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To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH).
Methods:
Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG).
Results:
At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6–8.3) at final follow-up (p = 0.002).
Conclusion:
When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.
Acute isolated optic neuritis is often the first manifestation of multiple sclerosis (MS), and its management remains controversial. Over the past decade, with the advent of new disease-modifying agents, management of isolated optic neuritis has become more complicated.
Objectives:
To evaluate the current practice patterns of Canadian ophthalmologists and neurologists in the management of acute optic neuritis, and to evaluate the impact of recently published randomized clinical trials.
Design:
Mail survey.
Methods:
All practicing ophthalmologists and neurologists in Canada were mailed a survey evaluating the management of isolated acute optic neuritis and familiarity with recent clinical trials. Surveys for 1158 were mailed, and completed surveys were collected anonymously through a datafax system. Second and third mailings were sent to non-respondents 6 and 12 weeks later.
Results:
The final response rate was 34.5%. Although many acute optic neuritis patients initially present to ophthalmologists, neurologists are the physicians primarily managing these patients. Ordering magnetic resonance imaging, and treating with high dose intravenous steroids has become the standard of care. However, 15% of physicians (14% of ophthalmologists and 16% of neurologists) continue to prescribe low dose oral steroids, and steroids are being given for reasons other than to shorten the duration of visual symptoms by 73% of ophthalmologists and 50% of neurologists. More neurologists than ophthalmologists are familiar with recent clinical trials involving disease-modifying agents.
Conclusion:
Although the management of acute optic neuritis has been evaluated in large clinical trials that were published in major international journals, some ophthalmologists and neurologists are not following evidence-based recommendations.
Sturge-Weber syndrome is characterized by a facial cutaneous nevus (port-wine stain) and a leptomeningeal angioma, often found ipsilateral to the facial lesion. Epileptic seizures, mental retardation, and focal neurological deficits are the primary neurologic abnormalities of Sturge-Weber syndrome. In a series of 52 adults with Sturge-Weber syndrome, 65% had neurologic deficits including stroke, hemiparesis, spasticity, and/or weakness. Neuroimaging, electroencephalography, and functional testing with Positron emission tomography (PET) and Single-photon emission computed tomography (SPECT) may also help to define the extent of the intracranial lesion for possible epilepsy surgery. Although gyral calcification is a classic feature of Sturge-Weber syndrome, this "tram track" appearance is not always present. Bilateral calcification is common. Calcification often becomes more apparent as the patient becomes older but is sometimes already present at birth. Daily aspirin has been tried in an effort to prevent recurrent vascular thrombosis that may cause neurologic deterioration.
There are four types of cerebral vascular malformations: arteriovenous malformations (AVMs), cavernous malformations (CMs), capillary telangiectasies, and venous malformations. This chapter presents a review of the pertinent literature on CMs regarding epidemiology, genetics, pathology, clinical findings, and therapeutic management with special emphasis placed upon the natural bleeding risk of these malformations. The most sensitive imaging study to detect CMs is magnetic resonance imaging (MRI). Predictive factors for intracranial hemorrhage in patients harboring CMs is a critical issue because the optimal therapeutic management of such lesions is tailored according to the bleeding risk. The main goal of radiosurgical treatment should be a significant reduction in bleeding risk, especially after a latency period of 2 years. By genetic linkage analyses, three cerebral CM loci have been assigned to chromosome 7p, 7q, and 3q. They account for all familial forms of CM, thus constituting a formidable Mendelian model of stroke.
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