathy in patients with Grave's disease. In four orbits (three patients), the procedure was performed, and in all cases, the vision returned to 20/30 or better. All other parameters of optic nerve function improved as well. No patient had worsening of ocular motility that was attributable to the surgery. The amount of retroplacement of the globe may be less than with standard orbital decompression.

Adult↗

Angle closure glaucoma following a combined blepharoplasty and ectropion repair.

This paper reports an occurrence of angle closure glaucoma following a combined blepharoplasty and ectropion repair. We are unaware of any previous reports of such an incident. Specific to this case was the coexistence of a cataract that contributed to the narrowing of the anterior chamber. This condition, along with pupil dilation secondary to the anesthetic, precipitated a phacomorphic angle closure glaucoma attack, necessitating emergency cataract surgery. Because other procedures involve pupillary dilation as a potential side effect, we recommend an increased awareness of this potential postoperative complication.

Acute Disease↗